DISEASES OF THE CARDIOVASCULAR Aortic (aneurysm) dissection: Very severe,

SYSTEM sharp, tearing chest pain;

Cardiovascular diseases (CVDs) carry a high typically radiating to the back; loss of pulses,
morbidity and the highest mortality unequal BP between arms, or
rate among all diseases in most countries. aortic insufficiency; neurologic signs;
CVDs are closely related to human life mediastinum widened on CXR. MI
styles and thus can be reduced significantly may occur if dissection extends into
by regular health examinations, coronary artery (Cor-A). Diagnosis is
consultations, and life style modifications. confirmed by TEE, CT scan, or aortography.
Pulmonary embolism (PE): Sudden chest
pain, dyspnea, tachycardia,
PEARLS: IMPORTANT DIFFERENTIATIONS
OF CHEST cough, and hypoxemia, usually 3-5 days
PAIN after a surgery or long immobility.
Chest pain is the most common symptom for The chest pain is usually pleuritic but may
most CVDs, respiratory diseases, resemble angina. CT pulmonary
and some upper abdominal diseases. Thus, it angiography has supplanted V/Q scanning as
’s important to grasp the differential the preferred means of
points. diagnosis.
Angina and myocardial infarction (MI): See Mitral valve prolapse: Transient chest pain
details on the same topic below. with typical midsystolic click
Myocarditis: It is usually preceded by a viral murmur.
disease, with a vague chest
pain. Creatine kinase (CK)-MB is often Pulmonary hypertension: Dull chest pain
increased. ECG (EKG) will show with symptoms and signs of right
abnormal conduction or Q waves. ventricle (RV) failure.
Pericarditis: It may be preceded by a viral Costochondritis: Chest pain is usually
illness. Chest pain is sharp, stabbing, localized, and exacerbated
pleuritic, and positional -- worse with lying with inspiration; reproducible or worse with
down and relieved by sitting up. chest palpation. ECG is normal
Pericardial rub is often positive. ECG (Nl).
usually shows diffuse ST elevation Gastric diseases: GERD (burning chest pain,
without Q waves. CK is mostly normal. It acid reflux, bad taste, relief with
responds well to anti-inflammatory antacids); stomach spasm; PUD (epigastric
drugs. pain before or after eating).
Pleuritis: It usually ocuurs after lung Pancreatitis: Post-meal persisting sharp
infection; with sharp chest pain worse epigastric pain radiating to the back,
on inspiration and certain position; with nausea/vomiting, fever, and increased
tenderness, friction rub or dullness may be amylase and lipase levels.
present. CXR or CT scan is the best means of Gallbladder disease: (Post-meal) right upper
diagnosis. quadrant (RUQ) abdominal pain
Pneumonia: Moderate chest pain with fever, with tenderness, nausea/vomiting, jaundice,
cough, sputum, and hemoptysis. etc.
CXR is the best test. Hiatal hernia: Burning chest or epigastric
Pneumothorax: Sudden, sharp, pleuritic pain; nausea/vomiting; reflux of
chest pain and dyspnea; absent food; relief with antacids.
breath sounds; mediastinum shifted to the
opposite site. Suspect of tension ARRHYTHMIAS
pneumothorax requires emergent intercostal Definition: Abnormality (Abnorm) of cardiac
needle puncture. Non-tension rhythm. It can be asymptomatic
pneumothorax can wait for CXR confirmation (Asympt), symptomatic (Sympt), or lethal.
and natural relief. Causes of arrhythmia are various and

1
should be treated specifically. Prophylactic
antiarrhythmic drugs are generally
not recommended because they increase the
mortality (especially for ventricular
tachycardia, V-Tach). Common cardiac

coronary artery (Cor-A), leading to cardiac

dysfunctioning. It’s fatal if there’s
narrowing of 1-2 major coronary arteries
that causes > 75% decrease in crosssectional
area (or 50% decrease in diameter).
Factors that increase O2 demand: Physical
exertion or stress, emotional or
mental stress (including anxiety), large
meals, etc.
arrhythmias are summarized in Table 2-
Factors that lower the O2-carrying capacity
ISCHEMIC HEART DISEASE (IHD) of the blood: Anemia, CO
-- CORONARY ARTERY DISEASE (CAD) poisoning, platelet microthrombi at the site
of coronary stenosis (local damage).
Etiology and pathogenesis Major risk factors
The basic mechanisms of CAD are that Age (male > 45, female > 55 y/a), male
coronary O2 demand surpasses supply gender, smoking, hypertension,
due to decreased blood flow secondary to diabetes, heredity (including race, family
atherosclerotic narrowing of the history < 55 y/a), atherosclerosis,

2
hypercholesterolemia (Hyper-Chol, LDL > 200 6. Associated symptoms: Short of breath
mg/dL, HDL < 40 mg/dL), (SOB), anxiety, fatigue, nausea and
physical inactivity, obesity and overweight, vomiting (N/V), palpitations, and
stress, excess alcohol use, and diaphoresis.
postmenopausal women. 7. Pain relief: Nitroglycerin (in a few min),
The metabolic syndrome (Insulin resistance resting (standing or sitting).
syndrome): It refers to any 8. P/E (Physical examination): Tachycardia,
combination of hyperglycemia, diaphoresis, and transient S4
hyperlipidemia, hyperuricemia, and gallop.
hypertension. Genetic predisposition, lack 9. ECG: ST-T depression.
of exercise, and body fat distribution II. Types of angina
may increase the likelihood of developing 1. Chronic stable angina: It occurs during
diabetes and CVD. exertion, can be relieved by rest or
(Cardiac) syndrome X: It refers to angina nitrates, and can recur. ECG usually shows
pectoris (ischemia) occurring in evidence of ischemia during pain or
patients with normal coronary arteriogram. stress testing. Angiography mostly
Prognostic indicators demonstrates significant obstruction of
(1) Left ventricular function--Ejection fraction major
(EF): Normal > 50%; if < coronary arteries.
50%, associated with increased mortality. 2. Unstable angina: New onset of chest pain
(2) Severity--Vessels involved: Left main that occurs at rest or with less
coronary artery or > 2 arteries exertion, or requires more medicines to
indicating worse prognosis. relieve. It follows a worsening pattern
in frequency, duration, or/and severity of
Angina Pectoris symptoms. It should be considered and
It mostly refers to “stable angina”, a managed as a form of acute coronary
paroxysmal chest pain resulting from syndrome. Diagnosis is based on
cardiac ischemia--an imbalance between presence of severe angina in those patterns
oxygen supply and demand, and is without ST-elevation on ECG. It
most commonly caused by the inability of
atherosclerotic coronary arteries to often progresses to myocardial infarction,
perfuse the heart under conditions of and thus should be stabilized with
increased myocardial oxygen consumption aggressive medications (aspirin, beta
(exercise, stress). Stable angina is when blockers, LMWH, nitrides, etc.) before
the chest pain is precipitated by stress testing or cardiac catheterization is
predictable factors (exercise, exertion). performed.
Unstable angina is angina that occurs at 3. Prinzmetal (variant) angina: Chest pain
any time. occurs at rest or stress without
Essentials of diagnosis the usual precipitating factor of exertion, due
I. Clinical features to coronary artery spasm. It
1. Nature of the pain: Heavy, pressing, or may result from an altered autonomic
squeezing. neurologic control of the coronary arteries
2. Location: Substernal, precordial. or altered artery contraction. ECG mostly
3. Radiation: Commonly to the left jaw or shows ST-T elevation. Cardiac
arms.
catheter will show no atherosclerosis.
4. Duration: 15 sec—15 min.
Management is mainly a diagnostic
5. Precipitating factors: Exertion, anxiety,
therapy of a Ca-blocker (the best diagnostic
meals, and coldness. Risk factors
treatment, which usually relieves
are the same as above.
symptoms immediately), or a nitrate.
III. Lab diagnosis

3
1. ECG: It is the best initial test for all forms to detect the presence of narrowing that is
of chest pain. It can exclude best treated with surgery or
previous myocardial infarction (MI) or angioplasty--usually when more than 70%
obvious arrhythmia; also to evaluate the stenosis exists. Coronary angiography
use of regular stress test versus thallium is often used when ECG or stress testing
testing. results are equivocal.
2. Exercise ECG/echocardiography or 7. Holter monitoring: It’s a continuous
treadmill (stress) test: When ECG is ambulatory ECG monitoring that records
normal or uncertain, stress testing is the best the rhythm, usually used for arrhythmias and
diagnostic test to confirm for a 24-hour period (but may
angina (ischemia), determine the severity of
extend to 48-72 hours). It does not detect
disease, and evaluate post MI
conditions. Echocardiography is more ischemia (It’s not accurate for ST
sensitive than ECG. Stress test is (+) if segment evaluation).
any of the chest pain, ST-segment 8. Cardiac catheterization: Usually applied
depression, hypotension, or significant in patients poorly controlled with
arrhythmia is induced by exercise. drugs or positive stress test, to help
3. Thallium (scan)-treadmill test--similar to determine the need for angioplasty--triple
exercise echocardiography but vessel disease or left main coronary artery
focusing more on the coronary arteries)-- disease. Occupational workers for
Indications: public safety (bus driver, airline pilot)
(1) Patients with uncertain regular with any CAD symptoms require cardiac
treadmill testing. catheterization.
(2) Patients with mitral valve prolapse. Differential diagnosis
(3) Patients with WPW syndrome. See “Important Differentiations of Chest
(4) Patients with left bundle branch block. Pain” above.
(5) Young female with high false positive Treatment
results on regular stress testing. 1. Medical therapies for angina:
Drugs that can reduce mortality for a patient
(6) Patient with uncertain acute ischemic
with chronic angina include
changes on ECG (nonspecific ST-wave aspirin, beta-R blockers, and nitrates
changes, inability to read the ECG, etc). (nitrates did not show decreased
(7) Patient requiring quinidine, mortality in acute MI).
procainamide, or digitalis. (1) Nitrates: They are the first-line therapy
Ischemia versus infarction: In ischemia, it for acute angina. Low doses of
will show a reversal of the decrease nitrates increase vein-dilation and decrease
in thallium uptake or wall motion that will preload; high doses of nitrates
return to normal after a period of rest. increase small-artery and coronary artery
4. For patients unable to exercise: Perform dilatation and O2 supply, and decrease
dipyridamole-thallium, adenosinethallium, both afterload and preload.
or stress or dobutamine echocardiography. Adverse effects (S/E): Vasodilation can lead
5. Contraindications for stress testing: to orthostatic hypotension, reflex
Unstable angina, aortic stenosis, tachycardia, throbbing headache, and
blushing. It’s contraindicated if systolic
idiopathic hypertrophic subaortic stenosis, BP
severe COPD, acute CHF, acute < 90 mmHg (to avoid syncope). It ’ s
ischemic changes on ECG, aortic dissection, important to have a > 8-hour window-free
and severe hypertension. period to reduce the incidence of
6. Coronary angiography: This is the most tachyphylaxis (usually 12 am—6 am).
accurate method of detecting CAD, (2) Beta-R blockers: They decrease HR,
contractility, and blood pressure, and

4
thus decrease myocardial O2 requirement. Diltiazem: It causes moderate A-V block and
They are contraindicated in severe increase in coronary blood flow,
asthma as well as vasospastic or variant mild decrease in contractility, and mild
(Prinzmetal) angina to avoid induction hypotension.
of coronary vasospasm from unopposed alpha- Nifedipine: It has minimal A-V block; mild
receptor activity. Long decrease in cardiac contractility;
maintenance therapy has been shown to significant hypotension; increase in
reduce mortality in acute MI and coronary blood flow, ankle edema, and
headache. It’s contraindicated in aortic
CHF and risk of reinfarction. stenosis and unstable angina.
Adverse effects (S/E): Fatigue, (4) Newer therapies: Ranolazine, a late Na-
bronchoconstriction, depression, channel blocker, is used either in
hallucinations, combination with a beta blocker or as a
sexual dysfunction, insomnia, dyslipidemia, substitute in patients who cannot receive
Raynaud phenomenon. A one.
nonselective beta-R blocker (propranolol) 2. Treatment of unstable angina:
may mask hypoglycemic symptoms in
IDDM and is not routinely used in CAD. (1) Hospitalize the patient and treat with
Selective beta1 blockers (with less S/E): aggressive medications --aspirin,
Atenolol, metoprolol, or acebutolol is nitrates, beta-R blockers, heparin, and
most commonly used in angina pectoris and
lipid-lowering agents as described above.
MI (and CHF).
Heparin (IV or SC) or low molecular weight
New nonselective beta-R blockers: Penbutolol
heparin (LMWH) is the major
and carteolol are used for
therapy because of its high efficacy.
hypertension.
(2) Glycoprotein inhibitors with angioplasty
(3) Ca-channel blockers: They decrease
and stent placement are very
preload and afterload. It may be
effective, but thrombolytics are not.
harmful during the post-infarction period,
(3) Revascularization:
especially if patient has left
(a) CBG (Coronary bypass graft): Very
ventricular failure. Its efficacy in angina
useful in those with major left
is limited.
coronary disease or 3-vessel disease and
S/E: Cardiac--reflex tachycardia,
left ventricular (LV) dysfunction. It’s
hypotension, and dizziness, CHF; noncardiac-
indicated in cases with symptoms despite
-
medical treatment or with severe
flushing, headache, weakness, nausea,
adverse effects from therapies. It’s more
constipation, wheezing, and peripheral
beneficial in those with diabetes or low
edema.
ejection fraction, although the performance
PEARLS: Specific Ca-channel blockers with
various selectivity carries more risk.
Strong on heart: Verapamil (b) PTCA (Percutaneous transluminal
Strong on peripheral vessels: Nifedipine coronary angioplasty): It is indicated
Intermediate: Diltiazem in significant cardiac lesions not eligible
Verapamil: It causes significant A-V block for CBG. It’s an easier procedure with
in ECG, moderate decrease in more risk of re-stenosis. Stent placement is
coronary blood flow and cardiac now a standard procedure.
contractility, hypotension, and ankle edema. Glycoprotein 2b/3a inhibitors (abciximab,
It ’ s contraindicated in sick sinus tirofiban, or eptifibatide) are usually
syndrome, A-V node block and ejection used with the procedure, followed by aspirin
fraction (EF) < 35%. plus ticlopidine or clopidogrel.
3. Preventive therapies:

5
(1) Lifestyle modification for risk reduction 2. Subendocardial infarct: It ’ s mostly
(highly important!): Smoking “non-Q wave” MI, confined to the inner
cessation; reduction of stress, weight, and 1/2 to 1/3 of the LV wall. The LV
Chol & Trig; regular exercise; subendocardium region is most susceptible to
treatment of diabetes, hypertension, anemia, ischemia, because of tenuous oxygen supply.
COPD, etc. Diltiazem use can reduce the risk
(2) Antiplatelet therapy: Low-dose aspirin of recurrence.
daily is very effective in prevention PEARLS: Coronary artery anatomy and MI
of angina. New antiplatelet drug --ticlopidine (Images 1-10)
or clopidogrel is an alternative LCA and LAD —Supply most of the LV and
to aspirin in patient who cannot tolerate the anterior interventricular
aspirin. Note that ticlopidine can cause septum; the most common occlusion in
adverse neutropenia. CAD, causing LV anterior wall MI.
(3) Lipid management: See CFX—Circumflex artery, supplies the left
“HYPERLIPIDEMIA”. lateral wall; its occlusion causes
lateral wall MI.
Myocardial Infarction (MI) RCA—Supplies the SA, AV nodes, and most
MI is ischemic myocardial necrosis as a of the inferior portion of the LV.
result of an abrupt reduction in the Its occlusion causes inferior MI.
coronary blood flow to a segment of Essentials of diagnosis
myocardium, usually due to a 1. Symptoms: Characteristic chest pain--
thrombotic occlusion of a coronary artery Severe, crushing, prolonged (usually
previously narrowed by > 20min) chest pain, similar in quality to but
atherosclerosis. MI is associated with a 30% more severe than angina;
mortality rate and 50% prehospital
deaths. associated with dyspnea, anxiety,
Etiology diaphoresis, nausea, vomiting, weakness, low
Atherosclerosis by all causes is the main fever, sense of impending doom, and
pathologic basis. Most cases are due to syncope (in elderly). Painless and
acute coronary thrombosis--atheromatous atypical MI can be up to 1/3 cases and more
plaque ruptures into the vessel lumen likely in postoperative or diabetic
and thrombosis forms on top of the lesion patients and the elderly. Sudden cardiac
causing the vascular occlusion. death can occur due to ventricular
Risk factors: Same as those for CAD (above). fibrillation (V-fib).
Non-atherosclerosis causes: Vasculitis, 2. Signs: Congestive heart failure (CHF) -
SLE, polyarteritis nodosa, Takayasu -arrhythmias (mostly tachycardia;
arteritis, mucocutaneous lymph node (LN) inferior MI may have bradycardia), S4
syndrome (Kawasaki disease, Image gallop, JVD, and dyskinetic left-Ventr
36), coronary spasm, variant angina, cocaine impulse. Cardiogenic shock signs are seen
abuse, coronary artery embolus, with > 40% of myocardial infarction
atrial myxoma, atrial or ventricular --BP decrease, S3 gallop, and rales.
thrombus, polycythemia vera, Systolic murmur of papillary muscle or
thrombocytosis, and anomaly of coronary Ventr-septal rupture or pericardial friction
arteries. rub (usually with transmural
Pathogenesis infarction) on the 3rd-4th day may be heard.
Acute MI is mostly “ST-elevated MI” and 3. PEARLS--ECG (Images 9-10): It’s the best
localized to the left ventricle (LV) diagnostic test before 6 hours
and in one of the two forms below: of onset and represented by ventricular wall
1. Transmural infarct: It ’ s more often hypokinesia, peaked T waves
associated with Q waves.

