Cardiology Ischemic Heart Disease Coronary artery disease (CAD) is the most common cause of death, by far, and kills 10 times more women than breast cancer. Risk factors are useful for 2 things: 1. 2. Help answer diagnostic questions in equivocal cases Modifying them can lower mortality For family history to be significant as a risk factor, the family member must be young (female relatives < 65, male relatives < 55). Emotional stress is not a clear risk factor. It is not possible to measure precisely. Risk factors include the following: • Diabetes mellitus • Hypertension • Tobacco use • Hyperlipidemia • Peripheral arterial disease • Obesity • Inactivity • Family history Coronary artery disease (CAD) presents with chest pain that does not change with body position of respiration. CAD is not associated with chest wall tenderness. The single worst or most dangerous factor for CAD is diabetes. When any one of the following 3 features is present, the patient does not have CAD. Pleuritic Pain (changes with respiration) Pulmonary embolism Pneumonia Pleuritis Pericarditis Pneumothorax Positional Pain (changes with bodily position) Pericarditis Tenderness (pain on palpation) Costochondritis The most common cause of chest pain that is not cardiac in etiology is a gastrointestinal (acid reflux) problem. 1. 2. 3. A patient comes to the emergency department with chest pain. The pain also occurs in the epigastric area and is associated with a sore throat, a bad metallic taste in the mouth, and a cough. What do you recommend? An alcoholic patient comes to the emergency department with chest pain. There is nausea and vomiting and epigastric tenderness. What do you recommend? A patient comes to the emergency department with chest pain. There is right-upper quadrant tenderness and mild fever. What do you recommend? Answers: 1. Give a proton pump inhibitor. 2. Check amylase and lipase levels. 3. Order an abdominal sonogram for gallstones. Beside chest pain, other clues to ischemic disease as the cause of chest pain are as follows: • • • • • Dull pain Lasts 15–30 minutes Occurs on exertion Substernal location Radiates to the jaw or left arm Physical Findings There is nothing unique or pathognomonic about the physical findings of ischemic heart disease. Physical findings such as tenderness only tell you the patient does not have ischemic disease. There is no buzzword for physical examination of CAD that indicates, “Aha! This is coronary disease.” Piece of Physical Exam Cardiovascular (CV) Chest General exam Extremities Basic Science Correlate Findings That Could Be Abnormal S3 gallop: Dilated left ventricle S4 gallop: Left ventricular hypertrophy Jugulovenous distention Holosystolic murmur of mitral regurgitation Rales suggestive of congestive heart failure Distressed patient, short of breath, clutching chest Edema The mechanism of an S3 gallop is rapid ventricular filling during diastole. As soon as the mitral valve opens, blood rushes into the ventricle, causing a splash sound transmitted as an S3. S4 gallop is the sound of atrial systole into a stiff or noncompliant left ventricle. It is heard just before S1 and occurs with any type of left Ventricular hypertrophy. S4 is the bang of atrial systole. Holosystolic Murmur: Mitral Regurgitation Diagnostic Testing An EKG is always the best initial diagnostic test for ischemic-type pain. If the case present to you is very clearly a case of ischemic pain and the examiners ask you to choose between an EKG and aspirin, nitrates, oxygen, and morphine, then choose treatment first. ST Elevation ST Depression T Wave Inversion The wrong “best initial tests” of steps are troponin, CK-MB, stress testing, echocardiogram, or angiography. These tests do not eliminate the need to give aspirin first. CCS Tip: When the question requests what the most accurate test is, answer CKMB or troponin. CK-MB and troponin levels both rise at 3–6 hours after the start of chest pain. They have nearly the same specificity. The main difference between CK-MB and troponin is that CK-MB only stays elevated 1–2 days while troponin lasts elevated for 1–2 weeks. Therefore, CK-MB testing is the best test to detect a reinfarction a few days after the initial infarction. CCS Tip: Always the wrong answer: LDH level or LDH isoenzymes. Basic Science Correlate Troponin C: Birds to calcium to activate actin:myosin interaction Troponin T: Binds to tropomyosin Troponin l: Blocks or inhibits actin:myosin interaction Myoglobin elevates as early as 1–4 hours after the start of chest pain. Myoglobin is the answer to the question “Which of the following will rise first?” when the choices are all cardiac enzymes. Stress testing is the answer when the case is not acute and the initial EKG and/or enzyme tests do not establish the diagnosis. Answer: Stress test when the case is equivocal or uncertain for the presence of CAD. Do not do an angiography unless the stress test is abnormal. Exercise tolerance, or “stress,” testing detects coronary artery disease when the heart rate is raised and ST segment depression is detected. This case is asking you to know that a stress test is a way of increasing the sensitivity of detection of CAD beyond an EKG and enzymes. When do I answer dipyridamole or When do I answer exercise thallium adenosine thallium stress test or testing or stress echocardiography? dobutamine echo? Patients who cannot exercise to a EKG is unreadable for ischemia: target heart rate of > 85% of maximum: • Left bundle branch block • COPD • Digoxin use • Amputation • Pacemaker in place • Deconditioning • Left ventricular hypertrophy • Weakness/previous stroke • Any baseline abnormality of the ST • Lower-extremity ulcer segment of the EKG • Dementia • Obesity Sestamibi nuclear stress testing is used in obese patients and those with large breasts because of the greater ability of this radioisotope to tissue. Answer: Angiography is the next diagnostic test to evaluate an abnormal stress test that shows “reversible” ischemia. Reversible ischemia is the most dangerous thing that a stress test can show. If the stress test shows “fixed" defects—that is, a defect unchanged between exercise and rest—this is a scar from a previous infarction. Fixed defects do not need angiography. Coronary bypass is the answer only if the angiogram has already been done. Echocardiography is the best initial test to evaluate valve function or ventricular wall fiction. Nuclear ventriculogram is the most accurate method to evaluate ejection fraction. Basic Science Correlate Mechanism of Thallium Nuclear isotopes are picked up by the Na/K ATPase of normal myocardium. If cardiac tissue is alive and perfused, it will pick up the nuclear isotope. To the myocardium, thallium looks like potassium. Decreased uptake = Damage Answer: The CK-MB level should return to normal 2–3 days after a myocardial infarction. If a reinfarction has occurred, the level will go back up again 5 days later, while the troponin level will still be up from the original infarction. Troponin can be elevated for 2 weeks after an infarction. Angiography can detect obstructive, stenotic lesions but cannot detect myocardial necrosis. Stress testing should never be performed if the patient is having current chest pain; chest pain is a reason to stop a stress test. Echo will show decreased wall movement, but this could have been present from the previous cardiac injury. Acute Coronary Syndrome (ACS) The definition of acute coronary syndrome (ACS) is as follows: • • • • • Causes acute chest pain Can be with exercise or at rest Can have ST segment elevation, depression, or even a normal EKG Not based on enzyme levels, angiography, or stress test results Based on a history of chest pain with features suggestive of ischemic disease Treatment Aspirin The best initial therapy for all cases of ACS is aspirin. Aspirin can be administrated orally or chewed and absorbed under the tongue. It has an instant effect on inhibiting platelets. Aspirin alone reduces mortality by 25 percent for acute myocardial infarction and by 50 percent for “unstable angina,” which may become a non-ST segment elevation myocardial infarction (NSTEMI). Nitrates and morphine should also be administered in acute coronary syndromes, but they do not lower mortality. Oxygen has no benefit if the patient is not hypoxic. Clopidogrel or ticagrelor is added to aspirin for all patients with acute myocardial infarction. Prasugrel is given only when angioplasty is done. Prasugrel, clopidogrel, or ticagrelor is added to everyone getting angioplasty and a stent. These meds inhibit ADP activation of platelets. Basic Science Correlate Mechanism of P2Y₁₂ Antagonists: Clopidogrel, Prasugrel, Ticagrelor These agents block aggregation of platelets to each other by inhibiting ADPinduced activation of the P2Y₁₂ receptor. Clopidogrel and prasugrel are in the thienopyridine class. Thrombolytics and Primary Angioplasty Thrombolytics and primary angioplasty both lower mortality in ST segment elevation myocardial infarction (STEMI) and are dependent on time; in other words, their benefit markedly diminishes with time. Prasugrel added only for angioplasty. Primary angioplasty means angioplasty during an acute episode of the chest pain, and infarction is the single best evidence for mortality benefit with angioplasty. Angioplasty is one type