6
(early), ST-segment elevation (transmural 7. Special type of MI: It may be clinically
infarct) or depression silent or present as CHF or
(subendocardial lesion), new left bundle dysrhythmia in the absence of chest pain,
branch block (LBBB), or Q waves
especially in elderly, postoperative,
(necrosis, late).
hypertensive, or diabetic patients.
LV anterior wall MI (#1 common): ST-segment
elevation (+ Q wave) in Figure 2-1: Biomarkers of Acute MI (Courtesy
anterior leads (V1-4). of www.circ.ahajournals.org)
Posterior MI: ST-segment depression (+ large
R wave) in inferior leads (V1- Differential diagnosis
2). Angina, pulmonary embolism (PE),
Inferior MI: ST-segment elevation (+ Q wave) pneumothorax, pneumonia, aortic
in inferior leads (II, III, and dissection, pericarditis, costochondritis,
aVF). etc.
Lateral wall MI: ST-segment elevation (+ Q Treatment of MI
wave) in leads I, aVL, and V5-6. 1. “ABC” first--airway, breathing, and
4. Cardiac enzymes (Figure 2-1): circulation. Supplemental oxygen has
(1) CK-MB is both highly sensitive and shown a reduction in the risk of death.
specific for MI when measured within 2. Treat sustained ventricular arrhythmia or
36 hrs of chest pain. It begins to elevate at heart failure rapidly.
4-6 hrs after MI, reaches a peak at 3. Beta1-R blockers: They’ve been shown to
12-24 hrs and is back to normal in 72 hrs. reduce post-MI mortality clearly
CK levels may increase following if no contraindications exist (bradycardia,
cardioversion, defibrillation, cardio- A-V block, hypotension, or COPD).
pulmonary resuscitation, or muscle trauma, A beta blocker (metoprolol) is a good early
but the MB fraction will only increase with start (IV per 5 min) after an acute
certain extent of myocardiocyte MI and continuum as a maintenance therapy.
death. 4. Nitrates (PO or IV): It can alleviate pain,
(2) Troponin is most specific but moderately lung congestion, and left heart
sensitive; it begins to rise 2-4 hrs failure, but not clearly reduce post-MI
after the start of the chest pain, and mortality.
remains high for 7-10 days. It’s a more 5. Antiplatelet therapy: Aspirin (PO) can
valuable biomarker for MI with the chest pain reduce coronary reocclusion by
within 8 hrs and after 36 hrs. inhibiting platelet aggregation on top of
(3) Lactate dehydrogenase (LDH): It’s non- the thrombus and clearly reduce post-
specific and not used for diagnosis MI mortality. It ’ s used as part of the
of acute MI, but useful for re-infarction. In maintenance therapy. Clopidogrel,
acute MI, LDH increases after 12 ticlopidine (less S/E), or prasugrel is
hrs of chest pain and peak in 24-72 hrs and indicated in (1) aspirin intolerance (such
remains high for 10-14 days after as allergy); (2) recent angioplasty with
stenting. Prasugrel has more risk of
MI. LDH-1/LDH-2 ratio > 1.0 supports of MI. hemorrhagic stroke in elder patients. Other
5. CBC: Leukocytosis of 10-20 x 103/uL. NSAIDs should be avoided or
6. Thallium-201 (Tl-201) and Technetium-99m
(Tc-99m) scan: Tl-201 scan stopped in MI.
is sensitive but not very specific because 6. Thrombolytic therapy: Best within 6-12
it cannot distinguish between zones of hrs for ST segment elevation MI;
severe ischemia ( “ cold spots ” ) and the earlier, the better outcome. Indications
infarction. Tc-99m scan provides better include typical chest pain of acute
resolution for the same function. MI <12 hrs and new LBBB. t-PA (with more
tissue selection), streptokinase

7
(with stronger effect), reteplase, or
anistreplase is among the good options. block; (2) Acute MI when thrombolytics are
Beyond 24 hrs of symptom onset, it is usually contraindicated or patient is in a
ineffective and well-equipped hospital; (3) Clinical CHF,
contraindicated. post-MI patient with CHF, EF < 40%,
Complications of thrombolysis: recurrent ischemia and ventricular
(1) Overuse: hemorrhage, more common with arrhythmias, or failed thrombolytic
tissue plasminogen activator. therapies.
(2) Re-perfusion arrhythmias. For most patients, clinical trials have
Contraindications to thrombolytic therapy:
demonstrated superiority of primary
(1) Active bleeding disease;
percutaneous coronary intervention (PCI),
(2) Dissecting aortic aneurysm (suspect);
irrespective of whether balloon
(3) Uncontrolled hypertension > 180/110
angioplasty or stenting is performed. Bypass
(First control BP, then give
surgery — Coronary artery bypass
thrombolytics);
graft surgery (CABG) is infrequently
(4) Known traumatic CPR;
performed in patients with STEMI. The
(5) Recent head trauma or stroke (< 3
main indications are for emergent or urgent
months);
CABG related to failure of
(6) History of major trauma or surgery (< 3
fibrinolysis or PCI, or hemodynamically
yrs).
important mechanical complications.
7. Analgesics: IV opiates (morphine) are
The benefit of revascularization must be
important to relieve pain, to supply
weighed against the increase in
relaxation and sedation, and to alleviate
mortality associated with CABG in the first
CVS and respiratory stress effectively.
three to seven days after STEMI.
8. ACE inhibitors (Angiotensin-converting
Thus, if the patient has stabilized, surgery
enzyme inhibitors, ACE-I): It has
should be delayed to allow myocardial
shown to reduce post-MI mortality. It’s best
recovery. Patients with critical anatomy
beneficial for post-MI patients
should undergo CABG during the initial
with CHF, LV dysfunction with an EF
hospitalization.
(ejection fraction) < 40%, or
Contraindications to full-dose
regurgitant disease. It should be started
anticoagulation:
early and in the maintenance therapy.
(1) Active bleeding disease;
It’s also used in any anterior wall MI and
(2) Recent major surgery;
should be stopped after 6 weeks. Dry
(3) Severe hypertension (sustained BP >
cough is the most common S/E. If it ’s
190/110 mmHg);
intolerable to the patient, the ACE-Inh
(4) Hemorrhagic diathesis (congenital,
should be ceased and another agent be
hepatic, or drug-induced);
considered.
(5) Presence of purpura;
9. Hypolipidemic therapy: Atorvastatin
(6) Infectious endocarditis;
should also be started early and before
(7) Anticipated invasive bedside procedures
PCI.
(e.g., thoracentesis, artery or vein
10. Anticoagulation: Heparin--IV bolus
insertion).
initially and then continuous infusion to
12. Erythropoietin: Its non-erythropoietic
keep the PTT 1.5-2 times the normal value.
effects including anti-inflammatory,
It’s useful for unstable angina and as
antiapoptotic, and angiogenic properties may
a follow-up treatment for t-PA use.
be cardioprotective in patients with
11. Coronary angiography and angioplasty—
acute ST-elevated MI.
indications:
13. Post-MI management:
(1) Patients with typical and persistent
(1) Stress testing: All post-MI patients
symptoms with new left bundle branch
should have a submaximal stress test

8
(70% target load) after 5-7 days or a maximal and perfusion through the coronary artery.
stress test (85% target load) after 3. Mechanical disruption: Papillary muscle
2-3 weeks. dysfunction or rupture (causing
(2) Postinfarction angina or ischemia on mitral regurgitation, with systolic murmurs
stress test: Angiography is at apex radiating to the left axilla),
recommended to determine the need for Ventr-septal rupture (within 10 days,
angioplasty or bypass surgery. repairable), free wall rupture (causing
(3) Medical treatment: A beta-R blocker and cardiac tamponade, with 90% mortality), or
aspirin should be given to all post- pseudoaneurysm (risky of rupture).
MI patients without a specific Treatment requires emergent surgical repair.
contraindication. ACE-Inh should be used in 4. Acute pericarditis—Dressler syndrome—
cases Post-MI syndrome: It’s
with EF < 40%. Lipid lowering drugs are used immunologically based, with fever, malaise,
to maintain LDL < 100 mg/dL. pericarditis, pleuritis, and
Smoking and alcohol drinking should be leucocytosis; usually late onset, 2-4 weeks
stopped. post-MI. The effective therapy is
aspirin. Steroids should be avoided because
Complications of acute MI they may hinder myocardial scar
1. Arrhythmias formation.
(1) Sinus bradycardia: commonly seen early 5. Thromboembolism: Mural thrombus with
stages of (inferior) MI due to sinus systemic embolism or DVT with
or A-V junctional block and may be protective. prolonged immobilization. Frequent
Usually no treatment is needed. movements are the best prevention and
If it ’ s severe, atropine or temporary treatment.
pacing can be applied. 6. Postinfarction angina: If it ’ s after
(2) Premature atrial or ventricular thrombolytic therapies, it should be treated
contractions (PVC): observation. with angioplasty or bypass surgery.
(3) Tachyarrhythmias: Supra-ventr (SVT) -- 7. Recurrent infarction: It includes
adenosine is the #1 choice; extension of existing infarction and
Ventrtachycardia reinfarction,
(V-tach) -- lidocaine is the #1 drug (smaller with high mortality. Diagnosis is difficult
dose for the elderly, but should be suspected if
and never used as prophylaxis because there is a persistent elevation or re-
lidocaine can induce V-tach or Vfibrillation elevation of ST-segment and high CK-MB
by itself); V-fibrillation (V-fib) or
asystole--immediate defibrillation after 36 hours. Treatment is repeating
and CPR to save life! thrombolysis or urgent cardiac
(4) Temporary transvenous pacing— catheterization and PTCA, along with
indications: Complete A-V block; 2nd standard medical therapies for MI.
degree A-V block (type 2); sinus bradycardia 8. Sudden cardiac death: Mostly due to
despite atropine use; LBBB during Ventr-fibrillation or/and asystole.
MI; new bifascicular block; with
hypoperfusion. Right Ventricular Myocardial Infarction (RVMI)
2. Pump dysfunction: Left or right RVMI mainly results from occlusion of the
ventricular or bi-ventricular failure; proximal right coronary artery,
Ventraneurysm; acompanying about 30% of the inferior LV-MI.
infarct expansion. Severe left or bi- The patient usually shows a
ventricular failure is an indication typical right-sided infarction and heart
for intra-aortic balloon counter-pulsation. failure, with the classic physical triad of
This can increase cardiac output

9
hypotension, JVD, and clear lungs on onset. Renal inefficiency can result in a
auscultation. ECG usually reveals STsegment false increase in troponin.
elevations in an inferior and a posterior 3. Reinfarction: If a patient presents with
distribution (I, III, aVF, and a new chest pain within a few days of
V4R). the first infarction or attack, perform an
Diagnosis: Based on the above “Triad”, ECG to detect new ST segment
increased cardiac enzymes, and abnormalities. Elevated CK-MB levels after
abnormal ECG results (ST-elevation) in right several days indicate new
ventricular leads (V4R). infarction.
Treatment: The primary treatment is PEARLS: Therapeutic guidelines for ACS
maintenance of the RV preload--fluids, 1. ST-elevation MI: Oxygen, aspirin and beta1
NOT diuretics; normal saline but not nitrates blockers should be started ASAP
or opioids, and augmentation of for best benefits. Primary PCI within 90 min
the cardiac contractility--dopamine. of first medical contact is the goal.
Patients with predominant RVMI usually do Thrombolysis within 30 min in hospital and
not benefit from afterload reducing 6-12 hours of onset of symptoms
treatment with either an intraaortic balloon reduces mortality.
pump or vasodilating agents. It’s critical 2. Post-MI take-home medications: Aspirin
for early effective corrections to restore (or clopidogrel if aspirinintolerant),
perfusion. beta1 blockers (metoprolol), ACE-I (or ARB-
-angiotensin-R blockers
Acute Coronary Syndrome (ACS) if cough-persistent), and statins.
ACS refers to any group of symptoms 3. Glycoprotein IIb/IIIa inhibitors (abciximab,
attributed to obstruction of the tirofiban, eptifibatide):
coronary arteries. ACS usually occurs as a Useful in ACS with ST depression (non-ST
result of one of three conditions: elevation MI) and patients to
ST-elevation myocardial infarction (30%), undergo angioplasty and stenting. tPA is
non-ST-elevation myocardial beneficial only with ST elevation MI.
infarction (25%), or unstable angina (38%). Heparin is best for non-ST elevation MI.
It is difficult to determine the precise 4. In non-ST elevation ACS, if patient is
etiology from its history and P/E alone. not better (persistent pain, S3 gallop,
The risk factors for ACS are the same as for worse ECG changes, and rising troponin
CAD. levels) after using all given
PEARLS: Diagnostic guidelines for ACS
1. The most common symptom prompting
medications, urgent angiography and possibly
diagnosis of ACS is pressure-like
angioplasty (PCI) should be
chest pain (> 30 min with infarction), often
performed.
radiating to the left arm, and
5. The No.1 common cause of death is
associated with anxiety, nausea, and
ventricular arrhythmia—tachycardia
diaphoresis.
and fibrillation. Immediate electrical
2. Lab diagnosis: (1) ECG is abnormal
cardioversion or defibrillation should be
immediately at onset of typical chest
ready to perform.
pain. ST-T elevation progresses to Q-waves or Contraindications: Do not use any
left branch block over up to 7 “prophylactic antiarrhythmic
days. (2) Abnormal myoglobin starts 1-4 hrs medications for ventricular tachycardia or
after chest pain and lasts 1-2 days; fibrillation” because it increases
CK-MB starts 4-6 hrs and lasts 3 days; ventricular arrhythmia and mortality!
troponin starts 2-4 hrs and last 7-10 days. Do not use nitrates and sildenafil together to
Troponin cannot distinguish a reinfarction avoid severe vascular
occurring several days after the first complications.

10
Post-MI impotence: Erection problem is 4. Precipitating factors: Increased
mostly due to anxiety. Sexual activity salt/fluid intake, excess exertion or
can be recovered when patient is emotion,
asymptomatic. arrhythmias, systemic infection, renal
failure, cardiac depressants (disopyramide
HEART FAILURE (HF) or beta-R blockers), or inappropriate
Heart failure (HF), usually referred to as decrease of a drug dose.
“Congestive heart failure” (CHF), is a Pathogenesis
common clinical syndrome resulting from any Heart failure is pathologically
structural or functional cardiac characterized by reduced cardiac output with
disorder that impairs the ability of the or
ventricle to fill with or eject blood and without pooling of blood in venous
the circulation (venous stasis).
heart to maintain an adequate output to meet 1. Systolic dysfunction: Impaired myocardial
the body’s circulatory and contractility (as from CAD) with
metabolic demands under normal conditions. decreased ejection fraction (EF).
HF due to left ventricular 2. Diastolic dysfunction: Pressure-volume
dysfunction is newly categorized according overload (as from hypertension,
to left ventricular ejection fraction valvular dysfunction) with normal or
(LVEF) into HF with reduced ejection supranormal EF, but decreased total
fraction (with LVEF ≤ 40 percent, cardiac output.
known as HFrEF; also referred to as systolic
HF) and HF with preserved ejection 3. Frank-Starling law: (1) In a normal
fraction (with LVEF>40 percent; known as heart, increasing preload results in greater
HFpEF; also referred to as diastolic contractility. (2) A failing heart with
HF). CHF is characterized by insufficient exertion generates relatively less
oxygen delivery to tissues contractility and significant symptoms.
accompanied by the accumulation of fluid in Essentials of diagnosis
the lungs and lower body. CHF is 1. Manifestations of left-sided (LV) heart
mostly from systolic dysfunction, with a low failure: Exertional dyspnea,
ejection fraction and dilation of the orthopnea, PND (paroxysmal nocturnal
heart. dyspnea), cough with frothy or pink
Etiology sputum, crackles/rales at lung bases,
1. The most common cause is primarily cardiac enlargement and displaced PMI (to
abnormal myocardiocytes from MI or the left), S3 (ventricular) gallop, and S4
ischemia. murmur.
2. The second common cause is abnormal 2. Manifestations of right-sided (RV) heart
myocardiocytes due to prolonged failure: Elevated venous pressure,
exposure to a hemodynamic burden (primary JVD (jugular vein distention), ankle edema,
hypertension, aortic regurgitation, hepatosplenomegaly, and hepatojugular
or pulmonary hypertension), myocarditis, reflux, ascites, right ventricular heave,
cardiomyopathy, alcohol, drug and nocturia due to elevated legs
toxicity, or infiltrative disease and venous return during sleep.
(sarcoidosis, amyloid, or hemochromatosis). 3. Chest X-ray (CXR) reveals signs of CHF--
3. Structural abnormalities: CAD, valvular fluid retention based on vascular
diseases, congenital heart diseases, congestion (prominent interstitial
pericardial diseases, outflow obstruction, markings), cardiac enlargement, Kerley’s B
high-output heart failure lines, and pleural effusion. See Image 14.
(hyperthyroidism, pregnancy, Vit-B1 4. Two-dimensional echocardiography will
deficiency). show the wall motion abnormality

11
and low ejection fraction (usually <40%). the ejection fraction < 40%, better with
Multigated (MUGA) scan or spironolactone. Angiotensin II receptor
radionuclide ventriculography can also blockers (losartan, valsartan, irbesartan,
measure EF. or candesartan) can be chosen if ACE-I
Differential diagnosis is intolerable. Beta -blockers are helpful
Cardiac dyspnea: in HF with systolic dysfunction but not
Typical presentations are sudden onset of with diastolic dysfunction or acute HF; be
dyspnea without sputum or history of cautious when the EF is very low.
pulmonary diseases or smoking. Pulmonary 2. Improvement of cardiac performance:
function test (PFT) shows restrictive (1) Positive inotropic agents: They are
ventilatory defect. indicated with reduced EF or if the
Pulmonary dyspnea: above treatment fails. These can improve
More gradual onset (except with infections, symptoms shortly but have not
pneumothorax, or asthma), dyspnea shown to reduce mortality.
at night, often associated with sputum, Cardiac glycosides—digitalis: Best for severe
CHF with atrial fibrillation or
history of COPD, smoking, or noxious
EF < 40% despite treatment with ACE-I and
inhalants. PFT usually shows obstructive or
beta-blockers, etc. Mechanisms
restrictive ventilatory defect.
are inhibition of Na-K-ATPase, resulting in
Treatment
intracellular Na and Ca increase, and
Treatment of HF with reduced EF includes
thus inotropic effect. Potassium competes
management of contributing
with digitalis’ binding sites and so
conditions such as hypertension, ischemic
hyper-K will decrease its activity, whereas
heart disease, valvular heart disease,
Hypo-K results in higher activity or
diabetes, thyroid dysfunction, and
toxicity (same as by quinidine, Ca-blockers,
infection, as well as lifestyle
thiazides, furosemide &
modifications
bumetanide). Spironolactone can decrease
including smoking cessation, restriction of
renal clearance of digitalis.
alcohol and salt ingestion, and weight
Cholestyramine and colestipol can interfere
control/reduction.
with its GI absorption.
1. Reduction of cardiac workload:
Digitalis intoxication:
(1) Non-medical treatment and correction of
Nausea, vomiting, blurred vision with yellow
reversible causes: Reduce physical
halo, and arrhythmias (#1 is
paroxysmal atrial tachycardia with A-V
and emotional stress and salt/fluid intake;
block). Treatment: (a) Mild—stop the
remove exacerbating factors
medication and correct hypo-K. (b) Severe A-
(infection, anemia, heat, obesity, etc);
V block—choose atropine. (c) If
treat vascular lesions, myocardial
digitalis level is > 10 mg/L, use anti-
ischemia, uncontrolled hypertension, etc.
digitalis fragment. (d) Anti-arrhythmia: use
(2) Medications: A diuretic and vasodilator
lidocaine or phenytoin.
are first-line therapies (especially
Other positive inotropic medicines:
with hypertension) to reduce preload,
Intermittent use of dobutamine or
afterload, and mortality. Hydralazine
plus spironolactone are recommended for phosphodiesterase inhibitor (amrinone,
chronic HF. Loop diuretics and milrinone).
nitrides are the best initial medicines for acute (2) Correction of underlying arrhythmias and
left HF with pulmonary deficiencies.
edema, plus O2-PEEP, morphine, 3. Surgical correction: It may be considered
nitroglycerin and dobutamine. ACE inhibitors for heart failure (HF) due to valve
(ACE-I) are more effective in decreasing lesions or diastolic dysfunction. Heart
preload (mainly) and afterload when transplantation can be the last option for

12
 fasting cholesterol is >200, patient is
an appropriate patient with end-stage HF. treated based on LDL-C levels and risk
factors.
HYPERLIPIDEMIA 3. Patients with LDL of 130-159 mg/dL or
It’s defined as total cholesterol (Chol) triglyceride >250 mg/mL with 0-1
level > 200 mg/dL, including increased risk factor: borderline risk, treat with an
LDL-C and triglyceride (TG). It’s a major initial 3-month plan of diet modification
risk factor for CAD as with age (M and exercise.
> 45, F > 55), smoking, diabetes, 4. Indications for starting lipid-lowering
hypertension, low HDL (< 40), and history of medications:
CAD, etc. Common causes include obesity, (1) Any CAD history (or an equivalent) and
diabetes, alcoholism, OCP use, LDL >130 mg/dL (some suggest
familial hypercholesterol, hypothyroidism, >100 mg/dL); CAD equivalents: peripheral
hepatic disease, nephritic syndrome, artery disease, diabetes mellitus,
Cushing syndrome, and high-dose diuretic
use. carotid or the aortic artery disease;
Essentials of diagnosis (2) No CAD but LDL >160 mg/dL with two risk
1. Most patients have no specific symptoms factors;
and are found out by lipid screening (3) LDL >190 mg/dL alone. The goal is to
testing: total serum cholesterol levels >200 keep LDL lower than 100 mg/dL
mg/dL on two different occasions (or <70 with CAD plus a CAD equivalent). HMG
is diagnostic. CoA reductase inhibitor
2. LDL-C >130 or HDL-C <40 (mg/dL) is (statins) is the first choice of drugs. Add
diagnostic of dyslipidemia, niacin if the statin alone is ineffective.
regardless of the total cholesterol levels. Use the fibrates if both are intolerable.
3. TG >100 mg/dL (in children <10 years) and (4) For future treatment with LDL >100
>130 mg/dL (children >10 mg/dL and a history of CAD or CAD
years). equivalent (diabetes, disease of the aota,
4. Hypercholesterolemia appearance: carotid or peripheral artery), lipidlowering
xanthelasmas--yellowish adipose drugs are started to lower LDL to less than
deposit around the eyelids, etc. 100 mg/dL.
Screening, prevention and treatment (5) If HDL is low (and TG is high): Treat
Cholesterol (Chol) monitoring is highly
with niacin or gemfibrozil.
important—it’s the best screening
PEARLS--Table 2-4: Summary of Lipid-
test and preventive measure for coronary
lowering Medications
artery disease (CAD). It should be
started at age 35 in males and 45 in females
for most people (5-10 years
earlier if with risk factors for CAD), then
every 5 years if results are normal, or
evaluation for 10-year risks for CAD.
Treatment is based on risk factors and
LDL-C levels mainly, not on a low HDL or high
triglyceride (TG) itself.
1. The best initial screening test is total
fasting cholesterol levels. If fasting is
not practical, non-fasting total cholesterol
and HDL can be the screening.
2. If total fasting cholesterol is <200 and
no risk factors, retest in 5 years. If the SYSTEMIC HYPERTENSION

13
Systemic hypertension (HTN) is defined as birth weight).
a state of repeatedly elevated blood 4. Defect in natriuresis: Causing insensitive
pressure (BP) ≥ 140/90 mmHg on three natriuresis and thus salt-sensitive
occasions. Both systolic and diastolic hypertension.
pressures are important in determination of 5. Risk factors: About 50% of risk is due to
the circulation and functions of lifestyle factors--high-Na diet,
organs. However, systolic hypertension is a smoking, obesity, hyperlipidemia, limited
more powerful predictor of exercise, and sleep apnea; and the
cardiovascular conditions and clinical other half is due to genetics (family
prognosis, especially after 50 y/a when history of hypertension or heart disease),
diastolic pressure tends to decrease. By advanced age, male gender, the Black
etiology it is generally classified into population, etc.
primary and secondary hypertension.
Conceptions: Essentials of diagnosis
Hypertension by office-based blood 1. Common symptoms include headaches
pressure: (typically back of the head and in the
Normal blood pressure: systolic < 120 mmHg morning), lightheadedness, vertigo,
and diastolic < 80 mmHg; tinnitus, altered vision, and fainting
Prehypertension: systolic 120 to 139 mmHg or episodes. Other possible findings include
diastolic 80 to 89 mmHg; systolic click, S4 and/or loud S2,
Stage 1 hypertension: systolic 140 to 159 increased cardiac size, and retinopathies
mmHg or diastolic 90 to 99 mmHg; (copper wires, artery-vein nicking,
Stage 2 hypertension: systolic ≥ 160 mmHg or papilledema, cotton wool spots, hard
diastolic ≥ 100 mmHg; exudates, and flame hemorrhage; Image
Isolated systolic hypertension: BP ≥ 140/< 146).
90 mmHg; 2. Diagnosis: Mainly based on physical
Isolated diastolic hypertension: BP < 140/ examination (P/E) results: BP
≥ 90 mmHg. ≥ 140/90 mmHg (either one) on > 3 tests
I. Primary (Essential) Hypertension separated by > 30 min.
It’s the hypertension without a definable Classification and management are summarized
cause and it accounts for > 95% of all in Table 2-5.
hypertensive cases. It’s mostly resulted Treatment
from either high peripheral resistance or Antihypertensive therapy (BP control) has
elevated cardiac output. Hypertension is a shown to produce a nearly 50%
multi-factorial disease; more factors relative risk (RR) reduction in the
imply more risk. Screening for hypertension incidence of heart failure, a 30-40% RR
may be started at age 18 or in the reduction in stroke, and a 20-25% RR
middle age. The interval depends on reduction in myocardial infarction. Major
individual’s conditions. mechanisms include decreases in blood
Etiology volume, cardiac output, and arterial
1. Sympathetic nervous system tension. Goals of treatment are BP < 140/90
hyperactivity: Mostly in young patients. mmHg for most patients and <
2. Renin-angiotensin system hyperactivity: 130/80 mmHg for patients with chronic
Causing increased intracellular Na- diseases (diabetes, kidney disease, etc).
Ca-water retention; mostly in young White 1. Nonpharmacologic therapy (lifestyle
and Asian populations. modification):
3. Abnormal cardiovascular or renal This includes exercise, weight reduction,
development: Abnormal aortic elasticity alcohol restriction, and Dietary
or reduced micro-vascular network and Approaches to Stop Hypertension (DASH) diet
nephrons numbers (associated with low – low in salt, fat, sugar, and red

14
meats, and high in vegetables, fruits, whole
grains, poultry, fish, and nuts.
2. Pharmacologic therapy:
Initial monotherapy in uncomplicated
hypertension — One of the following four
main classes:
(1) Thiazide diuretics (chlorthalidone or
indapamide); (2) Long-acting calcium
channel blockers (such as amlodipine); (3)
ACE inhibitors; (4) Angiotensin II
receptor blockers (ARBs).
The major exceptions to modest and gradual
BP lowering over the first 24
hours are:
(1) The acute phase of an ischemic stroke--
The blood pressure is usually not
lowered unless it is ≥ 185/110 mmHg
(candidates for reperfusion therapy) or ≥
220/120 mmHg (non-candidates for reperfusion
therapy.
(2) Acute aortic dissection--The systolic BP
should be rapidly lowered to a
target of 100-120 mmHg (to be attained in 20
min).
The best selection of antihypertensive is (estrogen) use is the #1 cause in > 35 year-
based on specific conditions of the old, obese females. Other substances
include cocaine, NSAIDs, cyclosporine,
patient, as summarized in Table 2-6. tacrolimus, decongestants, etc.
Table 2-5: Blood Pressure Classification and
Correction of the original cause is the
effective therapy.
2. Renal disease: Any primary renal
disease can cause hypertension and is
generally the most common cause of
secondary hypertension. ACE-I can
alleviate the hypertension and slow the
progression of renal diseases.
3. Renal artery stenosis (RAS): (1) More
than 90% of cases are caused by
artherosclerotic renal ischemia in
patients > age 45. (2) Fibromuscular
dysplasia
(FMD) is a rare cause of RAS and mostly
in women < age 50.
II. Secondary Hypertension Diagnosis: (1) Clues: 1) Resistant
It’s defined as hypertension due to an hypertension with onset < 30 or > 50 y/a
identifiable organic cause; some can be without obesity, family history, and other
surgically correctable. risk factors; 2) Refractory
Etiology, diagnosis, and treatment
hypertension or newly onset hypertension
1. Substance effects: Alcohol is the #1
that is resistant to > 3 medications; 3)
cause in young male patients; long OCP

15
There are abdominal/renal artery bruits; 4) also helpful in diagnosis.
There is a history of atherosclerosis; Treatment: Use both alpha- and beta-R
5) There is abrupt deterioration of the blockers followed by surgical resection.
renal function (elevation in the serum 6. PKD (Polycystic kidney disease):
creatinine) after the use of ACE-I; 6) Hypertension along with a family history
Malignant or accelerated hypertension with of PKD, flank mass, and enlarged kidneys.
signs of end-organ damage. Treatment: Symptomatic and conservative.
(2) The best screening method is a renal 7. Coarctation of the aorta: Hypertension
radionuclide flow scan with captopril with upper limbs only; right arm BP
(showing high rennin and aldosterone). (3) > left arm BP, with lower limb muscle
Confirmation is done by hypotrophy. Treatment options depend on
angiography or by renal vein renin ratio. conditions of the patient (age, severity,
Treatment: (1) For atherosclerotic RAS: 1) etc.).
Medical therapy first for most
unilateral and bilateral RAS; 2) If PULMONARY HEART DISEASE (COR
hypertension still cannot be controlled, PULMONALE)
percutaneous angioplasty with stenting is “Cor pulmonale” is defined as the right
recommended. Renal artery ventricular systolic and diastolic failure
revascularization is reserved for those with resulting from pulmonary disease and the
complex anatomic lesions or failed attendant hypoxia. It is most
stenting procedures. (2) For fibromuscular commonly cause by COPD or idiopathic
dysplasia: percutaneous angioplasty pulmonary fibrosis. Less frequent
causes include pneumoconiosis, idiopathic
with stent is usually curative. (3) ACE-I pulmonary hypertension,
can be a drug adjunct in elder patients kyphoscoliosis, etc.
for one-side stenosis, but contraindicated Essentials of diagnosis
in bilateral renal stenosis because it can 1. Common symptoms include dyspnea on
accelerate high-renin and renal failure by exertion, fatigue, lethargy, exertional
preferential vessel-dilation of the syncope, and exertional angina. P/E may show
efferent artery. signs of pulmonary hypertension
4. Primary hypoaldosteronism: (increased S2 +/- splitting) and right-sided
Characterized by hyper-aldosterone and heart failure (hypotension, jugular
hyper- vein distention, and foot edema).
Na, hypo-renin and hypo-K. 2. Lab tests: (1) CXR typically shows
(1) Conn syndrome: 70% unilateral, mostly enlarged central pulmonary arteries and
from an adrenal adenoma. right ventricle, and a loss of retrosternal
(2) Cushing syndrome: with above features, air space. (2) Echocardiography usually
along with decreased glucose demonstrates increased right ventricular
intolerance, central obesity, hirsutism, pressure with hypertrophy. (3) Right
striae, “buffalo hump” (Image 38). heart catheterization is the gold standard for
(3) Iatrogenesis: Usually due to chronic the diagnosis.
Treatment
steroid use.
The three major physiological goals include
5. Pheochromocytoma: Triad — episodic
reduction of right ventricular
hypertension, headache, and
afterload, decrease of right ventricular
diaphoresis. Other features: young age,
pressure, and improvement of right
paroxysmal symptoms, history of
ventricular contractility.
endocrine tumors (MEN IIA and IIB syndrome).
1. Treat underlying cause.
Diagnosis: By elevated 24-hour urinary
2. Treat “right heart failure” (see the
catecholamines or VMA. CT or MRI is
same topic in this chapter)

16
3. Treat “ chronic respiratory failure ” tenderness, Osler’s nodes (tender nodules on
(see Chapter 3). finger and toe pads, Image 39),
Janeway’s spots (small peripheral
ENDOCARDIAL AND VALVULAR HEART hemorrhages, splinter hemorrhages
DISEASES (subungual petechiae, Images 40), and
Roth’s spots (retinal hemorrhages,
Endocarditis Image 147).
It mostly refers to infectious endocarditis, 3. Lab diagnosis:
defined as an infection and (1) Culture and sensitivity (C/S) is the
inflammation of the endocardial surface of best means of diagnosis (> 95%
the heart (mainly a heart valve), sensitivity)--three sets of blood cultures
usually secondary to bacterial or other separated in time (> 1 hour) and
infections. Risk factors include history location, usually showing multiple positive
of rheumatic or valvular heart diseases same pathogens. ESR is increased.
(mitral or aorta valve), IV drug use ALWAYS take blood for C/S before use of
(tricuspid valve is the No.1 affected), antibiotics!
immunosuppression, recent dental or
surgical procedure, indwelling intravenous (2) Echocardiogram: Positive finding of
catheter, and use of prosthetic heart vegetations is confirmative, but
valve. negative result does not rule it out. In
PEARLS--Table 2-7: Summary of Etiologies of general, transthoracic echocardiography
Endocarditis (TTE) is the first diagnostic test for
Classification / Causes suspected patients. Transesophageal
Acute endocarditis: IV drug use--#1 by Staph- echocardiography (TEE) has higher
aureus; others: Pneumococcus,
sensitivity than TTE and is better for
Gonococcus.
detection of cardiac complications such as
Subacute endocarditis: Dental procedures,
valve damage, kids: #1 by Strep-mutans abscess, leaflet perforation, and
(viridans); pseudoaneurysm. CXR may reveal septic emboli
prosthetic heart valve: #1 by Staph- in right-sided endocarditis.
epidermidis; others: Enterococcus, Fungi. Other complications for diagnostic
consideration
Culture-negative infection: H. parainfluenza,
Conjunctival petechiae; brain lesion
Cardiobacterium, Actinobacillus, Eikenella,
(mycotic aneurism); renal lesions
Kingella.
(hematuria, glomerulonephritis);
Autoimmune: Rheumatic fever; SLE—Libman-
splenomegaly; septic emboli to the lungs.
Sacks: autoantibody to valve causing warty
Treatment
vegetations.
1. Empirical long-term antibiotic therapy for
Drug-induced & toxic: Cytotoxics
28 days, initially covering Gram+
(doxorubicin), emetine, catecholamines,
bacteria then adjusting by the
etc; causing inflammation &
culture/sensitivity results. For Staph-
necrosis.
aureus: 14-
Marantic: Carcinoma—Metastasis seeds valves,
day regimen of nafcillin or vancomycin; if >
causing emboli and infarcts; poor prognosis.
60 y/a, add ampicillin (for
Essentials of diagnosis
Listeria). Choose ceftriaxone (4 weeks) for
1. Variable systemic symptoms: High fever
pneumococcus or Strepviridians/
with shaking chills (for acute cases) bovis, ampicillin + gentamicin for
or low-grade fever (for the subacute); Enterococci, and choose
cough, dyspnea, anorexia, general amphotericin and valve replacement for
weakness, and weight loss. fungal endocarditis.
2. P/E results and complications: Include 2. For culture-negative/difficult endocarditis,
murmur (new or changed), joint possible organisms include