of “percutaneous coronary intervention” (PCI). PCI must be preformed within 90 minutes of arrival at the emergency department for a STEMI. Angioplasty has not been shown to decrease mortality in stable angina more than medical therapy (aspirin, beta blockers, and statins) alone. If PCI cannot be performed within 90 minutes of arrival in the emergency department, the patient should receive thrombolytics. The question will clearly state that “the patient is at a small rural hospital” or “the nearest catheterization facility is over an hour away.” The question must be clear on this point. Thrombolytics are indicated when the patient has chest pain for < 12 hours and has ST segment elevation in 2 or more leads. A new left bundle branch block (LBBB) is also an indication for thrombolytic therapy. Thrombolytics should be given within 30 minutes of a patients arrival in the emergency department with pain. Basic Science Correlate Mechanism of Thrombolytics Thrombolytics activate plasminogen into plasmin. Plasmin chops up fresh or newly formed fibrin strands into D-dimers. This is why all clots elevate levels of D-dimers. After several hours, the fibrin clot has been stabilized (made more permanent) by factor XIII. Plasmin will not cleave fibrin once stabilized by tar XIII. Beta Blockers, ACE Inhibitors, and Angiotensin Receptor Blockers Beta blockers lower mortality, but the timing of their administration is not critical. Beta blockers, such as metoprolol, should be given, but they are not as urgent to give as aspirin, thrombolytics or primary angioplasty. ACE inhibitors or angiotensin receptor blockers (ARBs) should be given to all patients with an acute coronary syndrome, but they only lower mortality if there is left ventricular dysfunction or systolic dysfunction. Make sure there is a lipid profile and start the patient on HMIG CoA reductase inhibitors, if indicated (i.e., if LDL is not at goal). Basic Science Correlate Mechanism of Beta Blockers in Myocardial Infarction The most common cause of death in both CHF and MI is ventricular arrhythmia brought on by ischemia. Beta blockers are both anti-arrhythmic and anti-ischemic. Slower heart rate means more time for coronary artery perfusion. Increased left ventricular filling time increase both stroke volume and cardiac output. Answer: Angioplasty will lower the risk of mortality most for this patient. If it can be obtained within 90 minutes, angioplasty is the best therapy. Metoprolol lowers mortality but is not dependent on how soon you give it, as long as the patient receives it before going home. Statins Statin medications, such as atorvastatin, should be given to all patients with an acute coronary syndrome, regardless of what the EKG shows or troponin or CKMB levels. Effects of Therapy Used in Acute Coronary Syndromes Always Lower Mortality Lower Mortality in Do Not Lower Mortality Certain Conditions • Aspirin • Thrombolytics • Primary angioplasty • Metoprolol • Statins • Clopidogrel, prasugrel, or ticagrelor • ⁃ ⁃ • ⁃ ⁃ ⁃ • ⁃ ⁃ ⁃ ⁃ ⁃ • ⁃ ⁃ • ACE inhibitors if ejection fraction is low • ARBs if ejection fraction is low • Oxygen • Morphine • Nitrates • Calcium channel blockers • Lidocaine • Amiodarone When is prasugrel, clopidogrel, or ticagrelor the answer? When a platelet antagonist is to be used for acute chest pain, there is aspirin allergy, the patient is to undergo angioplasty, or an acute infarction is occurring. Ticlopidine is associated with neutropenia. Add one of them to aspirin when there is an acute MI. When are calcium channel blockers (verapamil, diltiazem) the answer? The patient has an intolerance to beta blockers, such as severe reactive airway disease (asthma). There is cocaine-induced chest pain. There is coronary vasospasm/Prinzmetal's angina. When is a pacemaker the answer for acute MI? Third-degree AV block Mobitz Il, second-degree AV block Bifascicular block New left bundle branch block Symptomatic bradycardia When is a lidocaine or amiodarone the answer for acute MI? Only when there is ventricular tachycardia or ventricular fibrillation. Do not give antiarrhythmic medications to prevent ventricular arrhythmias. Clopidogrel, or ticagrelor is used when: • There is aspirin allergy. • The patient undergoes angioplasty and stenting. • There is acute MI. Prasugrel has greater efficacy than clopidogrel but causes more bleeding. Prasugrel increases bleeding in: • Age > 75 • Weight < 60 kg Complications of Myocardial Infarction (MI) All the complications of myocardial infarction result in hypotension. Diagnosis Diagnostic Test Treatment Cardiogenic shock Valve Rupture Septal rupture Echo, Swan-Ganz (right heart) catheter Echo Myocardial wall rupture Echo, right heart catheter showing a step up in saturation from the right atrium to right ventricle Echo Sinus bradycardia EKG Third-degree (complete) heart block Right ventricular infarction EKG, canon “a” waves EKG showing right ventricular leads ACEI, urgent revascularization ACEI, nitroprusside, intraaortic balloon pump as a bride to surgery ACEI, nitroprusside, and urgent surgery Pericardiocentesis, urgent cardiac repair Atropine, followed by pacemaker if there are still symptoms Atropine and a pacemaker even if symptoms resolve Fluid loading Basic Science Correlate Mechanism of Septal Rupture Systolic Murmur Left ventricular pressure is greater than till right ventricular pressure. This causes left-to-right shunt of oxygenated blood. Oxygen saturation in the right ventricle is markedly increased compared with the right atrium. Post-MI Discharge Instructions All patients post-MI should go home on aspirin, clopidogrel (or prasugrel), a beta blocker, a statin, and an ACE inhibitor. Answer: Some waiting is necessary to have sex after an infarction. Sex minimally increases the risk of infarction. The duration and the intensity of exertion are sufficient to provoke ischemia in some cases. Non-ST Segment Elevation Myocardial Infarction The predominant differences in the management of non-ST segment elevation MI (NSTEMI) are as follows: • • • No thrombolytic use. Heparin is used routinely. Low molecular weight (LMW) heparin is superior to IV unfractionated heparin. Glycoprotein IIb/IIIa inhibitors lower mortality, particularly in those under going angioplasty The single greatest benefits from GPIIb/IIIa inhibitors with ACS come in combination with angioplasty and stent placement. Abciximab does not benefit ST segment elevation MI. Answer: Heparin is the only one of these choices that has been shown to produce lower mortality. Thrombolytics do not lower mortality, unless there is ST elevation or a new LBBB. Positive cardiac enzymes are not an indication for thrombolytics. Other answers that could be right if they were choices GPIIb/IIIa inhibitors, such as eptifibatide, tirofiban, or abciximab, or the use of angioplasty/PCI. Basic Science Correlate Mechanism of Heparin Heparin potentiates the effect of antithrombin. Antithrombin actually inhibits almost every step of the clotting cascade. This is why it does not work with antithrombin deficiency. Heparin only prevents new clots from forming. Chronic Angina Office-based cases of further management will emphasize the same issues mortality benefit. Thrombolytics are only used if there is ST segment elevation or a new LBBB within 12 hours of the onset of chest pain. ARBs are used interchangeably with ACE inhibitors, especially if the patient has a cough with ACE inhibitors. Both ACE and ARBs cause hyperkalemia. Treatment Aspirin and metoprolol are the 2 main routinely indicated medication because of their benefit on mortality. Nitrates should be used for those with angina pain but they do not lower mortality. ACE inhibitors and ARBs should only be used in further management for stable cases if the question describes any of the following: • • • Congestive failure Systolic dysfunction Low ejection fraction Coronary angiography is predominately used to determine who is a candidate for coronary artery bypass grafting (CABG). You do not need to do angiography to diagnose CAD. Stress test can show “reversible ischemia.” However, you must do angiography to see who needs CABG. You do not need to do angiography to initiate the following: • • • • • • Aspirin + metoprolol (mortality benefit) Nitrates (pain) ACE/ARB (low ejection fraction) Clopidogrel, prasugrel, or ticagrelor (acute MI or can not tolerate aspirin) Statins If pain persists, add ranolazine or ivabradine Answer: The main difference between saphenous vein grafts and internal mammary artery grafts is that vein grafts start to become occluded after 5 years but internal mammary artery grafts are often patent at 10 years. There is no difference in the need for medications. Indications for CABG: • • • • Three coronary vessels with> 70 percent stenosis Left main coronary artery stenosis > 50–70 percent 2 vessels in a diabetic 2 or 3 vessels with low ejection fraction Ranolazine: • Anti-angina med • Added if other meds do not control pain Lipid Management The single strongest indication for lipid-lowering therapy is for a statin in a patient with an acute coronary syndrome. The goal of therapy in those with CAD is an LDL < 70. You will answer “statin therapy” for any case of CAD or an equivalent with an LDL > 100. Anyone with an acute coronary syndrome needs to be on a statin. The entire concept