17
Hemophilus aphrophilus or parainfluenza, Rheumatic Fever
Actinobacillus, Cardiobacterium, It is a systemic nonsuppurative immune
Eikenella, and Kingella. Ceftriaxone is the process following a pharyngeal
drug of choice for this group of streptococcal (Strep) infection. Most cases
organisms. of acute rheumatic fever occur in
3. Always monitor for relapse, add an children 5 to 15 years of age, more common
aminoglycoside, and extend the duration in developing countries. Rheumatic
of antibiotics. Perform valve replacement fever may result in valvular heart disease,
for recurrent Gram- or fungus with mitral stenosis as the most
infections, worsening valvular function common one. Other valvular lesions may also
(especially aorta or mitral stenosis), occur.
systemic emboli or conduction disturbances. Major criteria for diagnosis:
For endocardial abscess or 1. Migratory arthritis (multiple large
vegetation, perform immediate debridement. joints); 2. Carditis and valvulitis; 3. CNS
If valve replacement is intolerable, involvement (e.g., Sydenham chorea); 4.
valvulotomy can be chosen. Erythema marginatum; 5.
4. Indications for antibiotic prophylaxis: Subcutaneous nodules.
(1) High-risk procedures (dental procedures, Minor criteria:
bronchoscopy with biopsy, GI or Fever, polyarthralgias, increased ESR,
GU procedures with ongoing infection, prolonged PR interval in ECG, prior
procedures on infected skin or history of rheumatic fever, preceding Strep
musculoskeletal tissue, or intracardiac infection, or ASO (+).
procedures) with high risk diseases Diagnosis of acute rheumatic fever—Jones
Criteria:
(unrepaired valvular diseases, cyanotic
Two major criteria, or one major plus two
diseases, history of previous infectious minor criteria.
endocarditis, and severe rheumatic heart Differential diagnosis
diseases). Endocarditis, RA, SLE, osteomyelitis, SCD,
(2) High-risk procedures (as above) with and Lyme disease.
moderate-risk diseases: ASD, Treatment
PDA, VSD, bicuspid aorta, aorta coarctation, 1. Treat acute rheumatic fever with bed rest
rheumatic valvular diseases, MVP, and NSAIDs—aspirin has been
myxoma, hypertrophic cardiomyopathy. replaced by the stronger naproxen with less
5. Selection of preventive antibiotics: adverse effects. Treat streptococcal
pharyngitis with long-acting penicillin G
(1) For oral, respiratory, esophageal benzathine or erythromycin.
procedures: the first choice of Antibiotics help prevent cardiac
medicines is amoxicillin, ampicillin or complications from Strep infection, but not
erythromycin (if not available, choose post-Strep nephropathy.
clindamycin, azithromycin, clarithromycin). 2. Treat the valvular pathology of rheumatic
(2) For GU, GI procedures: choose heart disease.
ampicillin + gentamycin; if not available, 3. Indications for antibiotic prophylaxis:
choose vancomycin + gentamycin. During dental, GI, or genitourinary
Note: Low-moderate risks that do not need procedures. Long-acting benzathine PG is
antibiotics: used as the secondary prevention of
GI-endoscopy, esophageal intubation (but recurrent rheumatic fever.
needed for esophageal dilation),
vaginal delivery, hysterectomy, and valvular VALVULAR HEART DISEASES
diseases with arterial
catheterization. Mitral Stenosis (MS)

18
MS is the most common cardiac lesion pulmonary artery; Image 14).
caused by rheumatic fever, consisting Treatment
of thickened mitral valve leaflets, fused 1. Medical therapy: Diuretics and salt-
commissures, and chordae tendineae. It restricted diet for pulmonary congestion
can lead to left-to-right ventricular and edema; digitalis (to control the
failure. It affects more women than man. ventricle rate) and long-term oral
Pathogenesis anticoagulants in patients with atrial
Mitral valve stenosis impairs left ventricle fibrillation. For rheumatic MS,
filling. Increased left atrial pressure percutaneous mitral balloon valvotomy
causes pulmonary congestion. Forward cardiac (PMBV) is preferred to surgery.
output is reduced, secondary
pulmonary vasoconstriction occurs, and 2. Surgery: For most congenital MS and cases
eventually right ventricular failure with persistent symptomatic
results. despite above treatment, commissurotomy,
Essentials of diagnosis balloon valvuloplasty, or valve
1. Most patients present with gradual left- replacement (more severe) usually produces
heart-failure symptoms: Exertional good results. Open mitral
dyspnea, orthopnea, PND (paroxysmal commissurotomy or valve replacement is often
nocturnal dyspnea), fatigue, wasting, and indicated for patients with
hemoptysis. Later on with progression, contraindications for valvuloplasty.
systemic embolism, hoarseness (due to Pulmonary hypertension is not a
enlarged left atrium), and right-heart- contraindication for the surgery.
failure signs (hepatomegaly, ascites, and
peripheral edema). History of pregnancy or Mitral Regurgitation (MR)
immigration associated It is insufficiency of the mitral valve that
cardiomyopathy may be a precipitator and causes backflow of blood from the left
clue of diagnosis. ventricle (LV) into the left atrium. It may
2. P/E usually finds typical low-pitched remain asymptomatic for many years
apical diastolic rumble, loud S1, (or for life) or cause left-sided heart
opening snap following S2, decreased pulse failure.
pressure, pulmonary rales (edema), Etiology
and sternal lift. Murmurs are increased by Common causes are rheumatic fever and
squatting or leg lifting, and decreased dilation of the left ventricle, which lead
by standing or Valsalva’s maneuver. to abnormalities of the mitral leaflets,
3. Lab diagnosis: annulus, and chordae tendineae. Male >
(1) Echocardiography/Doppler confirms the female.
diagnosis: It usually shows 1. Acute MR: #1 cause is post-MI papillary
thickening of mitral valve leaflets, a muscle dysfunction. Others
reduction in the excursion and area of the include chordae tendinae rupture, papillary
valve leaflets, and increased left atrial muscle rupture, endocarditis, and
size. trauma.
(2) ECG: May show signs of RV hypertrophy, 2. Chronic MR: #1 cause is mitral valve
left and right atrial abnormalities, prolapse. Others include rheumatic
and atrial fibrillation. heart disease (scarring and retraction of
(3) CXR: May show a large left atrium valve and leaflets), papillary muscle
(early), straightening of the left heart dysfunction, endocarditis, calcification of
border, elevation of the left main-stem the mitral valve annulus, hypertrophic
bronchus, and signs of pulmonary cardiomyopathy, congenital endocardial
hypertension (Kerley’s B lines, increased cushion defect, and severe left ventricle
vascular markings, and a large (LV) dilatation.

19
Pathogenesis Hypertrophic obstructive cardiomyopathy
A portion of the left-Ventr stroke volume is (HOC):
pumped backward into the left It is also called idiopathic hypertrophic
atrium resulting in increased left atrial subaortic stenosis. (1) Typical systolic
pressure and decreased forward cardiac outflow murmur, increased with standing,
output. There are also overloaded volume, Valsalva maneuver, or amyl nitrite,
increased preload, and decreased and decreased with squatting, leg raising,
afterload involved, which help compensate or hand grip. The mechanisms are
for the regurgitation by increasing similar with other types of valvular heart
ejection fraction. Nevertheless, prolonged diseases. (2) Typical echocardiogram
compensation leads to left-Ventr features of HOC.
dysfunction. Papillary muscle rupture with acute MR:
Essentials of diagnosis It occurs in about 1% of MI, mostly 3-5 days
1. Rapid LV failure signs are common: after infarction, more frequent in
Dyspnea, orthopnea, PND, fatigue, inferior-posterior infarcts (posterior
hemoptysis, etc. With severe MR, right-heart papillary more frequent). Murmur is usually
failure signs can co-exist loud and holosystolic radiating to the aorta
(pulmonary HTN, edema, and ascites). area. Echocardiogram usually
2. P/E: Hyperdynamic and displaced (downward shows flair or prolapsing leaflet. Doppler
and to the left) LV impulse. shows systolic regurgitant jet into left
Carotid upstroke is decreased and brisky. atrium.
There is a holosystolic apical Ventricular septal rupture:
murmur radiating to the axilla, often with a It occurs in 1-2% of all infarcts; peak
thrill, S3 with a soft S1 and widely incidence is 3-5 days after infarction, more
split S2, and JVD. Murmurs are increased by frequent in anterior infarcts. Murmur is loud
squatting or leg lifting, and and holosystolic with
decreased by standing or Valsalva maneuver. widespread radiation and 50% have palpable
3. Lab diagnosis: precordial thrill. Echocardiogram
shows the septum defect. Doppler can show
(1) Echocardiography/Doppler confirms the the transseptal left-to-right shunt.
diagnosis (and helps decide when Ventricular septal defect (VSD):
to operate): Will show abnormal movement of There is a holosystolic shrill murmur over
the mitral valve, and left atrial lower left sternal border, with
and ventricular enlargement if chronic. strong and split S2. Radionuclide studies or
(2) Others: ECG will show signs of LV Doppler will confirm left-to-right
hypertrophy and left atrial enlargement. shunt. Cardiac catheterization reveals
CXR may show Cardiac enlargement, vascular oxygen jump from right atrium to
congestion if with CHF. ventricle.
Catheterization may show a large “v” wave
Treatment
(due to volume overload on the left
1. Medications for chronic MR: Use
atrium).
Differential diagnosis digitalis, diuretics, artery dilators (ACE-
Mitral valve prolapse: See below. I),
Aortic stenosis: and warfarin to relieve symptoms by
Systolic ejection murmur radiating to the increasing forward cardiac output and
carotid/aorta area; delayed carotid reducing afterload and pulmonary veinous
upstroke; soft, single or absent S2; hypertension.
echocardiogram features of aortic stenosis 2. Surgery: Mitral valve repair (EF<30%) or
and calcification of the aortic valve. replacement (>30%) is the

20
choice. Indications include persistent Systolic murmur does not peak late; carotids
symptoms with severe MR despite optimal do not have delayed upstrokes; no
medications. The surgery carries more risk LV hypertrophy by ECG; no significant
for patients with chronic heart failure results by cardiac catheterization.
and should be avoided if the heart failure Echocardiogram may show normal or reduced
is severe enough. With mild aortic valve leaflets.
symptoms, surgery should be deferred because Pulmonary stenosis: See below.
the condition may remain stable Treatment
for years. 1. Patients should be given antibiotic
prophylaxis for endocarditis.
Aortic Stenosis (AS) 2. Surgery is indicated for symptoms and
Etiology and pathogenesis asymptomatic patients with severe
The No.1 cause is calcification and fibrosis of aortic stenosis.
a normally aged aortic valve (1) Valve replacement is the mainstay of
(elderly patients) or congenitally bicuspid surgery for most AS cases when the
aortic valve. Rheumatic valvular valve area is ≤ 1.0 cm2. Surgery can also
diseases may affect both the mitral valve improve the symptoms if angina,
and aortic valve. The stroke volume is
normal until late stages, with elevated LV syncope, and left ventricle dysfunction are
end-diastolic pressure and gradual present, although the operative
pulmonary congestion. LV hypertrophy and mortality is higher.
high intramyocardial wall tension (2) Balloon valvuloplasty is preferred if the
will increase the oxygen demands and valve stenosis is very severe
decrease diastolic coronary blood flow, (usually the area is ≥ 1.0 cm2) without
resulting in the onset of angina. calcification, in selected children, or if
Essentials of diagnosis the patient is too weak (with high risk) to
1. Most patients are asymptomatic until tolerate surgery.
middle or old age. Typical
manifestations include angina, syncope, and Aortic Regurgitation (AR)
dyspnea from CHF; harsh systolic Etiology and pathogenesis
ejection murmur radiating to the carotid or Acute AR: Causes include endocarditis, aortic
aorta area; delayed carotid dissection, traumatic leaflet
upstroke; S4 gallop, decreased A2, and rupture, hypertension, rupture of a
aortic ejection click. Murmurs are congenitally fenestrated cusp, iatrogenic
increased by squatting or leg lifting, and valve injury during surgery, and prosthetic
decreased by standing or Valsalva aortic valve thrombosis or disk
maneuver. escape.
2. Lab diagnosis: Chronic AR: Rheumatic fever is the #1 cause,
(1) Echocardiography or Doppler is the best with the mitral valve frequently
means of diagnosis and shows affected together. Others include aortic
thick aortic valve leaflets with decreased root dilation, congenital bicuspid aortic
excursion and LV hypertrophy. valve, calcific valve disease, hypertension,
(2) CXR may present with calcification, and Marfan syndrome.
cardiomegaly, and pulmonary AR results in a volume overload of the left
congestion. ventricle (LV), which compensates
Differential diagnosis by increasing its end-diastolic volume
HOC, MR: See above. (Frank-Starling law). The LV overdilation
Elderly aortic valve sclerosis (without causes overstretch of the myofibrils and
stenosis):
decreased contractility. In

21
chronic AR the pulse, pressure and systolic 1. For acute AR: Emergent aortic valve
pressure are increased, and the replacement or repair is performed.
diastolic pressure is decreased due to the If there is any delay in surgery, temporary
AR and large stroke volume. stabilization is attempted in ICU using
Essentials of diagnosis IV vasodilators (nitroprusside) to reduce
1. Severe acute AR commonly presents with afterload, and possibly adding inotropic
sudden cardiovascular collapse and agents (dobutamine, digitalis) to lower LV
pulmonary edema. end-diastolic pressure.
2. Chronic AR is usually asymptomatic until 2. Medical treatment for chronic AR:
middle age to present with graduate Reduction in preload and afterload --
left-sided heart failure. History of Valsalva maneuver, salt restriction,
dyspnea, typical diastolic decrescendo diuresis; nitroglycerin, vasodilators.
murmur at the cardiac apex, with systolic 3. For severe chronic AR: Aortic valve
flow murmur; Austin-Flint murmur replacement is indicated when
(a mid-diastolic rumbling murmur at the symptoms worsen or ejection fraction (EF) is
apex); S3 in early LV decompensation; decreased, and endocarditis
systolic and diastolic thrill/murmur heard prophylaxis is needed.
over the femoral artery. Murmurs are
increased by squatting or leg lifting, and Tricuspid Stenosis (TS)
decreased by standing or Valsalva Tricuspid stenosis is characterized by
maneuver. right-sided heart failure. It is usually
3. Chronic AR is staged as “A, B, and C” rheumatic in origin but carcinoid syndrome
according to valve anatomy and and tricuspid surgery are the more
hemodynamics, hemodynamic consequences, and common causes in the US. Female has the
symptoms. predominance. TS rarely occurs as an
2. Lab diagnosis: isolated lesion; it is often accompanied by
(1) Echocardiogram is diagnostic by showing tricuspid regurgitation and associated
a dilated LV and aorta, volume with mitral valve disease or/and the aortic
overload, and fluttering of anterior mitral valve.
valve leaflet. Essentials of diagnosis
(2) Others: ECG may show LV hypertrophy, 1. History of rheumatic fever, carcinoid
often with narrow deep Q-waves in disease, or cardiac surgery.
left precordial leads. CXR may show LV and 2. Typical manifestations of right heart
aortic dilation. failure: JVD, hepatomegaly, ascites,
Differential diagnosis and dependent edema; giant a wave in the JVP
Mitral stenosis and aortic stenosis: See (pressure); typical diastolic
above. rumble along the lower left sternal border
Pulmonic regurgitation: (mimicking mitral stenosis)
Typical diastolic murmur, usually with 3. Lab tests: ECG reveals RA enlargement
pulmonary hypertension due to mitral without atrial fibrillation. CXR shows
stenosis or with right-to-left cardiac marked cardiomegaly with a normal PA size.
shunt; compensated RV enlargement; no Echocardiography/Doppler
peripheral manifestations of AR. confirms diagnosis. Mean valve gradient > 5
Patent ductus arteriosus: mm Hg by Echo indicates severe
Typical continued systole and diastole stenosis.
murmur with peak at S2, whereas systolic Treatment
ejection murmur with AR usually peaks in 1. Diuretics are the main treatment to
midsystole. Angiography or cardiac reduce symptoms associated with the fluid
catheterisation shows L-to-R shunt. congestion.
Treatment

22
2. When the stenosis progresses causing
right heart failure, the most effective Pulmonary Stenosis (PS)
and definitive treatment is tricuspid valve It is the stenosis of the pulmonary valve or
repair or replacement (by a RV infundibulum, which increases
bioprosthetic valve) or balloon valvotomy. the resistance to RV outflow and RV
The tricuspid valve replacement is pressure, and decrease pulmonary blood
often combined with mitral valve replacement flow. It is often congenital and associated
if it’s also with stenosis. with other cardiac lesions.
Essentials of diagnosis
Tricuspid Regurgitation (TR) 1. Asymptomatic until the lesion is at
TR frequently occurs in patients with least moderate. Severe cases may present
pulmonary or cardiac disease with pressure with right-sided heart failure.
or volume overload on the right ventricle 2. There is a high-pitched systolic ejection
(RV dilation). Thus TR is commonly murmur maximal in the second left
functional. interspace and radiating to the shoulder. P2
Etiology includes pulmonary hypertension, is delayed and soft or absent.
MI, endocarditis, left heart failure, Ejection click often exists and decreases
RV dysplasia, sarcoidosis, Ebstein anomaly, with inspiration –the only right heart
and tricuspid valve prolapse or auscultatory sign with this condition (all
injury. These can all result in RV dilation other ejection clicks increase with
and TR, which in turn worsens the inspiration).
severity of the regurgitation. 3. CXR and ECG may reveal enlarged right
Essentials of diagnosis ventricle. 2-D echocardiogram
1. History of a pulmonary or cardiac confirms diagnosis by visualization of the
disease/lesion (as above) associated with valve stenosis.
RV dilation. 4. Patients with peak pulmonic valve
2. Similar manifestations of right heart gradients > 60 mmHg or mean of 40
failure as in tricuspid stenosis: JVD, mmHg by echocardiography or Doppler should
hepatomegaly, ascites, and dependent edema; undergo intervention regardless
a holosystolic TR murmur may of symptoms.
be audible along left sternal border, which Treatment
increases with inspiration. A domed pulmonary valve stenosis (most
3. Lab tests: CXR may show an enlarged RA cases) can be treated by balloon
and pleural effusion. valvuloplasty, while a dysplastic pulmonary
Echocardiography confirms the diagnosis valve (with severe stenosis) usually
with severity of the TR (low- or requires surgery. Dynamic RV outflow
high-pressure TR), the RV size and RV obstruction may occur after surgical or
function. percutaneous treatment and tends to regress
Treatment with time.
1. Mild TR is common and usually well
tolerated, and can be managed with Pulmonic Regurgitation (PR)
diuretics (to reduce the fluid congestion). It is pulmonary valve regurgitation. Most
2. Surgery: When feasible, tricuspid valve cases are due to pulmonary
repair is generally preferred to valve hypertension (high-pressure PR) or surgical
replacement. However, repair is associated injury for treating RV outflow
with significant risk of recurrent TR. obstruction (low-pressure PR). Other “low-
Definitive treatment requires elimination of pressure” causes include a dilated
the cause of the TR. With pulmonary annulus, congenital
progressive right heart failure, tricuspid bicuspid/dysplastic pulmonary valve, or
valve replacement is indicated. carcinoid plaque.

23
Essentials of diagnosis Ventricular septal defect, Atrial septal
1. In high-pressure PR, the pulmonary defect, and Patent ductus. Oxygenated
diastolic murmur is readily audible, which blood from the lungs is shunted back into
is increased with inspiration and diminishes the pulmonary circulation, with late
with the Valsalva maneuver. In lowpressure and mild cyanosis.
PR, there are rarely murmur and abnormal 2. By stenosis: Aortic stenosis, pulmonic
echocardiography/Doppler stenosis, and coarctation.
findings.
2. Echocardiography is definitive in Mitral valve prolapse (MVP)
diagnosis of high-pressure PR but may be MVP (syndrome) is the most common
less definitive in low-pressure PR. congenital valvular abnormality, or is
Treatment considered a normal anatomic variant
1. In high-pressure PR: Target primary occurring in 2-5% of the population and
cause—pulmonary hypertension because more commonly in young females and
it’s poorly tolerated by the patient. associated with connective tissue diseases
2. Low-pressure PR is usually tolerated by (e.g., Marfan syndrome, Ehlers-Danlos
the patient and rarely requires syndrome, skeletal abnormalities, or
treatment. Exercise and pregnancy are not idiopathic).
interdicted. Essentials of diagnosis
3. Pulmonary valve replacement is indicated Usually it remains asymptomatic but may show
with (1) symptomatic, severe non-anginal chest pain (#1
PR; (2) right ventricular enlargement and/or symptom, due to arrhythmias),
dysfunction; (3) PR plus palpitations, exercise intolerance,
progressive tricuspid valve regurgitation. dizziness,
Untreated severe PR will result in right syncope, and panic and anxiety disorders.
ventricular enlargement, systolic Auscultation typically finds middleto-
dysfunction, arrhythmia, and death. late systolic click and a late systolic murmur
at the cardiac apex, worse
CONGENITAL HEART DISEASES with standing or Valsalva’s maneuver and
The prevalence is less than 1% among live improving with squatting or leg
births. Most diagnoses can usually be lifting.
made in early life, although initial murmurs Lab diagnosis: Echocardiography is the
and symptoms may not be best method to confirm marked
noticeable. Etiology is unknown. displacement of the mitral leaflets in
Intrauterine risk factors include teratogens systole into the left atrial side of the
(maternal alcohol and drug use), congenital annulus. Difference from MR: there is only
infections (rubella, etc), and genetic moderate systolic displacement of
diseases (Down syndrome, Marfan syndrome,
etc). the leaflets with MR.
Classification Treatment
1. By shunting: 1. Most cases are asymptomatic and not in
(1) Right to left—cyanotic (mnemonics: “4T- need of treatment. Lifestyle
blue baby”): Tetralogy of modification is benefitial, including
Fallot, Transposition of the great arteries avoidance of stimulants (caffeine) and
(TGA), Tricuspid atresia, and Truncus alcohol, reduction in stress, and moderate
arteriosus (mixing shunts). Deoxygenated exercise. Beta blockers are used with
blood is shunted into the systemic symptoms.
circulation, causing serious cyanosis. 2. Valve repairs can be performed with a
(2) Left to right—noncyanotic (Memo: “Late catheter by placing clip to tighten up
blue child”; “VAP”):

24
the valve, while surgery is rarely 1. Mostly patients are asymptomatic and
necessary. Neither is for endocarditis discovered on routine P/E. With a large
prophylaxis (even with a MR murmur). defective size, patient can present with
fatigability and frequent respiratory
Ventricular Septal Defect (VSD) infections. P/E finds RV lift, a wide, split
It’s the congenital opening between the S2 and systolic ejection murmur at
ventricles leading to left-to-right the upper left sternal border (from
shunting, the most common congenital heart increased right ventricular blood).
disease. It is often associated 2. Lab tests: Echocardiography or Doppler is
with Down syndrome, fetal alcohol diagnostic by showing blood
syndrome, cri-du-chat syndrome, Apert flow between the atria. ECG reveals right-
syndrome (cranial deformities, finger/toe axis deviation and RVH. CXR shows
fusion), and trisomies 13/18. enlarged heart size and pulmonary-vessel
Essentials of diagnosis markings.
1. Small defects are often asymptomatic but 3. A patent foramen ovale is present in 25%
with a harsh holosystolic murmur of the population but can lead to
at the lower left border, usually louder paradoxic emboli and stroke. Patient with a
than with larger defects. history of cryotogenic stroke before
2. Large defects can show dyspnea, frequent age 50 shoule be highly suspected of it.
respiratory infections, failure to Treatment
thrive, pulmonary hypertension, and CHF. The 1. Most small ASDs close spontaneously. If
holosystolic murmur is softer, there is any evidence of an RV
blowing and with a thrill, louder S2 and volume overload, a percutaneous device or
middle-diastolic murmur. surgery should be done to close it.
3. Diagnosis: Echocardiography or Doppler Surgery is usually required for closure of
confirms diagnosis by showing primum ASDs, sinus venosus defects,
blood flow between the ventricles. ECG and and coronary sinus defects.
CXR may show LVH, RVH, and 2. Antibiotic prophylaxis is required for
increased pulmonary vessel markings (CXR). ostium primum defect prior to dental
Treatment procedures.
1. Newborns are closely followed in the
first weeks of life. If it’s complicated Patent Ductus Arteriosus (PDA)
with heart failure, a diuretic, inotropic, It’s the congenital failure of the arterial
or ACE-I is used. Most small VSDs close ductus to close in the first few days of
spontaneously, and only need life, causing a left-right shunt from the aorta
echocardiography monitoring. to the pulmonary artery. Risk
2. For symptomatic VSD that fails medical factors include maternal rubella infection
treatment or has pulmonary (1st trimester), female gender, and
hypertension, surgical repair is indicated. prematurity.
3. Antibiotic prophylaxis should be given to Essentials of diagnosis
all VSD patients before dental, 1. It’s usually asymptomatic until middle
oropharyngeal, or GU procedures to prevent age or larger ductal size. Patient with a
bacterial endocarditis. large defect may present with recurrent
respiratory infection, lower extremity
Atrial Septal Defect (ASD) clubbing, and CHF.
It’s the congenital opening between the 2. Typical P/E results include: A widened
atria leading to left-right shunting. It’s pulse pressure, load S2, “continuous
also associated with Down syndrome and machinery murmur ” at the 2nd left
fetal alcohol syndrome, etc. intercostal space by the sternum, and
Essentials of diagnosis bounding peripheral pulse.

25
3. Lab tests: A color-Doppler ultrasound pulmonary congestion, and “rib notching” (due
(U/S) is helpful in showing blood flow to collateral circulation through
from the aorta into the pulmonary artery, the intercostal artery).
but the lesion is best visualized by 4. 50% of cases are associated with bicuspid
MRI, CT, or contrast angiography. With aortic valve; some with webbed
larger PDAs, echocardiography, ECG neck (Turner syndrome).
and CXR may all show left atrial/ventricular Treatment
enlargement. 1. With severe infant coarctation, PDA needs
Treatment be kept open with PGE1 for
1. For premature infants, indomethacin is survival.
used to close the PDA, unless the 2. Surgical correction or balloon
PDA is needed for survival (as in great angioplasty may be effective treatment.
artery transposition, Fallot tetralogy, Early
severe aorta-coarctation, and hypoplastic repair may lower risks of CHF and CAD.
left heart). 3. It ’ s important to have continuous
2. For most infants > 6-8 mo of age (including prevention for endocarditis and monitoring
indomethacin failure), for re-stenosis, aortic dissection, and
percutaneous PDA closure (catheter aneurysm development.
occlusion and surgical ligation) is
necessary. Tetralogy of Fallot (Fallot Tetralogy)
It consists of “VPOR”--VSD, Pulmonary
Coarctation of the Aorta (CA) artery stenosis, overriding aorta, and
It’s the constriction of an aorta portion RVH. It ’ s the most common cyanotic
leading to decreased flow below or distal congenital heart disease in children >
to it and increased flow above or proximal 24 hours of life. Early cyanosis results from
to it. Most cases are below the left right-to-left shunting across the
subclavian artery. It’s often associated VSD, and may be milder in the 1st 1-2 weeks
with Turner syndrome, Berry because of the pressure decrease in
aneurysm, bicuspid aorta valve, and male the right ventricle after birth. The
gender. cyanosis can be worse over time if the
Essentials of diagnosis pulmonary artery stenosis is severe to keep
1. Patient usually has a history of a high right-ventricle pressure. Risk
asymptomatic hypertension in childhood, and factors: Down syndrome, cri-du-chat
may presents with dyspnea on exertion, syndrome, maternal PKU, trisomy 13/18.
syncope, headache, claudication, and Essentials of diagnosis
epistaxis. 1. Gradual cyanosis, fatigability, and
2. Typical P/E signs: Systolic BP is higher dyspnea during infancy (> 24 hours after
in the arms than in the lower birth). Patients often squat (to increase
limbs; right arm BP is higher than the left arm systemic artery pressure) for
(determined by the coarctation symptomatic relief during hypoxemic
location); weak or absent femoral pulse; a episodes.
late, short systolic murmur in the left 2. Hypoxemia may cause secondary failure to
axilla; forceful apical impulses. thrive, mental status changes or
3. Lab tests: Echocardiography or Doppler CHF.
confirms the diagnosis (a gradient > 3. P/E usually reveals a systolic ejection
20 mm Hg). In older children, ECG may reveal murmur at the left upper sternal border
LVH and CXR may show (pulmonary artery stenosis), RV lift and a
“reverse 3” sign (due to pre-/post-dilation single S2, as well as signs of CHF.
of the coarctation), cardiomegaly, 4. Lab tests: Echocardiography/Doppler and
catheterisation are diagnostic.

26
Be wary if the RV is enlarged because the 2. P/E finds cyanosis, tachypnea, a single
Echo/Doppler may underestimate S2, progressive respiratory failure, or
significant pulmonic regurgitation. CXR signs of CHF.
shows a “boot-shaped” heart with 3. Diagnosis is confirmed by
decreased pulmonary-vessel markings echocardiogram. CXR may show narrow heart
(opposite to “single VSD”). ECG reveals base and absence of the main pulmonary
RVH and right-axis deviation. Arrhythmias artery segment (“egg-shaped
are common and period Holter silhouette ” ), and increased pulmonary
monitoring is recommended. Patient may be vessel markings.
risky of sudden death if the QRS Differential diagnosis
width is > 180 msec. Fallot tetralogy, large VSD, aortic
Differential diagnosis coarctation, hypoplastic left heart
TGA, large VSD, PDA, tricuspid or pulmonary syndrome.
artery stenosis, hypoplastic left Treatment
ventricle. 1. It’s necessary to administer IV PGE1 to
Treatment keep the PDA open followed by
1. Administer PGE1 to keep the PDA open immediate surgical correction (arterial or
followed by surgical correction atrial switch).
(intracardiac repair by patch closure of the 2. If immediate surgery is not possible, a
ventricular septal defect) to relieve fast balloon atrial septostomy (creating
severe pulmonary artery stenosis. An an ASD) can be done for the first few days
artificial shunt (balloon atrial septostomy) of life.
can be a temporary palliation.
2. Treat cyanosis with O2, knee-chest Persistent Truncus Arteriosus (PTA)
position, alpha-agonist, beta-R blocker, It’s the congenital separation failure of
morphine, and fluid. the great vessels that causes one single
Prognosis great vessel supplying both the systemic and
It mostly depends on the degree of pulmonary pulmonary artery beds with a
artery stenosis. Arrhythmia may mixture of oxygenated and deoxygenated
blood.
occur even after surgical repairs. Essentials of diagnosis
1. Cyanosis shortly after birth, followed by
Transposition of the Great Arteries (TGA) gradual dyspnea, easy fatigability,
It’s a congenital condition in which the failure to thrive, and CHF signs.
pulmonary and systemic circulations 2. P/E finds a harsh systolic murmur at the
exist in parallel --the aorta is connected lower left sternal border, with a loud
to the right ventricle and the pulmonary S1/S2 and systolic ejection click,
artery to the left ventricle. It’s the most cardiomegaly, CHF signs, and pounding
common cyanotic congenital heart pulses.
disease in the newborn at birth. It’s fatal 3. Lab tests: Angiocardiography is
without a PDA or VSD, and diagnostic. ECG often shows normal axis
requires immediate correction. and evidence of LVH and RVH. CXR may reveal
Risk factors: Down syndrome, cri-du-chat “boot-shaped heart”, absent
syndrome, Apert syndrome, trisomy main pulmonary artery, and a large aorta
13/18, and diabetic mothers. arching to the right.
Essentials of diagnosis Differential diagnosis
1. A severely ill, weak newborn with Fallot tetralogy, large ASD or VSD, etc.
cyanosis immediately after birth. Reverse Treatment
cyanosis may be present if aorta stenosis or Surgical repair is necessary.
coarctation coexists.

27
Tricuspid Atresia (TA) be the therapeutic choice for end-stage
It’s a congenital condition in which the disease.
tricuspid heart valve is missing or
abnormally developed. MYOCARDIAL DISEASES
Clinical features and diagnosis
Early cyanosis exists at birth, 90% with VSD, Myocarditis
and 30% with TGA. P/E It ’ s defined as the infection or
shows L2-sternal border systolic murmur and inflammation of the myocardial cells,
single S2. ECG reveals left axis leading to
deviation and CXR shows decreased reduced cardiac contractility and output.
pulmonary vessel markings. Etiology
Treatment It ’ s mostly caused by viruses (No.1 is
Initially give oxygen and IV PGE1 to keep Coxsachie B). Other causes include
ASD, VSD, or the arterial duct open bacteria (post-Strep-A rheumatic fever, Lyme
to allow survival, followed by 3-stage disease),
surgical palliation. autoimmune/autoreactive diseases (SLE,
giant cell myocarditis, eosinophilic
Hypoplastic Left Heart Syndrome myocarditis, sarcoidosis), rickettsia
It’s a congenital syndrome with left heart (Rocky Mountain spotted fever), protozoa
chamber defect, small left ventricle, (Chagas disease in South America),
stenosis of the aorta and mitral valves, medications (sulphonamides), and
ASD, pulmonary congestion, increased irradiation, etc.
right-ventricle pressure, pre-cordial Clinical features and diagnosis
hyperactivity, and loud S2, but no murmur. 1. Most patients have non-specific symptoms
Patient usually presents with cyanosis and such as fatigue, weakness, and
heart failure in the 1st week of life. fever (after an infection); some may have
Diagnosis precordial pain, arrhythmias, and signs
Echocardiography. of heart failure.
Treatment
2. Diagnosis is based on clinical
Initially give oxygen and mechanical
manifestations and combined with abnormal
ventilation and IV PGE1 to keep the
results from ECG (ST-T wave changes and
arterial duct open to allow survival,
deficient conduct), cardiac enzymes,
followed by 3-stage surgical palliation.
and ESR. Endomyocardial biopsy may be
indicated when patient shows
Kartagener Syndrome (KS) or Primary Ciliary
Dyskinesia (PCD) unexplained new-onset heart failure of less
KS is also known as immotile ciliary than two week’s duration associated
syndrome, a rare, ciliopathic, autosomal with hemodynamic compromise or new
recessive genetic disorder that causes a ventricular arrhythmias.
Treatment
defect in the action of the cilia lining the
1. Supportive and symptomatic therapy is the
respiratory tract (lower and upper, sinuses,
mainstay of treatment is the
Eustachian tube, and middle ear) and
choice for most cases. Because of the high
fallopian tube.
Typical triad: Dextrocardia, recurrent risk of arrhythmias and cardiac
sinusitis, and bronchiectasis. shock, at-risk patients should be cared in
Treatment ICU. IVIG is recommended. Antipathogen
It includes daily chest physiotherapy, and anti-arrhythmia therapy should be
antibiotics with good coverage on started if indicated, but be
Pseudomonas, and supportive pulmonary care. cautious with antiarrhythmic drugs with
Heart-lung transplantation may negative inotropic activity. If heart