of “goal directed” therapy to a specific LDL is not clear at this time. If a question requests for the LDL goal in a patient with CAD and diabetes, the answer is LDL goal at least < 70. There are also other risk factors to consider. If the 10-year risk of CAD is > 7.5%, your answer is a statin. Risk factors in lipid management: • • Tobacco use (cigarette smoking) High blood pressure (≥ 140/90 mm Hg or on blood pressure medication) • • • Low HDL cholesterol (< 40) Family history of early coronary heart disease (female relatives < 65, male relatives < 55) Age (males ≥ 45, females ≥ 55) Answer: The statins have a greater effect on lower mortality compared with the other medicines. Recent guidelines will be further clarified over time. Give statins if the 10-year risk is > 7.5%. Coronary artery disease equivalents: • • • • Diabetes mellitus Peripheral artery disease Aortic disease Carotid disease Answer: The goal of LDL can be < 70 for patients at the very highest risk of infarction. This includes those with acute coronary syndromes or the combination of coronary disease and a very severe risk factor, such as diabetes. The most common adverse effect of statin medications is liver toxicity. As many as 1 percent of patients will stop a statin because of its effect on raising transaminases. Liver function tests should be routinely checked. Rhabdomyolysis is not the most common adverse effect. There is no routine indication to check CPK levels. PCSK9 Inhibitors Evolocumab and alirocumab inhibit proprotein convertase subtilisin kexin type 9 (PCSK9). PCSK9 blocks the clearance of LDL by the liver from the blood, PCSK9 inhibitors can bring down enormously elevated levels of LDL in familial hypercholesterolemia. They massively increase hepatic clearance of LDL but they do not lower mortality. PCSK9 inhibitors are injectable medications. They are the answer when the question says a statin is used at the maximum dose and the LDL is not controlled in severe hyperlipidemia. Summary of Lipid Management Overall, the standards for the management of hyperlipidemia are in a state of change. The clear answers are: • • • • • • Statins are by far superior to any other drug. Every patient with coronary disease and stroke should be on a statin. Most patients with diabetes should be on a statin. If the question says “> 10% ten-year risk,” then answer “a statin.” It is likely the answer with > 7.5% as well. Goal-directed therapy to a particular LDL is not clear. PCSK9 inhibitors bring the LDL down but the mortality benefit is not clear. Sex and the Heart Answer: Anxiety is the most common cause of erectile dysfunction postinfarction. Although beta blockers may be the most common medication associated with erectile dysfunction, anxiety is still a more common cause of erectile dysfunction than beta blockers. Answer: Nitrates are contraindicated when medications such a sildenafil are to be used. When used at the same time, they can cause a dangerous level of hypotension. Congestive Heart Failure (CHF) The mechanism that matters for congestive failure has to do with the difference in treatment between systolic dysfunction with a low ejection fraction and diastolic dysfunction and a normal ejection fraction. There is no clear way to distinguish systolic from diastolic dysfunction from symptoms alone. Clues in the history are hypertension, valvular heart disease, and myocardial infarction. CHF present with shortness of breath, particularly on exertion, in person with any of the following: • • • • • • • • Edema Rales on lung examination Ascites Jugular venous distention S3 gallop Orthopnea Paroxysmal nocturnal dyspnea Fatigue S3: Splash S4: Bang Basic Science Correlate Mechanism of Rales Increased hydrostatic pressure develops in the pulmonary capillaries from left heart pressure overload. This causes transudation of liquid into the alveoli. During inhalation, the alveoli open with a “popping” sound referred to as rales Pulmonary Edema Answer: Oxygen, furosemide, nitrates, and morphine are the mainstay of therapy for acute pulmonary edema. Although they are not associated with a concrete mortality benefit, they are the standard of care for pulmonary edema, which is the worst manifestation of CHF. Removing volume from the vascular system and, therefore, the lungs is more important than any form of diagnostic testing. Pulmonary edema is a clinical diagnosis. Shortness of breath, rales, S3, and orthopnea are more important in establishing the diagnosis than any single test. CCS Tip: On CCS, move the clock forward no more than 15–30 minutes at a time for acutely unstable ICU or emergency department patients. Basic Science Correlate Mechanism of Carvedilol Carvedilol is an antagonist of both beta-1 and beta-2 receptors as well as alpha-1 receptors. This makes it anti-arrhythmic, anti-ischemic, and antihypertensive. Diagnostic Testing All of the following should be ordered on the first screen on the CCS portion of the exam. They should be ordered with the initial therapy (i.e., with the oxygen, furosemide, nitrates, and morphine). Initial Tests to Be Ordered Chest x-ray EKG Oximeter (consider ordering arterial blood gases [ABG]) Echocardiogram Basic Science Correlate What the Tests Show • Pulmonary vascular congestion • Cephalization of flow • Effusion • Cardiomegaly • Sinus tachycardia • Atrial and ventricular arrhythmia • Hypoxia • Respiratory alkalosis • Distinguishes systolic from diastolic dysfunction Mechanism of "Cephalization" of Flow The bases or bottom of the lungs are generally more “full” of blood because of gravity. As fluid builds up in the lungs, it fills the vessels from the bottom to the top, like a cup filling with water. This moves the fluid towards the head, a process called “cephalization.” Mechanisms of Dobutamine, Inamrinone, and Milrinone Inamrinone and milrinone are phosphodiesterase inhibitors. They increase contractility and decrease afterload as vasodilators, yielding much the same effect as dobutamine. Dopamine increases contractility, but dopamine's alpha-1 agonist activity causes vasoconstriction. This increases afterload. Mechanism of Respiratory Alkalosis in CHF Fluid overload causes hypoxia. Hypoxia causes hyperventilation. Hyperventilation decreases pCO₂. Decreased pCO₂ causes alkalosis. Hence, hypoxia causes respiratory alkalosis. Cases of pulmonary edema and myocardial infarction should be placed in the intensive care unit. CCS Tip: Pulmonary edema is the perfect example of a CCS in which all the tests should be ordered at the same time as the treatment. Further Management The vast majority of patients with pulmonary edema will respond to preload reduction alone to control the acute symptoms. In a small number of patients, acute management with a positive inotrope will be necessary. There is no evidence that any positive inotrope or contractility inducing agent will lower mortality. They are also the answer on a CCS case of pulmonary edema when furosemide, oxygen, nitrates, and morphine are given and the patient is still short of breath after the clock is moved forward. Digoxin is never the right answer as an acute treatment for pulmonary edema. Digoxin can be used to slow the rate of atrial fibrillation. Positive Inotropic Agents Used Intravenously in the Intensive Care Unit Dobutamine (drug of choice) These are used as further management Inamrinone of acute pulmonary edema cases after the clock is moved forward 30–60 Milrinone minutes and there is no response to preload reduction. Answer: Synchronized cardioversion is used when ventricular tachycardia is associated with acute pulmonary edema. The same answer would have been used if the acute pulmonary edema was associated with the onset of atrial fibrillation, flutter, or supraventricular tachycardia. Unsynchronized cardioversion is used for ventricular fibrillation or ventricular tachycardia without a pulse. Medical therapy, such as lidocaine, amiodarone, or procainamide, can be used for sustained ventricular tachycardia that is hemodynamically stable. “Synchronized” = Timing with cardia cycle Answer: Nesiritide is a synthetic version of atrial natriuretic peptide that is used for acute pulmonary edema as a part of preload reduction. It decreases symptoms of shortness of breath and is not clearly associated with a reduction in mortality. There is no clear indication that the answer is nesiritide. Answer: BNP or brain natriuretic peptide level is a blood test that can be used to establish a diagnosis of CHF in a patient who is short of breath. If the presentation is not clear, a BNP level can be used to help distinguish between pulmonary embolus, pneumonia, asthma, and CHF. BNP level goes up in CHF but is rather nonspecific normal BNP level excludes CHF. Answer: Cardiac Output a. Decreased Systemic Vascular Resistance Increased Wedge Pressure Right Atrial Pressure Increased Increased b. Decreased Increased Decreased Decreased c. Increased Decreased Decreased Decreased d. Decreased Increased Decreased Increased Pulmonary edema is associated with a decrease in cardiac output due to pump failure, which results in the backup of blood into the left atrium and an increased wedge pressure. There is also an increase in right atrial pressure, which is the same as saying jugular venous distention. Increases in sympathetic outflow will increase systemic vascular resistance in an attempt to maintain intravascular filling pressure. Choice B represents hypovolemic shock, such as dehydration. Choice C representations septic shock, which is driven by massive systemic vasodilation, such as from gram-negative sepsis. Choice D represents pulmonary hypertension. Basic Science Correlate Mechanism of Increased Wedge Pressure in CHF Wedge pressure = Left atrial (LA) pressure The inflated balloon blocks pressure from behind catheter, making the catheter tip pick up flow from “in front,” or downstream. “Downstream" for the pulmonary capillaries means the left atrium. LV failure = Increased LA pressure = Increased wedge pressure Chronic Management of CHF All patients, having been stabilized from acute pulmonary edema, should have an echocardiogram to establish whether there is systolic dysfunction with a low ejection fraction or diastolic dysfunction with a normal ejection fraction. Long-term management of dilated cardiomyopathy or systolic dysfunction is based on the use of ACE inhibitors, beta blockers, and spironolactone. Diuretics are also used but have not been proven to lower mortality. Digoxin is used to decrease symptoms and decrease the frequency of hospitalization but has not been shown to decrease mortality in congestive failure. ARBs can be used interchangeably with ACE inhibitors. The beta blockers with evidence for lower mortality in CHF are metoprolol, carvedilol, and bisoprolol. ACE inhibitors and beta blockers are indicated for CHF patients with systolic dysfunction at any stage of disease. Hydralazine combined with nitrates is used if ACE inhibitors and ARBs cannot be used; hydralazine acts to reduce afterload. Spironolactone lowers mortality but has only been proved to do so for more advanced, symptomatic disease; any patient originally presenting with pulmonary edema should get spironolactone. Spironolactone is anti-androgenic. Spironolactone causes gynecomastia and erectile dysfunction in men; switch to eplerenone. Eplerenone is a mineralocorticoid antagonist. Eplerenone lowers mortality in CHF without the antiandrogenic side effects of spironolactone. The patient is still dyspneic after wine ACE Inhibitors, beta blockers, diuretics, digoxin, and mineralocorticoid inhibitors. Answer: • Ivabradine: SA nodal inhibitor of “funny channels” that slows the heart rate. Add it to systolic dysfunction if the pulse is > 70/minute or beta blockers can't be used. There is mortality benefit with ivabradine. • Sacubitril/valsartan: This combination is used instead of an ACE inhibitor. Sacubitril is added only to an ARB; this neprilysin inhibitor has a mortality benefit for systolic dysfunction. Diastolic dysfunction is treated with beta blockers and diuretics. However, you have to be careful not to overuse diuretics. The benefit of ACE inhibitors for diastolic dysfunction is not clear. Digoxin and spironolactone definitely do not help diastolic dysfunction. Further management of CHF calls for the following treatments: Systolic Dysfunction (Low Ejection Fraction) • ACEI or ARB • Metoprolol, carvedilol, or bisoprolol • Spironolactone or eplerenone • Diuretics • Digoxin • Hydralazine/nitrates Diastolic Dysfunction (Normal Ejection Fraction) • Metoprolol, carvedilol, or bisoprolol • Diuretic The single most important fact about the “further management” of CHF is that mortality is decreed by ACE/ARB, beta blockers, and spironolactone. Digoxin decreases symptoms but does not lower mortality. Answer: Implantable cardioverter/defibrillators are indicated in dilated cardiomyopathy. The most common cause of death in CHF is sudden death froIn arrhythmia. Those with an ejection fraction below 35 percent that persists are candidates for implantable defibrillator placement. Answer: Biventricular pacemaker is associated with a decrease in mortality in those with severe congestive failure with an election fraction < 35 percent and a QRS duration > 120 msec. This is also referred to as "cardiac resynchronization therapy.” If the patient is still short of breath and QRS is wide (> 120), then resynchronize with a biventricular pacer. Basic Science Correlate Mechanism of Biventricular Pacemaker Wide QRS means ventricles not beating together. Ventricles not beating together means inefficient forward flow, like trying to hop on one leg. Biventricular pacemaker means both ventricles go back to beating at same time. Effect instant. Answer: There is no place for routine anticoagulation with warfarin, no matter how low the ejection fraction may be in CHF in the absence of a clot of chronic atrial fibrillation. Warfarin is a wrong answer for CHF. Answer: Symptomatic bradycardia is an absolute contraindication for the use of beta blockers. The overwhelming majority of patients with peripheral artery disease can still use beta blockers. In a patient with a myocardial infarction, the mortality benefit of metoprolol far exceeds the risk of its use when asthma, emphysema, or peripheral artery disease is present. Two thirds of asthma patients can tolerate beta blockers. Valvular Heart Disease All valvular heart disease presents with shortness of breath as the chief complaint. Look for the phrase “worse with exertion or exercise.” Hypertension, myocardial infarction, ischemia, increasing age, and rheumatic fever will be in the history but, with the exception of rheumatic heart disease, are probably too nonspecific to give you the diagnosis. Young patients with valvular heart disease will have mitral valve prolapse, hypertrophic obstructive cardiomyopathy, mitral stenosis, or bicuspid aortic valves. Following are clues to the diagnosis: Clue to Diagnosis Young female, general population Likely Diagnosis Mitral valve prolapse Healthy young athlete Hypertrophic obstructive cardiomyopathy (HOCM) Mitral stenosis Immigrant, pregnant Turner’s syndrome, coarctation of aorta Bicuspid aortic valve Palpitations, atypical chest pain not with exertion Mitral valve prolapse Physical Findings All valvular heart disease can be expected to have murmurs and rales on lung exam. Possible findings on exam are as follows: • • • Peripheral edema Carotid pulse finds Gallops Therefore, when you suspect valvular disease, select the cardiovascular, chest, and extremities portion of the physical exam. Murmurs The most difficult important aspect for the exam is the part of the valvular heart disease section that concerns understanding auscultation, particularly the effects of various maneuvers on these murmurs. Systolic murmurs are most commonly aortic stenosis, mitral regurgitation, mitral valve prolapse, and hypertrophic obstructive cardiomyopathy (HOCM). Diastolic murmurs are most commonly aortic regurgitation and mitral stenosis. All right-sided murmurs increase in intensity with inhalation. All left-sided murmurs increase with exhalation. Murmur intensity increases with… Side of murmur Exhalation Inhalation Left Right Associated disease Mitral and aortic valve lesions Both stenosis and regurgitation of tricuspid valves Effects of Valsalva, Standing, Squatting, and Leg Raise Maneuvers predominately affect the volume of blood entering the heart. Squatting and lifting the legs in the air increase venous return to the heart. Valsalva maneuver and standing up suddenly decrease venous return to the heart. Valsalva maneuver is exhaling against a closed glottis, like bearing down a bowel movement or blowing against a thumb stuck in the mouth. This increases intrathoracic pressure, which decreased blood return to the heart. When suddenly squat, you are squeezing the veins of the legs, which are rather large. This essentially squeeze blood up into the heart like squeezing on a tube of toothpaste. For those too weak to squat suddenly, the physician can lift up the legs. This has the same effect as squatting, which is to drain blood into the chest from the lower extremities. The majority of murmurs increase in intensity with squatting and leg raise. Aortic stenosis (AS), aortic regurgitation (AR), mitral stenosis (MS), mitral regurgitation (MR), and all right-sided heart lesions will become louder with squatting and leg raising. The only two murmurs that become softer with these maneuvers are mitral valve prolapse (MVP) and hypertrophic obstructive cardiomyopathy (HOCM). The following table shows the effect of venous return on murmurs. Aortic stenosis (AS) Effects of Change in Venous Return Increase (squat, leg raise) Increased murmur Effects of Change in Venous Return Decrease (stand, Valsalva) Decreased murmur Aortic regurgitation (AR) Increased murmur Decreased murmur Mitral stenosis (MS) Increased murmur Decreased murmur Mitral regurgitation (MR) Increased murmur Decreased murmur Ventricular septal detect (VSD) Hypertrophic obstructive cardiomyopathy (HOCM) Mitral valve prolapse (MVP) Increased murmur Decreased murmur Decreased murmur Increased murmur Decreased murmur Increased murmur Valvular lesion Effects of Handgrip and Amyl Nitrate Handgrip is a maneuver that increases afterload by compressing the arteries of the arm by contracting the muscles of the arm. Handgrip does not significantly increase venous return to the heart: the veins of the arms are not as large as those of the legs, so compressing them does not make much difference in venous return to the heart. Handgrip, because it increase afterload, essentially