28
failure develops, digitalis, ICE-I or tricuspid regurgitation may be present.
diuretics may be needed. Immunosuppressive 2. Lab diagnosis:
therapy (including steroids) is suggested (1) Echocardiogram is the #1 diagnostic
for giant cell myocarditis, sarcoidosis, means, showing dilated left ventricle,
noninfectious eosinophilic myocarditis, and decreased wall motion, mitral valve
autoreactive myocarditis. regurgitation, and increased biventricular
2. Patients should avoid NSAIDs, heavy chamber size.
alcohol consumption, and exercise, and (2) CXR: Cardiomegaly with pulmonary
should receive routine follow-up with serial congestion.
echocardiography. Most patients (3) ECG: Sinus tachycardia, arrhythmias, and
recover well. conduction disturbances.
(4) Catheterization: Dilated
hypocontractile ventricle; MR may also
Cardiomyopathies exist.
Cardiomyopathies refer to diseases of the Differential diagnosis
heart muscle itself that lead to cardiac Acute infectious myocarditis; valvular heart
dysfunctions. Current major definitions of diseases; CAD; hypertensive heart
cardiomyopathy exclude heart disease diseases.
secondary to cardiovascular causes (such as
hypertension, ischemic heart Treatment
disease, or valvular disease). According to 1. Same medications as for heart failure:
morphologic and hemodynamic digoxin, diuretics (spironolactone),
features, they can be classified as dilated Beta1-R blockers, vasodilators (ACE-I,
(#1 common), hypertrophic, angiotensin-II-R blockers), and/or
restrictive, arrhythmogenic right ventricular, antiarrhythmic drugs. Most of these
and unclassified medications can lower its mortality. If the
cardiomyopathies. QRS is wide, a biventricular pacemaker can
I. Dilated (Congestive) Cardiomyopathy
be placed to improve both
It is the cardiac weakening and enlargement
symptoms and survival.
due to decreased systolic function
2. Remove the offending agent if possible.
(mainly LV).
Preventive warfarin is recommended
Etiology
due to the high risk of pulmonary and
No.1--idiopathic; No.2--toxic: alcohol. Others:
systemic embolism.
myocarditis (virus, parasite,
3. Dilated cardiomyopathy is the No.1 reason
mycobacteria, or Ricketts), peripartum,
for heart transplantation if other
neuromuscular diseases (muscular
therapies fail.
dystrophy, myotonic dystrophy),
chemotherapy toxicity (doxorubicin,
Peripartum Cardiomyopathy (PPCM)
cyclophosphamide, vincristine), Vit-B1
PPCM is a rare form of dilated
deficiency, connective tissue diseases
cardiomyopathy, usually presenting between
(rheumatoid arthritis, SLE, polyarteritis),
the
glycogen storage diseases, toxins
last month of pregnancy and up to five months
(cobalt, lead, arsenic), metabolic diseases
postpartum. PPCM also involves
(thyroid disease, pheochromocytoma,
a decrease in the LV ejection fraction (EF),
chronic Hypo-P2, Hypo-K, Hypo-Ca, uremia).
which is often limited and reversible.
Essentials of diagnosis
Some patients develop heart failure.
1. Symptoms and signs of left and right
The etiology is unknown but may be
ventricular failure (due to decreased
associated with viruses, autoimmunity (by
myocardial contractility, EF, and cardiac
antibodies against myocardium), and toxins.
output); S3, S4 and murmur of mitral or
Essentials of diagnosis

29
1. Manifestations may be caused by gradual occur without other symptoms, particularly
heart failure and pulmonary edema: among athletes.
Orthopnea, dyspnea, pitting edema, cough, 3. HOCM has more severe symptoms
palpitations, chest pain, frequent (dyspnea, angina, and syncope), which
night-time urination, and excessive weight are worsened by factors causing increased
gain during the last month of heart rate (exercise, dehydration,
pregnancy. and diuretics) and decreased LV chamber
2. PPCM is a diagnosis of exclusion--no size (ACEI, ARBs, digoxin,
prior history of heart disease and no hydralazine, Valsalva, suddenly standing,
other known causes of heart failure. etc.).
Echocardiogram is used to both diagnose 4. Lab diagnosis:
and monitor the effectiveness of treatment. (1) Echocardiogram is the #1 means of
Medications of treatment are the same as for diagnosis, showing hypertrophy,
dilated cardiomyopathy. systolic anterior motion of mitral valve and
Treatment midsystolic closure of aortic valve.
1. Medications of treatment are the same as (2) CXR: Shows left ventricular predominance
for dilated cardiomyopathy. and dilated left atrium. ECG is
2. Prognosis is mostly good if treated in usually normal.
time. Repeat pregnancy in a patient with (3) Catheterization: Shows hypertrophy with
PPCM will provoke significant antibody vigorous systolic function and
production against the myocardium, cavity obliteration.
with worse prognosis. Treatment
II. Hypertrophic Cardiomyopathy (HCM) 1. All patients should avoid strenuous
Etiology and pathogenesis exercise. Symptomatic patients are best
It ’ s an autosomal dominant hereditary treated with beta-blockers, which help reduce
disease with abnormal chromosome myocardial contractility and
14. Pathologically it’s characteristic by improve diastolic filling and symptoms. A
marked myocardial hypertrophy (mostly Ca-blocker or disopyramide may help
left ventricle) and disproportionate improve symptoms if patient is unresponsive
thickening of the inter-ventr septum. There to beta-blockers. Implantable
are normal or increased chamber size, defibrillator is used in HOCM patient with
cardiac output and stroke volume, syncope. Diuretics may be helpful
increased ejection fraction and decreased in HCM but are contraindicated in HOCM.
diastolic compliance. Obstruction may 2. Surgery: Septal ablation, myomectomy, or
develop between inter-ventr septum and mitral valve replacement can be a
septal leaflet of mitral valve, causing good therapy for severe cases.
hypertrophic obstructive cardiomyopathy III. Restrictive Cardiomyopathy
(HOCM). MR may be present. Etiology and pathogenesis
Essentials of diagnosis It’s less common. It is the infiltration of
1. Family history of the disease or of “ the myocardium results in rigid
sudden death” (especially HOCM). ventricular walls, reduced ventricular
2. Clinical manifestations include compliance, normal to decreased cardiac
palpitations, dyspnea, angina, presyncope, output and stroke volume, and decreased
and diastolic compliance. The ventricular
syncope; bifid carotid pulse, palpable/loud pressure tracing resembles those as recorded
S4 gallop, systolic ejection murmur in constrictive pericarditis, with an
and thrill, possible MR murmur, and large early diastolic dip-and-plateau pattern.
jugular “a” wave. Sudden death may Causes and subtypes
1. Infiltrative: Sarcoidosis, amyloidosis,
hemochromatosis, or neoplasia.

30
2. Endocardial fibroelastosis: Cardiac Recommended management includes
dilatation with diffuse endocardial antiarrhythmic medicines and implanted
hyperplasia. cardioverter-defibrillator (ICD) for
3. Endomyocardial fibrosis: Fibrous secondary prevention of sudden cardiac
endocardial lesions of the ventricular death in patients with sustained ventricular
tachycardia (VT) or ventricular
inflow portion. fibrillation (VF) and for primary prevention
4. Loffler endocarditis: Dense endocardial in selected high-risk patients.
fibrosis with overlying thrombosis. V. Unclassified Cardiomyopathies
5. Becker disease: Cardiac dilatation with These include cardiomyopathies that do not
fibrosis of the papillary muscles and readily fit into any of the above
subendocardium associated with necrosis and
mural thrombosis. It has been phenotypic categories, such as LV
mostly found in South Africa. noncompaction, stress-induced (takotsubo)
Essentials of diagnosis cardiomyopathy, and cirrhotic
1. Weakness, dyspnea, exercise intolerance; cardiomyopathy.
JVD, edema, hepatomegaly, ascites, Left ventricular noncompaction
S4 and S3 gallop, and Kussmaul sign. Also called isolated ventricular
2. Lab diagnosis: noncompaction, it is a rare cardiomyopathy
(1) Echocardiogram may show characteristic with an altered myocardial wall due to
myocardial texture in amyloidosis intrauterine arrest of compaction of the
with thickened pericardium, and increased loose interwoven meshwork. When the LV
left and right atrium sizes. noncompaction is severe, it may
(2) CXR: Mild cardiomegaly and pulmonary cause ventricular arrhythmias, heart
congestion. failure, and thromboembolism. Treatment
(3) ECG: Low voltage, conduction varies with the clinical manifestations.
abnormalities, and Q-waves. Stress-induced cardiomyopathy
(4) Catheterization: Typical “square root Also called apical ballooning syndrome,
sign”; M-shaped atrial wave form, broken heart syndrome, and takotsubo
elevated left- or right-sided filling cardiomyopathy, it is characterized by
pressures. transient systolic dysfunction of the apical
Treatment and/or mid segments of the LV that is often
There is no effective treatment but provoked by stress. This disorder is
symptomatic and supportive care. Try to generally transient and only in need of
treat supportive therapy.
underlying disorders (amyloidosis, Cirrhotic cardiomyopathy
sarcoidosis, hemochromatosis, etc.). It will While alcoholic cardiomyopathy is one cause
eventually result in death from heart of heart disease in patients with
failure or arrhythmias. Heart cirrhosis, cirrhosis is found to be
transplantation associated with myocardial dysfunction
may be the last resort. independent of alcohol exposure. The
IV. Arrhythmogenic Right Ventricular pathophysiology remains unclear.
Cardiomyopathy (ARVC) Treatment is focused on supportive care of
This is a genetically determined myocardial the cardiomyopathy and cirrhosis,
disease characterized by and their complications.
ventricular arrhythmias, fibrous
cardiomyopathy, and RV dysfunctions. PERICARDIAL DISEASES
Gene mutations may be involved in the
pathology. Acute Pericarditis
Treatment

31
It’s the inflammation of the pericardial 1. A transudate indicates pericardial
lining around the heart. injury, and an exudate reflects
Etiology inflammation.
1. Idiopathic (Dressler syndrome, postviral 2. Serosanguineous pericardial fluid is
infections, etc). typically from TB or neoplasm.
2. Infection (viruses, bacteria, fungi, 3. Hemopericardium may be from aortic
toxoplasmosis), connective tissue and aneurysm rupture, aortic dissection,
autoimmune diseases (vasculitis, SLE, post-MI Ventr-rupture, severe chest trauma,
rheumatoid arthritis, scleroderma, or bleeding by coagulopathy. When
sarcoidosis), metabolic diseases, neoplasm, fluid accumulates rapidly, it compresses the
trauma, and drug reactions (lupus heart and inhibits cardiac filling,
syndrome), radiation, etc. resulting in life-threatening cardiac
Essentials of diagnosis tamponade.
1. Patient typically has positional, Lab diagnosis
substernal chest pain that is worsened by 1. Echocardiography is the most sensitive
lying down, coughing and deep inspiration, and specific means of diagnosis,
and relieved by sitting up and which can display > 20mL of fluid).
leaning forward. Fever and nonproductive 2. CXR can show a “ water-bottle ”
cough may be present. configuration of the cardiac silhouette
2. Characteristic pericardial friction rub (>250
(diagnostic of pericarditis)—a highpitched, mL of fluid).
scratchy sound, usually transient and best Treatment
heard as the patient sits 1. If the effusion is small and clinically
forward at forced-end expiration. The insignificant, a repeated
ventricular systole sound is present more echocardiography in 1-2 weeks is appropriate
consistently. following etiologic therapies.
3. ECG: It’s helpful in diagnosis, usually 2. Patients with a significant pericardial
revealing a diffuse ST-elevation with effusion and evidence of hemodynamic
upright T waves and PR depression at the compromise (ie, cardiac tamponade) should
onset of chest pain, but no Q-wave undergo urgent drainage of the
(different from MI). Echocardiography is often effusion.
normal but abnormal if
pericarditis with effusion is present. Cardiac Tamponade
Treatment It’s a life-threatening condition in which
For most acute idiopathic or viral a pericardial effusion has developed so
pericarditis, colchicine plus NSAIDs rapidly and largely that it compresses the
(ibuprofen) is recommended. For post-MI and heart, leading to decreased ventricular
most pericarditis, aspirin plus filling and cardiac output.
colchicine is the mainstay of therapy. Treat Etiology: It’s mostly the same as for acute
underlying cause if known pericarditis and pericardial effusion.
(including drainage of a pericardial Essentials of diagnosis
effusion). Most cases are self-limited, 1. Patient typically has dyspnea, orthopnea,
resolving in 2-6 weeks. pulsus paradoxus (marked
weakening pulses during inspiration),
Pericardial Effusion hypotension, JVD with clear lung, and
Etiology and pathogenesis decreased heart sounds. Paradoxical pulse is
Fluid may accumulate in the pericardial not diagnostic of cardiac tamponade
cavity in pericarditis and other forms of and can occur in severe CHF, chronic
pericardial diseases. pulmonary disease, acute asthma, and
certain hypovolemic shock.

32
2. Echocardiogram (must be performed) and root” sign. The end-diastolic pressures in
cardiac catheterisation will confirm all four chambers and the pulmonary
tamponade and equal pressure of the left and arteries are about the same.
right atrium. Differential diagnosis
Treatment Restrictive and constrictive cardiomyopathy:
Perform emergent pericardiocentesis and Left ventricular ejection fraction is more
drainage to save life! If symptoms likely to be decreased in patients with
persist, perform subxiphoid surgical restrictive cardiomyopathy. CT can
drainage. effectively demonstrate the thickened
pericardium. Differentiation can be
Constrictive Pericarditis difficult sometimes.
Etiology and pathogenesis Treatment
Constrictive Pericarditis is diffuse fibrous Most newly diagnosed cases are initially
scarring and thickening of the treated conservatively with Narestriction,
pericardium in reaction to prior mild diuretics, NSAIDs, steroids, or/and
inflammation, leading to obliteration of the antibiotics, etc.
pericardial cavity and reduced Pericardiectomy or resection may be
distensibility of the cardiac chambers and performed in chronic, severe cases as the
diastolic filling. Cardiac output is limited definitive therapy. However, the mortality
and filling pressures are increased to rate is high.
match the external constrictive force placed VASCULAR DISEASES
on the heart by the pericardium. The Aortic Dissection
heart size is usually normal. The cause in It’s a transverse tear in the intima of the
most cases is idiopathic. Others aorta, leading blood into the media and
include viruses, TB, chronic pericardial creating a false lumen and a hematoma that
effusion, uremia, connective tissue propagates longitudinally.
diseases, heart surgery, and thoracic Longstanding hypertension is the major risk
radiation. factor. Other predisposing factors
Essentials of diagnosis include trauma, connective tissue diseases
1. Most patients appear very ill. Initial (Marfan and Ehlers-Danlos
manifestations are secondary to increased syndrome), bicuspid aortic valve,
systemic venous pressure — edema, ascites, coarctation of the aorta, etc. The most
hepatomegaly, JVD, and Kussmaul common site of origin is above the aortic
sign. Later, patients usually have dyspnea valve and distal to the left subclavian
on exertion and orthopnea. P/E usually artery. It’s more common in 50-60 y/o men.
finds distant heart sounds and “pericardial Essentials of diagnosis
knock”, which can be confused with 1. Sudden, severe, tearing pain in the
an S3 gallop. anterior chest (in ascending aorta
2. Lab diagnosis: dissection) or interscapular back pain
(1) ECG: Low-voltage and non-specific T-wave (descending aorta dissection),
changes. Atrial fibrillation may accompanied with diaphoresis.
occur in some patients. 2. P/E results are usually asymmetric pulses
(2) Chest CT or MRI scan: May show thickened and BP (difference > 20 mmHg)
pericardium, and pericardial between the left and right arm. BP is mostly
calcifications (TB). hypertensive. If it is hypotensive,
(3) Cardiac catheterization: A marked ‘y’ pericardial tamponade (with pulsus
descent with the right atrial pressure paradoxus, decreased heart sound and JVD),
tracing. Left and right ventricular pressure acute MI from coronary ischemia (see “MI”
tracings may show a typical “square for the symptoms), or hypovolemic

33
shock from blood loss should be suspected. AA (AAA) secondary to atherosclerosis or
If the aorta valve is involved, signs hypertension, which causes local
and murmur of aorta regurgitation may exist. aorta wall weakness and dilation. Other
Neurologic signs may exist if causes include high cholesterol, family
carotid artery is obstructed. history, smoking, trauma, infection (No.1 is
3. Lab diagnosis: syphilis), congenital weakness
(1) CXR shows widened mediastinum (Marfan syndrome), or elder men. Most AAAs
(usually >8 mm), cardiomegaly, or new occur between the renal arteries
pleural effusion. and iliac bifurcation. The incidence
(2) CT angiography is the most accurate increases with age and more common in
means of diagnosis to show details of men.
the dissection, if the patient is stable and Essentials of diagnosis
confirmation is required. 1. It’s usually asymptomatic and discovered
(3) TEE is also sensitive and specific, incidentally on P/E or radiologic
showing details of the thoracic aorta, imaging.
coronary arteries, aortic valve, and 2. It can be symptomatic, with “sense of
possible pericardial effusion. Serum fullness”, severe or boring low back
biomarker D-dimer (increase) indicates pain or flank pain radiating into the testis
recent or ongoing intravascular blood or leg. It may be confused with other
coagulation and possible acute aortic back/flank pain. P/E mostly reveals a
dissection. pulsatile abdominal mass or abdominal
4. There are two classification systems: bruit +/- tenderness. Symptoms suggest
(1) Stanford system: Ascending aorta expansion and impending rupture.
dissection is Type A, and all others are 3. Ruptured AA: Acute, severe chest or
Type B. abdominal pain, hypotension, shock, or
(2) DeBakey system: Type I --dissection MI. For an AAA rupture, Grey Turner’s sign
involving both the ascending and (ecchymoses on back and flanks,
descending aorta; Type II --confined to the Image 41) and Cullen’s sign (ecchymoses
ascending aorta; Type III --confined around umbilicus) may be seen.
to the descending aorta. 4. Lab diagnosis:
Differential diagnosis (1) Ultrasound is usually the initial
Myocardial ischemia, pericarditis, diagnostic tool.
pulmonary embolus, aortic (2) CT or aortography with contrast is the
regurgitation/aneurysm without dissection, most accurate and confirmative
musculoskeletal pain, pleuritis, and means of diagnosis.
(3) Plain X-ray often picks up an AA
digestive disorders. accidentally with aortic wall calcification.
Treatment Treatment
1. For the life-threatening ascending aorta 1. Symptomatic patients should be
dissection, an emergent surgical hospitalized and prepared for surgical
repair is indicated. fixing.
2. For descending aorta dissection, BP and Ruptured AA or impending rupture requires IV
heart rate monitoring and control are fluid and packed RBC followed
needed. IV beta-blockers are given to reduce by an emergent operation to save life.
cardiac rate and force, and 2. Asymptomatic, < 5 cm AA may be monitored
nitroprusside-Na to lower BP below 120 mmHg. for size changes and BP control
(using beta blockers) as an outpatient; > 5
Aortic Aneurisms (AA) cm (ascending) or > 6 cm
AA is a localized dilation of the aorta, and (descending) AA should be electively
the most common one is abdominal repaired (by surgical resection with