functions as the opposite of an ACE inhibitor, worsening the murmurs of conditions that would get better with an ACE inhibitor. For instance, AR and MR are treated with ACE inhibitors, because afterload reduction increases the forward flow of blood into the aorta. Handgrip will, therefore, worsen AR and MR murmurs by pushing blood back into the heart. Handgrip will make the murmurs of AR and MR louder and more intense. The same is true for VSD. Handgrip worsens the murmur of VSD, because more blood now goes from the left ventricle into the right ventricle. Amyl nitrate is a vasodilator that decreases afterload by dilating peripheral arteries. Amyl nitrate has the opposite effect of handgrip. Giving amyl nitrate is like giving an ACE inhibitor or ARB. If handgrip worsens AR and MR, then amyl nitrate improves AR and MR. Handgrip improvements or lessens the murmurs of MVP and HOCM. The murmurs of MVP and HOCM lessen when the left ventricular chamber is larger or more full. What happens to the size of the left ventricular (LV) chamber if there is increased afterload? The LV chamber will not empty and, therefore, the LV will be larger. A larger LV chamber relieves or lessens the obstruction in HOCM. Amyl nitrate will do the opposite. It increases ventricular emptying and, therefore, decrees the size of the LV. Amyl nitrate worsens the murmurs of MVP and HOCM by increasing the obstruction and the degree of prolapse of the valves in MVP. The effect of handgrip and amyl nitrate on aortic stenosis can be hard to understand. Handgrip softens the murmur of aortic stenosis. This happens by preventing blood from leaving the ventricle; you can't have a murmur if blood is not moving. If afterload goes up, blood can't eject from the LV, and the AS murmur will soften. Another way of saying it is that the murmur of AS is based on the gradient between the LV and the aorta. If the LV pressure is greater than the aorta pressure, then the gradient or difference is high. The higher the gradient, the louder the murmur and the more sever the AS is considered. Handgrip increases pressure in the aorta; therefore, the gradient or difference between the LV and aorta decreases. Handgrip is like covering up a trombone or trumpet. You can't produce music if you put a hand over the front of a wind instrument. Amyl nitrate has exactly the opposite effect on AS compared to handgrip. Amyl nitrate decreases afterload and decreases the pressure in the aorta, thus increasing the gradient between LV and aorta and worsening (making louder) the murmur of AS. Mitral stenosis (MS) is little affected by either handgrip or amyl nitrate. These generally do not affect ventricular filling, which is the major component of MS. ACE inhibitors, likewise, have very little effect on MS. Aortic stenosis (AS) Effect on Murmur Volume Handgrip (Increased Afterload) Decrease Effect on Murmur Volume Amyl Nitrate (Decreased Afterload) Increase Aortic regurgitation (AR) Increase Decrease Mitral stenosis (MS) Negligible effect Negligible effect Mitral regurgitation (MR) Increase Decrease Ventricular septal detect (VSD) Hypertrophic obstructive cardiomyopathy (HOCM) Mitral valve prolapse (MVP) Increase Decrease Decrease Increase Decrease Increase Valvular Lesion Location and Radiation of Murmurs One of the main clues to the identity of a murmur is the location at which the murmur is heard. • • • • Aortic stenosis is heard best at the second right intercostal space and radiates to the carotid arteries. It is classically described as a crescendo decrescendo murmur. Pulmonic valve murmurs are heard at the second left intercostal space. Aortic regurgitation and tricuspid murmurs, as well as VSD murmurs, are heard at the lower left sternal border. Mitral regurgitation (MR) is heard at the apex and radiates into the axilla. The apex is at the level of the 5th intercostal space, below the left nipple. Intensity of Murmurs • • • • • I/VI: Only heard with special maneuvers (e.g., Valsalva, handgrip) II/VI and III/VI: Majority of murmurs; no objective difference between them IV/VI: Thrill present (a thrill is a palpable vibration you can feel from severe valve lesion) V/VI: Can be heard with stethoscope partly of the chest VI/VI: Stethoscope not needed to hear it Diagnostic Testing The best initial diagnostic test for valve lesions on single best answer questions is an echocardiogram. The most accurate test is a left heart catheterization. This can also measure pressure gradients, such as in aortic stenosis, most accurately. On CCS cases, you should also add an EKG and chest x-ray for valvular lesion assessment. Treatment Regurgitant lesions are best treated with vasodilator therapy, such as ACE Inhibitors, ARBs, or nifedipine. Afterload reduction will slow the profession of regurgitant lesions. If handgrip makes it worse, then ACE inhibitors are the “most effective medical therapy.” If the patient still progresses despite medical therapy, then surgical replacement of the valve should be preformed. Valve replacement with a catheter can be done in some patients. Order transthoracic echocardiography (TTE) first on CCS. Then order a transesophageal echocardiogram (TEE) if the TTE is not fully diagnostic. Stenotic lesions are best treated with anatomic repair. Mitral stenosis should undergo balloon valvuloplasty, even if the patient is pregnant. Aortic stenosis that is severe must be surgically replaced. Aortic valve replacement is well tolerated even in the very old. Diuretics can decrease pulmonary vascular congestion with stenotic lesions, but they are not as effective or important as anatomical repair. • • Valsalva improvements murmur = Diuretics indicated Amyl nitrate improvements murmur = ACE inhibitor indicated Valvular Lesion Aortic stenosis (AS) Standing/Valsalva Decrease Diuretics Indicated Yes (replace best) Aortic regurgitation (AR) Decrease Yes Mitral stenosis (MS) Decrease Yes (balloon best) Mitral regurgitation (MR) Decrease Yes Ventricular septal detect (VSD) Hypertrophic obstructive cardiomyopathy (HOCM) Mitral valve prolapse (MVP) Decrease Yes Increase No Increase No Valvular Lesion Aortic stenosis (AS) Amyl Nitrate Increase ACE Inhibitor Indicated No Aortic regurgitation (AR) Decrease Yes Mitral stenosis (MS) Negligible effect No Mitral regurgitation (MR) Decrease Yes Ventricular septal detect (VSD) Decrease Yes Hypertrophic obstructive cardiomyopathy (HOCM) Mitral valve prolapse (MVP) Increase No Increase No Aortic Stenosis (AS) Aortic stenosis most commonly present with chest pain. Syncope and CHF are less common presentations. The patient will be older and often has a history of hypertension. Coronary artery disease will be present in as many as 50 percent of patients. Prognosis is as follows: • • • Coronary disease: 3- to 5- year average survival Syncope: 2- to 3- year average survival CHF: 1.5- to 2- year average survival Basic Science Correlate Mechanism of Syncope/Angina in AS In AS, a stiff valve just proximal to the entry point of coronaries blocks blood flow into the vertebral and basilar arteries and carotids. No flow to brain = Passing out. Thus, AS causes LV hypertrophy. LV hypertrophy = Increased demand. AS = Blocked flow with increased demand = Chest pain Physical Exam Choose the cardiovascular (CV) exam, chest, and extremities. AS gives a crescendo-decrescendo systolic murmur. The murmur will be heard best at the second right intercostal space and radiate to the carotid arteries. The murmur will increase in intensity with leg raising, squatting, and amyl nitrate. The murmur will decrease with Valsalva, standing, and handgrip. The case may describe delayed carotid upstroke as well. Normal aortic valve gradient is zero. Basic Science Correlate Mechanism of Crescendo/Decrescendo Murmur of AS The first part of the Cardiac cycle is isovolumetric contraction. With isovolumetric contraction, no blood moves. No blood moving = No murmur. Peak flow occurs in mid-systole. Peak flow = Peak noise. Here, AS yields a diamond-shaped crescendodecrescendo murmur. Diagnostic Testing The transthoracic echocardiogram (TTE) is the best initial diagnostic test. A transesophageal echocardiogram (TEE) is more accurate. Left heart catheterization is the most accurate diagnostic test and allows the most accurate method of assessing the pressure gradient across the valve. Mild disease is a gradient < 30 mm Hg. Moderate disease is indicated by 30–70 mm Hg, and severe disease is indicated by a gradient > 70 mm Hg. For CCS cases, also choose an EKG and a chest x-ray, which will show left ventricular hypertrophy. Treatment Diuretics are the best initial therapy but will not alter long-term prognosis. Further, overdiuresis is dangerous, and use of diuretics needs to be very judicious. The treatment of choice is valve replacement. Bioprosthetic valves (porcine, bovine) will last 10 years on average, but do not require anticoagulation with warfarin. Mechanical valves do not have to be replaced as often, but must also be treated with warfarin to an INR of 2—3. Valve replacement is well tolerated, even in the elderly. Valve replacement by catheter is done when a patient can’t tolerate surgery. Balloon dilate AS only if the patient is to sick to go surgery. Mechanical values can wear out after 15-20 years. Aortic Regurgitation (AR) Hypertension, rheumatic heart disease, endocarditis, and cystic medial necrosis cause AR. Rarer causes are Marfan’s syndrome, ankylosing spondylitis, syphilis, and reactive arthritis. Reactive arthritis is an inflammatory arthritis tis of large joints, inflammation of the eyes (conjunctivitis and uveitis), and urethritis, previously commonly referred to as Reiter's syndrome. Shortness of breath and fatigue are the most common presentation. Physical Exam Choose the cardiovascular (CV) exam, chest, and extremities. The murmur of AR is a diastolic decrescendo murmur heard best at the left stern border. There are several unique physical findings that are rarely seen with AR. The murmur will increase in intensity with leg raising, squatting, and handgrip. • • • Quincke pulse: Arterial or capillary pulsations in the fingernails Corrigan's pulse: High bounding pulses (also known as a “water-hammer pulse”) Musset's sign: Head bobbing up and down with each pulse • • Duroziez's sign: Murmur heard over the femoral artery Hill sign: Blood pressure gradient much higher in the lower extremities Diagnostic Testing TTE is the best initial diagnostic test. TEE is more accurate. Left heart catheterization is the most accurate test. For CCS cases, also choose an EKG and a chest x-ray, which will show left ventricular hypertrophy. Treatment ACE inhibitors, ARBs, and nifedipine are the best initial therapy. For CCS cases, you should add a loop diuretic, such as furosemide. Surgery is the answer when the ejection fraction drops below 55 percent or the left ventricular end systolic diameter goes above 55 mm; surgery should be done in patients with these criteria even if they are asymptomatic. Basic Science Correlate Why Does High Pressure Dilate the Aortic Valve? The law of LaPlace says: The wall tension, or force dilating a vessel, is proportionate to the radius of the vessel times the pressure on the inside. Tension = Radius x Pressure The wider a vessel is, the faster it will get dropped apart. Here, the more the aortic ring of aorta is dilated, the faster it will get folded apart. Likewise, the higher the interior pressure, the faster the interior will be pulled apart. This force of being “pulled apart,” or dilation, is called “wall tension” in the law of LaPlace. ACE and ARB drugs vasodilate peripheral arterioles. Lower pressure = Lower wall tension. Mitral Stenosis (MS) Rheumatic fever is the most common cause of MS. Look for an immigrant patient because of the low rates of rheumatic fever in the United States. Also look for a pregnant patient because of the large increase in plasma volume with pregnancy. Special features of MS are as follows: • • • Dysphagia: Large left atrium pressing on the esophagus Hoarseness: Pressure on recurrent laryngeal nerve Atrial fibrillation leading to stroke Basic Science Correlate Mechanism of Increased MS Symptoms in Pregnant Women Pregnant women have a 50 percent increase in plasma volume. More volume with the same valve diameter Tears more pressure, backflow, and symptoms. Pregnancy also changes the hypothalamic osmolar receptors. ADH levels stay higher in pregnancy, so the collecting duct absorbs more free water. Physical Exam Choose the cardiovascular (CV) exam, chest, and extremities. The murmur of MS is a diastolic rumble after an opening snap, which can be described as an “extra sound” in diastole. The S1 is louder. As the mitral stenosis worsens, the opening snap moves closer to S2. The murmur will increase in intensity with leg raising, squatting, and expiration. Basic Science Correlate Mechanism of Opening Snap Earlier in Worsening MS The mitral valve opens when LA pressure > LV pressure. Worse MS = Higher LA pressure. Higher LA pressure pushes the mitral valve open earlier. Diagnostic Testing TTE is the best initial diagnostic test. TEE is more accurate. Left heart catheterization is the most accurate test. For CCS cases, also choose an EKG and a chest x-ray, which will show left atrial hypertrophy. On chest x-ray, there is straightening of the left heart border and elevation of the left mainstem bronchus. There may also be a description of a double density in the cardiac silhouette (from left atrial enlargement). Treatment Diuretics are the best initial therapy. They do not alter progression. Balloon valvuloplasty is the most effective therapy. Pregnant women can and should be readily treated with balloon valvuloplasty. Pregnancy is not a contraindication to valvuloplasty. Basic Science Correlate Balloon valvuloplasty works in MS because the stenosis results from excess fibrosis of the valve. Rheumatic fever causes cardiac endomyocardial and valvular fibrosis. Fibrosis can be stretched by the balloon. By contrast, aortic stenosis is calcified, and calcification does not stretch or rip easily with a balloon. MS = Balloon fibrosis AS = Remove/replace calcification Mitral Regurgitation (MR) MR is caused by hypertension, ischemic heart disease, and any other condition that leads to the dilation of the heart. You cannot have dilation of the heart without the mitral valve leaflets separating. Dyspnea on exertion is the most common symptom. Physical Exam Choose the cardiovascular (CV) exam, chest, and extremities. S3 gallop is associated with fluid overload states, such as congestive heart failure or mitral regurgitation. An S3 can be normal in patients under the age of 30. The murmur of MR holosystolic and obscures both S1 and S2. MR is heard best at the apex and radiates to the axilla. The murmur increases in intensity with leg raising, squatting, and handgrip. Standing, Valsalva, and amyl nitrate decrease the intensity S3 gallop is often present. Diagnostic Testing TTE is the best initial diagnostic test for MR. TEE is more accurate. Treatment ACE inhibitors, ARBs, and nifedipine are the best initial therapy and the medications most likely to decrease the rate of progress of the disease. For CCS cases, you should add a loop diuretic, such as furosemide. Surgery is the answer when the left ventricular ejection fraction drops below 60 percent or the left ventricular end systolic diameter goes above 40 mm. Surgery should be done in patients with these criteria even if they are asymptomatic. Surgery can be valve repair or replacement. Repair means placing clips or sutures on the valve to tighten it up. The operative criteria for regurgitant lesions in asymptomatic patients are as follows (repair is preferred to replacement): Ejection fraction Left ventricular end systolic diameter Aortic Regurgitation < 55% Mitral Regurgitation < 60% > 55 mm > 40 mm Ventricular Septal Defect (VSD) Asymptomatic patients may present with only a holosystolic murmur at the lower left sternal border. Larger defects leads to shortness of breath. The murmur worsens with exhalation, squatting, and leg raise. Diagnostic Testing Echocardiography is the diagnostic test to use first, but catheterization is used to determine the degree of left-to-right shunting most precisely. Treatment Mild defects can be left without mechanical closure. Atrial Septal Defect (ASD) Small ASDs are asymptomatic. Larger ones may lead to shortness of breath or signs of right ventricular failure, such as shortness of breath and a parasternal heave. The most frequently tested knowledge is that ASD is associated with fixed splitting of S2. Basic Science Correlate Mechanism of Fixed Splitting of S2 in ASD S2 splitting is caused by different pressures on different sides of heart. Same pressure on both sides means no splitting. LA/RA pressure no change in respiration = No change in splitting Diagnostic Testing Diagnose with an echocardiogram. Treatment Percutaneous or catheter devices are the best therapy. Repair is most often indicated when the shunt ratio exceeds 1.5 to 1. Splitting of S2 Wide, P2 Delayed • Right bundle branch block • Pulmonic stenosis • Right ventricular hypertrophy • Pulmonary hypertension Paradoxical, A2 Delayed • Left bundle branch block • Aortic stenosis • Left ventricular hypertrophy • Hypertension Cardiomyopathy Dilated Cardiomyopathy Fixed ASD Dilated cardiomyopathy presents and is managed in the same way as the case of CHF described above. Diagnostic Testing Echocardiography is the best initial test to determine the ejection fraction and look for wall motion activity. MUGA or nuclear ventriculography is the most accurate method of determining ejection fraction. Treatment Besides ischemia, the most common causes of dilated cardiomyopathy are alcohol, adriamycin, radiation, and Chagas’ disease. The treatment for all forms of dilated cardiomyopathy, no matter their cause, is with ACE inhibitors, ARBs, beta blockers, and spironolactone. Spironolactone and eplerenone are mineralocorticoid or aldosterone antagonists. They are used to decrease the work of the heart; they are not given for their diuretic effect. Spironolactone is antiandrogenic and inhibits testosterone. Eplerenone does not inhibit androgens. Digoxin decreases symptoms but does not prolong survival. Ivabradine is a sodium funny channel blocking the SA node. Add ivabradine if the heart rate is > 70 after the use of beta blockers. Hypertrophic Cardiomyopathy This condition presents with shortness of breath on exertion and an S4 gallon on examination. S4 gallop is a sign of left ventricular hypertrophy and decreased compliance or stiffness of the ventricle. S4 gallop does