34
grafting). CVS status evaluation is required 1. Most patients with claudication will
prior to surgery. improve with smoking cessation (highly
important), diet modification, and gradual
exercise program (mainly targeting
Peripheral Vascular Diseases (PVD) lipid reduction), as well as control of the
It refers to reduction or occlusion of the underlying disease.
blood supply to the extremities caused Aspirin/clopidogrel and thromboxane inhibitor
by atherosclerotic plaques (#1), thrombosis, also help reduce incidences.
embolism, or trauma. Chronic, 2. Surgical treatment: Indications include
insufficient blood supply to the affected rest pain, ischemic ulcerations (tissue
area leads to ischemic pain and mostly necrosis), and severe symptoms refractory to
affects the lower extremity, which is called conservative therapies that affect
intermittent claudication. Acute quality of life or work. Usually angioplasty
ischemic symptoms are usually caused by (with patient’s saphenous vein),
embolism from the heart. Symptoms stenting, or bypass grafting can be chosen.
depend on the vessel and extent involved. If the limb can’t be saved by
Essentials of diagnosis angioplasty, amputation is the last option.
1. Most patients have risk factors and history
of atherosclerosis
(hyperlipidemia, hypertension, smoking, Deep Venous Thrombosis (DVT)
diabetes, lack of exercise), CAD, and See “RESPIRATORY DISEASES: PE-DVT”.
intermittent claudication. The claudication Virchow’s triad factors for venous
can be relieved by exercise cessation thrombosis:
or worsened by obstruction, leading to rest Stasis, endothelial injury, and
pain. hypercoagulable states. See Images 42 for
2. P/E are typically “5Ps”—“Pain, Pallor, DVT.
Pulseless, Paresthesia, Paralysis”.
Others are cool skin and hair loss. ABI Eisenmenger Syndrome (ES)
(ankle-brachial index) measures the ES is a disorder of gradual right-to-left
ankle-brachial systolic BP and provides shunt, the triad of systemic-to-pulmonary
objective evidence of obstruction. When shunt, pulmonary hypertension, and cyanosis.
ABI is < 0.4, ischemia is severe enough to It usually develops in a patient
cause rest pain. with a ventricular septal defect and
3. Two common types: significant left-to-right shunting that
(1) Femoropopliteal obstruction: With calf eventually leads to pulmonary hypertension.
claudication but no pulses below Severe pulmonary hypertension
the femoral artery. eventually reverses the shunting to “right-
(2) Aortoiliac obstruction: Associated with to-left”.
Leriche syndrome —decreased Diagnosis: P/E generally finds central
femoral pulses, buttock claudication, and cyanosis, digital clubbing, RV impulse,
male impotence. and a load/palpable P2 sound. The prognosis
4. Lab diagnosis: is better than those with pulmonary
(1) Initial ultrasound may show decreased hypertension who have similar hemodynamics.
blood flow due to the obstruction. Echocardiography is
(2) The best diagnostic means is confirmative.
angiography, which will demonstrate the Management: Most evaluations and treatment
area of ES should be performed in a
and severity of stenosis necessary for center with expertise in managing cardiac
surgical consideration. catheterization, the congenital heart
Treatment

35
disease, and pulmonary hypertension. Heart J. Ventr-pacemaker
or/and lung transplantation may be K. Asynchrony-e-cardioversion
the last treatment option for severe ES. L. t-PA
M. Metoprolol
VASCULITIS SYNDROMES N. Heparin
See Chapter 8: DISEASES OF THE O. Warfarin
MUSCULOSKELETAL SYSTEM AND P. Morphine
CONNECTIVE TISSUE. 3. A 55 y/o man complains of periodic
palpitations and lightheadedness after an
Chapter 2: High-yield Questions (HYQ) acute MI two weeks ago.
1. A 50 y/o man presents with chest pain, P/E finds stable vital signs. ECG reveals
shortness of breath (SOB), palpitations, and wide QRS and inverted T waves in V5-6 leads.
ankle edema for the Other findings are
past month (mo), with worsening of symptoms unremarkable.
over 2 days. Physical examination (P/E) 4. A 55 y/o man with chronic heart disease
finds tachycardia, complains of occasional palpitations,
normal BP, JVD, and lower limb edema. ECG lightheadedness, and
(EKG) reveals an atrial rate of 380/min, syncope. P/E finds a fast pulse with stable
irregular QRS, and vital signs. ECG reveals an atrial rate of
no clear P-wave with baseline. What’s the 300/min and a
most appropriate next step of treatment ventricular rate of 150/min. QRS waves are
(Tx)? regular and P-waves are in a “sawtooth”
A. Aspirin pattern.
B. Warfarin for 3 weeks 5. A 55 y/o man complains of frequent
C. Metoprolol palpitations, lightheadedness, and syncope
D. Digoxin after an acute MI 2
E. Electric cardioversion (50-100j) weeks ago. He is brought to the ER after a
2. A week later, the above patient complains syncopal episode. P/E results: HR = 120/min;
of seeing flying objects and feeling extreme BP = 90/50
weak. Lab tests
reveal hypo-K and hypo-Mg. ECG reveals mmHg. ECG reveals a ventricular rate of
atrial tachycardia and A-V block, with a 130/min, wide bizarre QRS with regular
ventricular rate of rhythms and A-V
50/min. The best immediate Tx is dissociation.
A. atropine 6. A 50 y/o female complains of frequent
B. KCl and MgCl2 palpitations, lightheadedness, and syncope
C. electric pacing for the past 3 days. She
D. anti-digitalis fragment has a 5-year history of chronic
E. digoxin cessation cardiomyopathy. P/E finds HR = 130/min, BP
3-9: Match the following clinical scenarios = 90/60 mmHg. ECG reveals
with the best initial treatment. ventricular rate = 150/min, short PR
A. Observation intervals and wide, slurred QRS (delta
B. Atropine wave).
C. Lidocaine 7. A 35 y/o female complains of frequent
D. Verapamil palpitations, dizziness, and syncope for the
E. Propranolol past 3 days. She has a
F. Procainamide congenital hearing deficit. P/E finds HR =
G. Digoxin 120/min, BP = 90/60 mmHg. Serum K and Mg
H. Aspirin levels are low.
I. Electrocardioversion

36
ECG reveals ventricular rate = 130/min, QRS and Kerley ’ s B lines. Echocardiogram
rotations, and prolonged QT. reveals EF = 45%. Serum glucose is 150 mg/dL.
8. A 50 y/o man with SOB and crushing chest What’s the most
pain for 3 hours is brought to the ER. He appropriate medicine to use now?
has a history of A. Metoprolol
hypertension, smoking, and alcohol use for B. Thiazides
the past 10 years. P/E finds a weak pulse of C. altace (ramipril)
120/min, labored D. Diltiazem
breathing at 24/min, BP of 90/60 mmHg, and E. Doxazosin
an S3 gallop. ECG shows ST-elevation in 12. The above patient obtains general relief
leads II, III, and of symptoms and stable BP of 145/95 mmHg
aVF. Lab tests show normal CBC, CK-MB, after taking
troponin, LDH, and CXR. ramipril for 5 days, but now has oliguria,
9. The above patient ’ s symptoms were persistent dry cough and a skin rash. P/E
relieved within 1 week of Tx. One month later finds a smoothly
he presents with enlarged prostate and urinary bladder. Urine
milder chest pain. P/E finds normal (Nl) analysis reveals mild proteinuria without
vital signs except a transient high-pitched, WBC. 300ml of urine
scratchy sound over the is drained out through a catheter. What’s
heart. ECG reveals a diffuse ST-elevation the best next drug for replacement?
without Q-waves. A. Metoprolol
10. A 50 y/o man with a 10-year history of B. Thiazides
smoking comes to the physician for a health C. Valsartan
exam. He has no D. Diltiazem
history of CAD or other diseases except E. Doxazosin
chronic bronchitis with productive cough and
sputum. P/E finds 13. A 30 y/o man has his BP checked in your
rough respiratory sound and Nl vital signs. clinic for the first time and it is 150/95
CXR shows increased tracks in both lungs. mmHg. He denies any
CBC is Nl. His history of alcohol or drug use, or chronic
random total Chol is 220, HDL is 40, and LDL diseases. What’s the best next step of Dx?
is 140 (mg/dL). The best next step is A. Abdominal ultrasound
A. 3-month diet and exercise plan B. Abdominal CT
B. giving lovastatin C. Blood renin and aldosterone level
C. smoking cessation D. Angiography
D. giving gemfibrozil E. BP re-testing in 2 weeks
E. lipid re-testing in a month 14. The above patient comes back 2 weeks
11. A 60 y/o man with a 3-month history of later and the BP is 145/95 mmHg. He has no
dyspnea on exertion and ankle edema other symptoms
complains of orthopnea except for a periodic headache and sweating.
and coughing up pink sputum for 1 hour. He P/E only finds a tachycardia. What’s the
has a history of smoking, hypertension (HTN) most appropriate next
and diabetes step now?
(DM) for the past 2 years and is on “ A. Abdominal ultrasound
intermittent oral drugs” for HTN and DM. B. Abdominal CT
P/E finds HR =110/min, BP C. Blood renin and aldosterone test
=150/100 mmHg, with an S3 gallop and JVD. D. 24-hour urinary VMA test
CXR reveals pulmonary congestion, cardiac E. Alpha and beta R blocker use
enlargement,

37
15. A 65 y/o female complains of low-grade 17. A 65 y/o man complains of mild chest
fever, short of breath, cough, joint pain, pain, dyspnea on exertion, and fatigability
and generalized for the past month.
weakness for the past 3 days. She had a P/E finds normal vital signs, and a harsh
dental procedure performed a week ago. P/E systolic murmur at the apex radiating to the
finds rough systolic aorta area without late
murmur at the apex, tender nodules on peak and carotid upstrokes. ECG and CXR
fingers, and subungual petechiae. ESR is results are normal. Echocardiography reveals
increased. CXR shows reduced aortic
increased vessel markings in the lungs. valve leaflets.
Echocardiography and ECG results are 18. A 60 y/o female presents with an abrupt
unremarkable. Blood worsening of her chest pain and dyspnea 5
samples are taken for culture and days after being
sensitivity (C/S). The best empirical treated for an acute MI. P/E finds
antibiotics to start on this patient is tachypnea, tachycardia, hypotension, and
A. vancomycin diffuse pulmonary rales. A loud
B. nafcillin + gentamicin holosystolic murmur is heard at the apex
C. vancomycin + gentamicin radiating to the left axilla. CXR shows an
D. vancomycin + ampicillin enlarged left atrium and
E. nafcillin + gentamicin + ampicillin increased pulmonary infiltrates.
16. Ten days later, the above patient is Echocardiography shows prolapsing leaflet.
found with (+) culture for Staph-aureus and 19. A 30 y/o female complains of
a Gram- rod, persistent lightheadedness, palpitations, and mild
fever, increased BUN and creatinine, and chest pain for the past 2 days. P/E
signs of progressive CHF. What ’ s the finds normal vital signs, and a thin woman
strongest indication for with long limbs. Auscultation reveals mid-
valve replacement surgery for this patient? systolic click and a
A. Staph-aureus culture (+) after antibiotic late systolic murmur at the cardiac apex,
Tx getting worse with Valsalva and improving
B. Persistent fever after antibiotic Tx with squatting.
C. Gram- rod culture (+) after antibiotic Tx Echocardiography confirms the diagnosis.
D. Progressive CHF 20. A 30 y/o man presents with fatigue,
E. Increased BUN and creatinine periodic chest pain, and dyspnea on exertion
17-22: Match the following clinical for the past 3 days.
scenarios with the best treatment. P/E finds a systolic murmur on the left
A. Conservative Tx sternal border between the 3-4th rib without
B. Valve commissurotomy radiation, increased with
C. Valve replacement Valsalva maneuver, and decreased with
D. Valvuloplasty squatting and handgrip. Echocardiography
E. Diuretic confirms the diagnosis.
F. Surgical repair 21. A 55 y/o man has been treated for an
acute MI (anterior infarct) for the past 3
G. Beta-R blocker days, and has an abrupt
H. Antibiotic worsening of chest pain. P/E finds
I. Warfarin tachypnea, tachycardia, hypotension, and a
J. Digitalis loud holosystolic murmur at
K. Vascular dilator the cardiac apex with widespread radiation.
Echocardiography confirms the diagnosis.

38
22. A 40 y/o female has fever, skin rash and 25. A 60 y/o man presents with boring low
migrant polyarthritis for a week followed by back pain radiating down to the legs for the
gradual dyspnea, past 2 days. He has
orthopnea, and fatigability. P/E shows mild a 3-year history of hyperlipidemia and is on
tachycardia and fever, normal BP, lovastatin. P/E finds normal vital signs and
erythematous skin nodules, a pulsatile
and a low-pitched apical diastolic rumble abdominal mass with bruits without
with loud S1. CXR shows mild increase in tenderness. Blood tests reveal mild
pulmonary vessel hyperlipidemia. Urine analysis
markings. CXR shows mild increase in (U/A) is normal. What ’ s the most
pulmonary vessel markings. CBC is normal. appropriate next step?
ESR is increased. A. Abdominal ultrasound
Aspirin has been used. She is scheduled for B. Abdominal CT
an echocardiography and arterial C. Angiography
catheterization for diagnosis. D. Abdominal X-ray
23. A 25 y/o man comes for a health check- E. Abdominal MRI
up due to prolonged palpitations, chest 26. A 50 y/o man presents with a month
pain, and dyspnea after history of exercise intolerance and leg pain
an exercise program. P/E finds a man who after more than 30 min
generally looks in good health with normal walking. Symptoms are alleviated with rest.
vital signs, bifid He has no history of alcohol use, smoking,
carotid pulse, and a systolic murmur with drug use, or recent
thrill at the cardiac apex. What’s the most health evaluation. His wife complains of “
important next step for too little sexual life.” His family life is
diagnosis? otherwise fine. P/E finds
A. Echocardiography normal vital signs, pale and hairless legs,
B. ECG and weak femoral and ankle pulses.
C. CXR Ultrasound of the lower body
D. Cardiac catheterization is unremarkable. What’s the most likely
E. Obtaining detailed history cause of his sexual problem?
24. A 50 y/o female presents with A. Psycho-social factors
progressive weakness, dyspnea, and exercise B. Atherosclerosis
intolerance for the past 3 C. Thrombosis
days. P/E finds normal BP, JVD, S4 and S3 D. Embolism
gallop, and ankle edema. ECG reveals low E. Trauma
voltage, A-V block, 27. A 25 y/o man is brought to the ER by his
and Q-waves. Echocardiography reveals friend for pressing chest pain and short of
thickened pericardium. CXR shows mild breath for a few
cardiomegaly and hours after a fight. He denies any history
pulmonary congestion. Among the following of smoking, alcohol, drug use, and medical
causes, which one is the LEAST likely? problems. He briefly
A. Alcohol and lipids mentions that both his parents have “heart
B. Hemochromatosis disease.” P/E results: pale, dyspneic, and
C. Sarcoidosis diaphoretic; BP =
D. Endomyocardial fibrosis 180/100 mmHg, HR = 120/min. He is irritable
and aggressive, with dilated pupils. ABG is
E. Becker disease normal on room

39
air. ECG shows acute ST-segment elevations C. Intensive medical Tx until EF < 40%
in the anterolateral leads. What’s the most D. Intensive medical Tx until his artery
likely cause for his occlusion > 80% for surgical
conditions? revascularization
A. Alcoholic cardiomyopathy E. Bypass surgery for the two main coronary
B. Cardiac contusion arteries now
C. Pheochromocytoma 30. A 60 y/o man with prolonged hypertension
D. MI presents with progressive short of breath at
E. Drug overdose rest and at night
28. A 60 y/o man is brought to the ER with in bed, and bilateral ankle swelling. P/E
nausea and crushing chest pain radiating results are: RR = 22/min, HR = 85/min, BP =
down the left arm 120/80 mmHg; JVD
after a big meal an hr ago. He feels an “ (+), bilateral ankle edema (+), cardiac S3
impending doom” to come. He has a history gallop (+), bilateral lung crackles (+). CXR
of smoking, alcohol reveals an enlarged
drinking, GERD, hypertension, and high heart image and bilateral pulmonary edema.
cholesterol for several years. P/E results: Among the following medicines used in this
Alert, pale, RR = 27/min, patient, which one
HR = 105/min, BP = 77/55 mmHg on both arms, does NOT show decrease in the mortality?
T = 37.5 oC, and ABG = 96% on room air. Neck A. Aspirin
shows B. Digoxin
JVD and lungs are (-) on auscultation. ECG C. Captopril
reveals ST-elevations in the leads II, III, D. Valsartan
and aVF. CK-MB and E. Spironolactone
troponin are (+). What ’ s the best F. Hydralazine
explanation for these findings? 31. A 30 y/o female is brought to the ER
A. Acute GERD with sharp, shearing chest pain radiating to
B. Diastolic heart failure her mid back. It started
C. Acute mitral regurgitation 30 min ago and is still continuing. She is
D. Left-ventr infarction tall, thin, and generally healthy except for
E. Right-ventr infarction “occasional
F. Acute aorta dissection hypertension.” P/E results: RR = 25/min, HR
= 102/min, BP = 180/105 mmHg in the right
29. A 60 y/o man has progressive, unstable arm and 140/95
angina that is resistant to regular medical mmHg in the left arm. Cardiac exam shows a
Tx. His uncle died of blowing diastolic murmur best heard at the
heart attacks before the age of 50. He has right upper sternal
a 10-year history of moderate smoking, border. ECG reveals sinus tachycardia and
alcohol drinking, and normal QRS-ST waves. CXR, CT, and
hyperlipidemia. Cardiac catheterization angiography all seem
demonstrates about 70% occlusion of three normal. What’s the best diagnostic test
coronary arteries. His next?
left ventricular EF (ejection fraction) is A. Cardiac enzymes
50%. Which of the following is the best B. Transesophageal echocardiogram (TEE)
therapy for this man? C. Repeat chest CT
A. Triple coronary bypass surgery using D. Repeat angiography
vessel grafts E. Chest MRI
B. Angioplasty and stenting of all three
arteries

40
32. A 30 y/o female comes to you for an medication ingestion, except for a 4-kg
evaluation before planning a pregnancy. She weight loss over the past 2 months. P/E
is generally active results: normal vital signs,
and healthy without any personal or family low fever, and a cachectic appearance; the
history of heart disease. She exercises lungs are (-); JVD is (+) and alleviated with
often and feels well. P/E deep inspiration; S1
finds P = 85/min, normal BP, S1, and S2, a and S2 heart sounds are distant, with an
mid-diastolic rumbling murmur; the lungs are early S3; the liver is enlarged. ECG shows
(-). CXR is low voltages without
normal. Echocardiogram reveals mild mitral ST-elevation. CXR reveals an enlarged heart.
stenosis and the left atrium enlargement; What’s the most likely diagnosis?
the left-ventr cavity A. Tamponade
size and systolic function are normal. Her B. Congestive cardiomyopathy
blood glucose level is normal. Based on this C. Constrictive pericarditis
information, what’s D. Restrictive cardiomyopathy
your best advice to the patient? E. Right ventricular infarction
A. She should avoid pregnancy because of her 35. A 50 y/o administrative man comes to you
risky heart conditions for a health evaluation. He has a 5-year
B. She can start pregnancy now history of heavy
C. She should start a beta-R blocker before smoking (1 pack/day) and alcohol drinking (>
pregnancy 5 cups/day). He also has a family history of
D. She needs an exercise stress test to hypertension and
evaluate the risk diabetes. He rarely goes to a physician for
E. She needs a prophylactic mitral balloon health examinations. P/E finds BP = 144/94
valvotomy before pregnancy mmHg at rest, body
33. You are asked to review and decide weight = 70 kg, height = 1.65 meters. There
antimicrobial prophylaxis for multiple cases are no other abnormal findings. What’s your
of infective best advice for
endocarditis. Which of the following cases him to reduce the high BP?
is most likely to benefit from antimicrobial A. Cut down alcohol drinking
prophylaxis? B. Stop smoking
A. A 65 y/o man with aortic stenosis that is C. Reduce weight
scheduled for a cardiac catheterization D. Do regular exercise
E. Reduce work stress
B. A 45 y/o man with mitral stenosis that is 36. A 65 y/o man complains of “crushing
scheduled for an esophageal dilatation chest pain” radiating to the left arm and
C. A 65 y/o man with mitral regurgitation is brought to the
that is scheduled for an esophageal emergency room. He also has anxiety,
endoscopy sweating, and nausea. His vital signs are
D. A 45 y/o man with mitral stenosis that is stable. He has a history of
scheduled for a TEE diabetes mellitus. In the P/E, the physician
E. A 35 y/o woman with mitral regurgitation will most likely find
that is scheduled for a hysterectomy A. a displaced point of maximal impulse
34. A 40 y/o man comes from the poor (PMI)
countryside presenting with progressive B. S4 gallops
dyspnea on exertion, C. continuous “machinery” murmurs
chronic cough, and chest pain for the past D. triphasic scratchy sounds
month. He denies any history of chronic E. increased jugular venous pressure on
diseases and present inhalation

41
37-38. 37. A 60 y/o woman presents with 40. A 13 y/o girl is brought to the clinic
dyspnea, cough with pink sputum, and for delayed development, fatigue, headache,
pressure-like chest pain and periodic syncope.
for the past 3 months. P/E finds cyanosis, P/E finds a pale girl with a short stature
RR = 36/min, HR = 110/min, BP = 180/118 mmHg, and tachycardia; no cyanosis. Systolic BP is
PMI shift to higher in the right arm
the left, wet rales, and S3 gallops over the than in the left. ECG reveals LVH. CXR shows
apex at auscultation. CXR and ECG confirm a “ reverse 3 ” sign and pulmonary
the diagnosis. The congestion.
best immediate treatment is 41. A 1-month infant is found with a blue
A. metoprolol face, dyspnea, and weakness. The mother has
B. nitroprusside a 5-year history of
C. furosemide alcohol use and smoking before pregnancy at
D. valsartan age 37. P/E finds a flat face and cyanosis.
E. digoxin A systolic ejection
F. nifedipine murmur is heard at the upper left sternal
border with a single S2. ECG reveals left
38. The most likely cause of death for the axis deviation. CXR
above patient will be shows mildly reduced pulmonary vascular
A. myocardial infarction markings.
B. myocardial rupture 42. A 1-month infant is found with a blue
C. pulmonary edema face, dyspnea, and fatigability for the past
D. pulmonary emboli week. The mother has
E. cardiac emboli a 5-year history of alcohol use and smoking
F. cardiac arrhythmia for before pregnancy at age 37. P/E finds a
39-42: Match the following clinical weak infant with
scenarios with the most likely Dx. cyanosis and a flat facial profile. A
A. Ventricular septal Defect (VSD) systolic ejection murmur is heard at the
B. Atrial septal Defect (ASD) upper left sternal border with a
C. Patent ductus arteriosus (PDA) single S2. ECG reveals RVH. CXR shows a “
D. Coarctation of the aorta (CA) boot-shaped” heart with decreased pulmonary
E. Tetralogy of Fallot (TF) vascular
F. Kartagener syndrome (KS) markings.
G. Persistent truncus arteriosus (PTA) Answers and Explanations
H. Tricuspid atresia (TA) 1. (D). Digoxin is the best initial
I. Hypoplastic left heart syndrome treatment (Tx) for this patient with CHF +
J. Transposition of the great arteries (TGA) atrial fibrillation. Warfarin (to
39. A 3 y/o boy is brought to the clinic for prolong PT) +/- aspirin should be added for
fatigue and frequent respiratory infections. 3 weeks before cardioversion and be
The mother has a 5- continued until normal
year history of alcohol use and smoking (Nl) sinus rhythm has been maintained for at
before the pregnancy. P/E finds a weak, least 4 weeks, to prevent embolism. “C” is
alert boy without cyanosis. good for acute
A wide, split S2 and systolic ejection Atrial-fib rate control, but should be used
murmur is heard at the upper left sternal cautiously with heart failure. “ E ” is
border. ECG reveals RVH. indicated if the patient is
Echocardiography is ordered. unstable.
2. (A). It ’ s a case of digitalis
intoxication. A-V block must be first
corrected with atropine; if the ventricular

42
 11. (C). This is a complicated case of DM,
(Ventr) rate rate is still < 50 bpm after HTN, and CHF. Diuretics should be first
atropine administration, e-pacing is chosen with HTN +
performed. Meanwhile, supply K CHF, but contraindicated with hyperglycemia.
+ Mg and check digitalis levels; if it’s > Thus an ACEI is best here. A beta -R blocker
10mg/L, stop it and add anti-digitalis is good with
fragment. MI, but not with DM and hypoglycemia.
3. (J). This is a case of LBBB (left bundle 12. (E). Alpha-R-blocker is the best for
branch block) and requires a Ventr- this patient with mild hypertension + BPH
pacemaker. If the ECG signs (benign prostate
are in leads V1-2, it’s RBBB and only needs hyperplasia). Otherwise, valsartan (an
observation. angiotensin-II-R blocker) is also a good
4. (D). This is typical atrial flutter and replacement for ICEI
best treated with a Ca-blocker. If it’s sensitivity.
unstable, e-cardioversion is 13. (E). No special Dx or Tx is needed if
done. the BP recheck is < 135/85 mmHg. If it’s
5. (K). This is an unstable Ventr- still > 140/90 mmHg,
tachycardia requiring immediate e- it’s most likely secondary hypertension.
cardioversion. If it’s stable, lidocaine is Suspect renal artery stenosis, Conn ’ s
the Tx. syndrome, Cushing’s
6. (F). Wolff-Parkinson White syndrome syndrome, or adrenal tumors.
(WPW), with procainamide as the first 14. (D). Most likely, a pheochromocytoma
choice. Radiofrequency (Triad—episodic hypertension, headache and
ablation is the definitive Tx. Beta-R diaphoresis), and
blockers, Ca-channel blockers and digitalis ‘D’ is the best Dx test to confirm it. ‘
are contraindicated. B’ also helps locate the tumor. After the
7. (M). Most likely, it’s a case of Torsade Dx is confirmed, Tx is an
de Pointes or long Q-T syndrome, often alpha and beta R blocker followed by
associated with recurrent surgical resection. If there are other
Ventr-tachycardia or Ventr-fibrillation. manifestations, “A and C” help
The best initial Tx is a beta -R blocker plus distinguish renal artery stenosis or
correction of hypo- Conn/Cushing syndrome. Polycystic kidney
K/Mg. If unstable, e-cardioversion is disease and coarctation of
needed. the aorta are other differential Dx.
8. (M). Most likely, an acute inferior MI, 15. (E). This is most likely subacute
and best treated initially with “ABC” and endocarditis caused by Strep or Listeria (>
a beta -R blocker, 60 y/a) after a dental
followed by morphine, aspirin, t-PA, etc. procedure, and thus ‘ E ’ is the best
Cardiac enzymes usually won ’ t increase choice. Vancomycin is usually saved for
until 4-6 hours after MI. PCNase-resistant Staph-aureus
9. (H). It’s most likely a case of post-MI and other life-threatening Gram+ infections;
pericarditis (Dressler syndrome), and not as the initial trial.
aspirin is the best Tx. 16. (D). Absolute indications include
10. (E). The best strategy for this man with progressive CHF despite Tx, fungal
2-3 risk factors for CAD is to confirm the endocarditis, valve ring abscess,
hyperlipidemia first, and failure to clear infection after a long
then stop smoking, and “A” if the next LDL course of antibiotic Tx.
is < 160 mg/dL, or “A + B” if the LDL >
160.

43
17. (A). It’s a case of elderly aortic valve disease, every generation of the family
sclerosis without stenosis. Aortic stenosis should have a similar patient or “sudden
has similar murmurs but death”. If the family history
also has a late peak and delayed carotid is (+), then “A” can be the first Dx test,
upstroke, with a soft or absent S2. and “D” is confirmative.
Echocardiogram usually shows 24. (A). It ’ s a case of restrictive
aortic stenosis and calcification of the cardiomyopathy with CHF due to rigid Ventr-
aortic valve. walls and reduced Ventrcompliance
18. (C). Papillary muscle rupture with acute and cardiac output. Heart transplants may be
MR (mitral regurgitation) after acute MI: the only cure. Alcohol usually causes
stabilize vital signs dilated
and perform an urgent valve replacement. cardiomyopathy, and high lipids (LDL) mostly
19. (A). Mitral valve prolapse: No.1 common cause CAD.
congenital valvular abnormality, usually 25. (A). Ultrasound is the proper initial Dx
seen in young step for abdominal aortic aneurisms (AAA).
female and associated with Marfan syndrome, CT or aortography
etc.; presenting with signs of chronic MR. with contrast supplies more accurate and
Acute MR: M > F; confirmative Dx. Plain X-ray can pick up a
No.1 cause is post-MI papillary muscle calcified AA
dysfunction; rapid Left-Ventr failure signs accidentally.
are common; 26. (B). This patient has intermittent
holosystolic apical murmur radiating to the claudication and impotence most likely
axilla, often with a thrill, S3, and widely caused by atherosclerosis and
split S2; treated with aortoiliac obstruction (Leriche syndrome).
Other causes are less possible for this
“E, F”. case. The best diagnostic
20. (G). A typical case of hypertrophic tool is angiography.
obstructive cardiomyopathy, distinguishable 27. (E). In a healthy young person with
from aortic stenosis. similar presentations of myocardial
21. (F). A typical ventricular septal ischemia, drug abuse (especially
rupture that requires emergent supportive Tx with cocaine or amphetamine overdose) should
and surgical repair. be highly suspected, although patient
22. (E). This patient has rheumatic fever usually denies it. Most
with mitral stenosis, and should be treated myocardial ischemia by similar drug overdose
with aspirin, diuretics is secondary to coronary vasospasm. Other
and salt-restricted diet. If failed, mitral differential Dx
commissurotomy is indicated. Valvular for ST-segment elevations on the ECG
disease with arterial include: MI, cardiac contusion, Ventr-
catheterization, GI-endoscopy, esophageal aneurysm, pericarditis, and
intubation, etc are low-moderate risks that normal variants. “ A ” usually causes
do not need dilated cardiomyopathy and heart failure. “
preventive antibiotics. B” usually requires a heavy
23. (E). This patient should be highly blunt trauma as in a traffic accident. “C
suspected of HOC for his age and P/E results, ” is characterized by periodic
and detailed hypertension, headache, profuse sweating,
family history is the most important initial palpitations, and apprehension. MI usually
step of Dx. Because HOC is an autosomal occurs in a patient > age 45 with risk
dominant genetic factors, low BP

44
and ABG, and abnormal QRS-ST waves on the and connective tissue diseases (as Marfan
ECG. syndrome probably in this case).
28. (E). Patient has a typical right-sided Visualization is necessary to
MI, with the classic P/E triad of guide Dx and Tx. Although CT or angiography
hypotension, JVD, and clear lungs on is usually the first test done, it misses
auscultation. ECG indicates infarction in an about 10% of the
inferior and a posterior distribution. Since cases. Thus TEE is the most sensitive test
instances of for aortic dissections. Cardiac enzymes a
isolated posterior wall MI is rare, isolated few hours after the
right-Ventr MI is the most likely Dx. “A or
F” is also possible but onset may help exclude MI, but MI is less
less typical. “B” is mostly associated likely with the present presentations and
with a hypertrophied heart, as with long- normal ECG. Repeating
standing hypertension or previously (-) tests ( “ C and D ” ) is
infiltrative cardiomyopathy, and mainly unlikely to provide more information
presenting with left-sided failure. “C” immediately. MRI for aortic
can occur during a large dissection is not as sensitive and
anterior wall MI secondary to papillary convenient as echocardiogram.
muscle dysfunction, causing left-sided 32. (C). Most patients with mild
failure. “D” is typically asymptomatic mitral stenosis with active
associated with ST-segment elevation in daily activities can tolerate
leads V1-4 in ECG and left-sided failure. pregnancy without any complications. Thus
29. (A). For this patient with progressive she only needs a beta-R blocker to improve
angina, 70% of major artery occlusion, and the cardiac function
multiple risk factors, (without affecting the blood glucose level)
there is a clear indication for aggressive before pregnancy rather than stress test or
surgical Tx. Coronary bypass surgery is mitral valve
better than angioplasty for procedures.
multiple artery occlusions ≥ 70%. The most 33. (B). Antimicrobial prophylaxis for
critical graft is usually done with the best infective endocarditis is mainly determined
available internal by two factors: the
mammary artery. Angioplasty and stenting ( preexisting risk of the patient, and the
“B”) is better suited for isolated vessels risk of the procedure or surgery. All the
rather than multiple above cases are with low to
ones. 70% occlusion is already an indication moderate risk of diseases (valvular
for surgical Tx, not delayed until EF < 40% diseases) and procedures except esophageal
(“C”) or occlusion dilation, which is a highrisk
> 80% (“D”). “E” is inadequate in this traumatic procedure that induces bacterial
case. translocation and microtrauma and thus
30. (B). All the listed medicines, ACE-I, requires antibiotics.
angiotensin-II-R blockers, vascular 34. (C). Constrictive pericarditis usually
dilators, diuretics, aspirin, and results from the healing of a prior
beta-R blockers have documented evidence of pericarditis, or destruction of
reducing mortality in patients with heart pericardial cavity, and presents with
failure except diastolic dysfunction and low ECG voltages,
digitalis. etc. It’s mostly idiopathic
31. (B). Most likely it ’ s an aortic and may also follow infectious causes.
dissection. Major risk factors include Tamponade (“A”) will typically show pulsus
hypertension, blunt chest trauma, paradoxus and a

45
significant decrease in the inspiratory hypertension with CHF, but the beta1 blocker
systolic BP. “B” is dilated cardiomyopathy should be watchfully used. Digoxin should be
with systolic dysfunction, avoided with
mostly caused by alcoholism, myocarditis, HTN and nifedipine is contraindicated in
etc. Echocardiography shows an enlarged CHF.
heart and ECG reveals 38. (F). This is a typical case of congestive
arrhythmias. “ D ” usually results from heart failure (CHF) and the most common
infiltrative or connective tissue disease, cause of death is from
typically showing apical ventricular arrhythmia provoked by cardiac
beats, left-ventr hypertrophy, S3, diastolic ischemia, not from direct pulmonary edema,
dysfunction, and LBBB. “E” is less likely dyspnea, emboli,
without acute crushing or myocardial infarction or rupture. A
chest pain and abnormal QRS-ST in the ECG. diuretic and vasodilator are first-line
35. (A). Excessive alcohol intake (> 5 therapies (especially with
drinks/day) has been clearly associated with hypertension) to reduce preload, afterload,
hypertension, and mortality.
cardiomyopathy, and liver disease, whereas 39. (B). Presentations are characteristic of
mild alcohol intake (< 1 drink/day) may ASD, which is associated with alcoholism,
reduce the risk of Down syndrome, etc.
CVD. Smoking significantly increases the Echocardiagraphy confirms Dx. Most small
risk of CVD but not hypertension directly. ASDs close spontaneously.
Weight reduction can 40. (D). CA is often associated with Turner
reduce the risk of CVD but not hypertension. syndrome and Berry aneurysm. It’s diagnosed
Reduction of work stress can help reduce by
hypertension but echocardiogram and treated with surgery.
alcoholism is the major concern for this 41. (H). This is a case of “TA + VSD + Down
man. syndrome ” . TA is associated with early
36. (B). This is an acute coronary syndrome cyanosis at birth,
with cardiac ischemia, which leads to 90% with VSD. VSD here has partially
noncompliance of the left compensated for the reduced R-ventr and
ventricle and S4 gallops. A displaced PMI pulmonary artery blood
towards the left is typical of left flow from TA. Tx: Give PGE1 to keep ASD and
ventricular hypertrophy and VSD open for survival.
dilated cardiomyopathy. Continuous “ 42. (E). Fallot tetralogy is associated with
machinery” murmurs are characteristic of Down syndrome and with gradual cyanosis and
patent ductus arteriosus. CHF. Give PGE1
Triphasic scratchy sounds are typical of followed by surgery to correct it.
chronic pericarditis. Kussmaul sign (E) is
mostly associated with
constrictive pericarditis.
37. (C). This is a case of hypertensive heart
failure with pulmonary edema. The best
treatment is IV
furosemide to remove a large volume of fluid
followed by nitroprusside to immediately
reduce both preload
and afterload of the heart. Metoprolol and
valsartan can be added in the maintenance
treatment of

46