not automatically indicate the need for additional therapy. Diagnostic Testing Echocardiography shows a normal ejection fraction. Treatment The mainstay of therapy is with beta blockers and diuretics. ACE inhibitors can be used, but their benefit is not as clear. Digoxin and spironolactone do not benefit hypertrophic cardiomyopathy. Restrictive Cardiomyopathy Restrictive cardiomyopathy presents with a history of sarcoidosis, amyloidosis, hemochromatosis, cancer, myocardial fibrosis, or glycogen storage diseases. Shortness of breath is the main presenting complaint in all forms of cardiomyopathy. Kussmaul's sign is present: this is an increase in jugular venous pressure on inhalation. Diagnostic Testing Cardiac catheterization shows rapid x and y descent. The EKG shows low voltage. Echocardiography is the mainstay of diagnosis Endomyocardial biopsy is the single most accurate diagnostic test of the etiology. Amyloid: • Low-voltage EKG • Speckled pattern on echo Treatment Diuretics and correcting the underlying cause are the best treatments. Takotsubo Cardiomyopathy This is a rare, sudden systolic dysfunction brought on by extreme emotions. Look for a vignette involving a postmenopausal woman with sudden psychological stress. Takotsubo cardiomyopathy presents like acute myocardial infarction with ventricular dysfunction. Coronary arteries are normal. Treatment Treat the patient with ACE inhibitors, diuretics, and beta blockers—as in any other ventricular failure. If there is no acute death, the patient recovers in a few weeks. Pericardial Disease Pericarditis Chest pain that is pleuritic (changes with respiration) and positional (relieved by sitting up and leaving forward) is the presentation that is most often given on Step 3. The pain will be described as sharp and brief. Ischemic pain is dull and sore, like being punched. The vast majority of pericarditis cases are viral. Although any infectious agent, collagen-vascular disease, or trauma that can be in the history, remember that Step 3 most often hands you a clear diagnosis and asks what you want to do about it, such as testing and treatment. Physical Exam The only pertinent positive finding is a friction rub, which can have 3 components. The rub is only present in 30 percent of patients. There is no pulsus paradoxus, tenderness, edema, or Kussmaul's sign present. Blood pressure is normal, and there is no jugular venous distention of organomegaly. Diagnostic Testing The best initial test is the EKG. ST segment elevation is present everywhere (all leads). PR segment depression is pathognomonic in lead II, but is not always present. Treatment The best initial therapy is an NSAID, such as indomethacin, naproxen, aspirin or ibuprofen combined with colchicine. Advance the clock 1–2 days and have the patient visit the office. If the pain persists, add prednisone orally to the treatment and advance the clock 1–2 more days. Colchicine adds efficacy to NSAIDs and prevents recurrent episodes. Pericardial Tamponade Tamponade presents with shortness of breath, hypotension, and jugular venous distention. On CCS, also examine the lungs, because they will be clear. Following the unique features of tamponade: • • Pulsus paradoxus: This is a reduction of blood pressure > 10 mm Hg on inhalation. Electrical alternans: This is alterations of the axis of the QRS complex on EKG, manifested as the height of the QRS complex. Basic Science Correlate Mechanism of Pulsus Paradoxus Inhalation increases venous return. Increased venous return expands the right ventricle (RV). Expanded RV compresses the left ventricle (LV). Compressed LV decreases blood pressure. Tamponade compresses the whole heart. Inhale = Big RV = Smaller LV = BP drop > 10 mm Hg Diagnostic Testing Echocardiography is the most accurate diagnostic test. The earliest finding of tamponade is diastolic collapse of the right atrium and right ventricle. Remember that it is normal to have 50 ml or less of pericardial fluid, but there should be no collapse of the cardiac structures. EKG will show low voltage and electrical alternans. Electrical alternans is variation of the height of the QRS complex from the heart moving backward and forward in the chest. Right heart catheterization will show “equalization” of all the pressures in the heart during diastole. The wedge pressure will be the same as the right atrial and pulmonary artery diastolic pressure. Treatment • • • Best initial therapy: Pericardiocentesis Most effective long-term therapy: Pericardial window placement Most dangerous therapy: Diuretics Constrictive Pericarditis Constrictive pericarditis presents with shortness of breath and the following signs of chronic right heart failure: • • • • Edema Jugular venous distention Hepatosplenomegaly Ascites Following are the unique features of constrictive pericarditis: • • Kussmaul's sign: Increase in jugular venous pressure on inhalation Pericardial knock: Extra diastolic sound from the heart hitting a calcified, thickened pericardium Diagnostic Testing • • • Chest x-ray: Showing calcification EKG: Low voltage CT and MRI: Showing thickening of the pericardium Treatment • • Best initial therapy: Diuretic Most effective therapy: surgical removal of the pericardium (i.e., pericardial stripping) Aortic Disease Dissection of the Thoracic Aorta Dissection of the thoracic aorta presents with the following symptoms: • • • Chest pain radiating to the back between the scapula Pain that can be described as very severe and “ripping” Difference in blood pressure between right and left arms Diagnostic Testing • Best initial test: Chest x-ray showing a widened mediastinum • Most accurate test: CT angiography Treatment When the case describes severe chest pain radiating to the back and hypertension, order beta blockers with the first screen in addition to an EKG and chest x-ray. No matter what the EKG shows, move the clock forward and order either CT angiography, TEE, or magnetic resonance angiography (MRA): all 3 are equally accurate. • CT angiography = TEE = Magnetic resonance angiography (MRA) After starting beta blockers, order nitroprusside to control the blood pressure. Aortic dissection cases should be placed in the ICU, and a surgical consultation should be ordered. Survival correction is the most effective therapy. Abdominal Aortic Aneurysm (AAA) Screening with an ultrasound should be ordered in 65- to 75- year old men who are current or former smokers. Abdominal aortic aneurysm is detected by ultrasound first. AAAs are repaired when they are > 5 cm. Smaller ones are monitored. Basic Science Correlate As an aneurysm enlarges, the rate of expansion increases: Wider aorta = Widens faster. This principle is expressed in the law of LaPlace: Wall tension = Radius x Pressure. Next step: Lower BP and repair with stent or endovascular procedure when the aneurysm goes above 5 cm. Peripheral Arterial Disease (PAD) PAD presents with claudication (pain in the calves on exertion). The case may also describe “smooth, shiny skin” with loss of hair and sweat glands, as well as loss of pulses in the feet. Spinal stenosis will give pain that is worse with walking downhill and less with walking uphill or while cycling or sitting. Pulses and skin exam will be normal with spinal stenosis. Pain + Pallor + Pulseless = Arterial occlusion Acute arterial embolus will be very sudden in onset with loss of pulse and cold extremity. It is also quite painful. AS and atrial fibrillation are often in the history for arterial embolus. Diagnostic Testing • • Best initial test: Ankle-brachial index (ABI). (A normal ABI should be ≥ 0.9.) Blood pressure in the legs should be equal to or greater than the pressure in the arms. If there is > 10 percent difference, then an obstruction is present.) Most accurate test: Angiography Treatment • ⁃ ⁃ ⁃ ⁃ ⁃ ⁃ • • Best initial therapy: Aspirin Blood pressure control with ACE inhibitors Exercise as tolerated Cilostazol Lipid control with statin a target LDL < 100 Vorapaxar, an antiplatelet drug added to aspirin or clopidogrel Marginally effective therapy: Pentoxifylline Ineffective therapy: Calcium channel blocker Beta blockers are not contraindicated with PAD. If the patient needs them for ischemic disease, they should be used. Basic Science Correlate Mechanism of Calcium Blockers: Why They Don’t Work in PAD In PAD the atherosclerotic obstruction is on the inside the vessel. Calcium blockers dilate the muscular layer, which is exterior to the atherosclerosis in the center. Dilating the outer layer does not expand the inside. CCS Tip: On CCS, move the clock forward several weeks. PAD is not an emergency! If initial therapies do not work and the pain progresses, or there are signs of ischemia such as gangrene or pain at rest, then perform surgical bypass. Rhythm Disorders Atrial Fibrillation (A-Fib) A-fib presents with palpitations and an irregular pulse in a person with a history of hypertension, ischemia, or cardiomyopathy. Diagnostic Testing If the initial EKG does not show the answer, a patient in the hospital should be placed on telemetry monitoring. Outpatients who are hemodynamically stable should go under Holter monitoring, which is continuous, ambulatory cardiac rhythm monitoring for 24 hours or longer. CCS Tip: For CCS cases, other tests to order once A-fib is found on EKG are the following: • • • • Echocardiography: Looking for clot, valve function, and left atrial size Thyroid function testing: T4 and TSH levels Electrolytes: Potassium, magnesium, and calcium levels Troponin or CK-MB levels: These may be appropriate to test in some acute-onset cases. Treatment Unstable patients should go immediately synchronized electrical cardio version. Unstable patients should be cardioverted with the first screen, without waiting for TEE or anticoagulation with heparin or warfarin. Instability is defined as a systolic blood pressure < 90, congestive failure, confusion related to hemodynamic instability, or chest pain. Stable patients should have ventricular heart rate slowed if it is > 100–110 per minute. Rate control medications are beta blockers (metoprolol, carvedilol), calcium channel blockers (diltiazem), or digoxin. In the acute setting, such as the emergency department, these agents should be given intravenously. Anticoagulation Once the rate has been controlled, anticoagulation is the next best step in all patients with an atrial arrhythmia persisting beyond 2 days. If the question does not state the duration, you are to treat it as if it were persisting for longer than 2 days. CHADS C = CHF H = Hypertension A = Age > 75 D = Diabetes S = Stroke or TIA CHADS score of 0 – 1 needs aspirin. At 2 or more, use warfarin, dabigatran, rivaroxaban, apixaban. Stroke/TIA = 2 points Dabigatran, rivaroxaban, edoxaban, and apixaban are oral anticoagulants with similar or better efficiency to warfarin but without the required to monitor the INR. The long-term use of rate control medications, such as metoprolol, diltiazem or digoxin, combined with anticoagulation is equal or better than cardioversion with electricity or medicines. Routine cardioversion of trial fibrillation is not indicated. CHADS₂ (CHF, hypertension, age > 75, diabetes, or stroke/TIA) is a screening system to indicate the need for anticoagulation. A score of 2 or more means warfarin, apixaban, dabigatran, edoxaban, of rivaroxaban. A score of 0 or 1 means aspirin. When CHADS₂ is at 2 or more points, control the rate and anticoagulate. Novel oral anticoagulants (NOACs) such as rivaroxaban, dabigatran, or apixaban become therapeutic in a few hours: Unlike warfarin, NOACs do not need several days to reach the therapeutic levels. Even when warfarin is used for atrial fibrillation, there is no need to bolus the patient with heparin. Why? Because atrial fibrillation is a long-term disease that takes months or years to develop a risk of stroke, while fulldose heparin carries a risk of bleeding. Just start the warfarin or NOACs. It is safe. Heparin treatment bridging to the therapeutic warfarin is a wrong answer. CHADS-VASc means adding points for Vascular disease such as peripheral artery disease, Age 65–74, and Sex as in female gender. The same cutoff applies: A score of 2 or more means use a NOAC. CHADS-VASc V = vascular disease A = age 65—74 S = female sex A score of 2 or more needs anticoagulation. Anticoagulation in Atrial Arrhythmias The novel (or new) oral anticoagulants (NOACs) work either by inhibiting factor Xa (rivaroxaban, apixaban, edoxaban) or by inhibiting thrombin with dabigatran. If severe bleeding occurs with warfarin, it is reversible with fresh frozen plasma (FFP). If bleeding occurs with dabigatran, it is reversible with idarucizumab. If bleeding occurs with the Xa inhibitors, it is reversible with andexanet. What to know about NOACs: • • • • NOACs prevent more stroke than warfarin. NOACs cause less intracranial bleeding than warfarin. NOACs decrease mortality more than warfarin in atrial fibrillation. NOACs treat DVT and PE. The main indication for warfarin is a patient with atrial fibrillation who have metallic heart valves. Idarucizumab reverses dabigatran. Atrial Flutter (A-Flutter) Atrial flutter is administered in the same way as atrial fibrillation. The only difference is that the rhythm is regular on presentation. The following table shows how to choose the right rate control medication for a-fib anal a-flutter. Beta Blockers (Metoprolol) • Ischemic heart disease • Migraines • Graves disease • Pheochromocytoma Calcium Channel Blockers (Diltiazem) • Asthma • Migraine Digoxin • Borderline hypotension Multifocal Atrial Tachycardia (MAT) This condition presents like an atrial arrhythmia in association with COPD/ emphysema. EKG will show polymorphic P waves, revealing different atrial foci for the QRS complexes. As the name implies, patients with MAT have tachycardia (heart rate > 100). MAT manifests as an irregular chaotic rhythm on EKG. Do not use beta blockers. Give oxygen first, then diltiazem. Give oxygen first for MAT. Supraventricular Tachycardia (SVT) SVT presents with palpitations and tachycardia and occasionally syncope. It is not associated with ischemic heart disease. SVT has a regular rhythm with a ventricular rate of 160–180. Diagnostic Testing If the EKG does not show SVT, order Holter monitor or telemetry to increase the sensitivity of detection. CCS Tip: On CCS, all cases of dysrhythmia should undergo transthoracic echocardiography (TTE) after the initial set of orders. Treatment • • • • Best initial management for unstable patients: Synchronized cardioversion Best initial management for stable patients: Vagal maneuvers (carotid sinus massage, ice immersion of the face, Valsalva) Next best step in management if vagal maneuvers do not work: Intravenous adenosine (Note: This is the most frequently asked SVT question.) Best long-term management: Radiofrequency catheter ablation Wolff-Parkinson-White Syndrome (WPW) WPW presents as SVT that can alternate with ventricular tachycardia (VT). The other main due to the diagnosis is worsening of SVT after the use of calcium blockers or digoxin. Diagnostic Testing WPW is diagnosed with finding a delta wave on the EKG. The most accurate test is electrophysiological studies. Treatment Treatment is as follows: • • Best initial therapy if the patient is described as being in SVT or VT from WPW: Procainamide Best long-term therapy: Radiofrequency catheter ablation Basic Science Correlate Mechanism of WPW There is an abnormal piece of neutralized cardiac muscle going around the AV node in WPW. This can result in either atrial or ventricular arrhythmia. The slowest conduct in the heart is the AV node. Conduction in the aberrant tract is faster; that is why the PR is short (< 120 mSec) and there is a delta wave on EKG. Calcium channel blockers and digoxin block conduction more in the normal AV and force the conduction down the abnormal conduction tract. Ventricular Tachycardia (VT) VT can present as palpitation, syncope, chest pain, or sudden death. Diagnostic Testing You cannot determine that VT is present without an EKG. If the EKG does not detect VT, then telemetry monitoring should be ordered. The most accurate diagnostic test is electrophysiological studies. Treatment The table below shows therapy options for persistent VT: Hemodynamically Stable • Amiodarone • Lidocaine • Procainamide • Magnesium Hemodynamically Unstable • Synchronized cardioversion Torsade de pointes is ventricular tachycardia with an undulating amplitude. Magnesium should always be given in addition to medical or electrical therapy. Ventricular Fibrillation (V-Fib) V-fb presents as sudden death. Diagnostic Testing You cannot tell what caused the loss of pulse without an EKG. Treatment Treatment of V-fib is always with unsynchronized cardioversion first. Do not answer intubation first. Dead people are never breathing. If you shock them back to life, they are more likely to breathe! When to deliver electricity: Unsynchronized At any point in cycle Synchronized Not during the T-wave Indications: V-fib, pulseless VT Everything except V-fib and pulseless VT Basic Science Correlate Mechanism of Need for Synchronization The T-wave represents the refractory period. An electrical shock delivered during the T-wave can set off a worse rhythm—specifically, asystole and ventricular fibrillation are worse than ventricular tachycardia. Do not deliver a shock during the refractory period. Unsynchronized cardioversion (defibrillation) is administrated as follows: 1. 2. 3. 4. 5. 6. 7. Continue CPR Reattempt defibrillation Administer IV epinephrine Reattempt defibrillation Administer IV amiodarone or lidocaine Reattempt defibrillation Repeat several cycles of CPR between each shock Syncope Evaluation The management of syncope is based on 3 criteria: 1. 2. 3. Was the loss of consciousness sudden or gradual? Was the regaining of consciousness sudden or gradual? Is the cardiac exam normal or abnormal? Diagnostic Testing On the initial screen, order the following: • • • • • • Cardiac and neurological examination EKG Chemistries (glucose) Oximeter CBC Cardiac enzymes (CK-MB, troponin) Carotid Dopplers are not useful in syncope. A patient cannot pass out from a carotid embolus. CCS Tip: Treat special circumstances as follows: • If a murmur is present, order an echocardiogram. • • • If the neurological exam is focal or there is a history of head trauma due to syncope, order head CT. If a headache is described, order head CT. If a seizure is described or suspected, order head CT and EEG. Basic Science Correlate Mechanism of Syncope: Caused by Brainstem Stroke Only The brainstem control sleep and wake in the brain. Only stroke or TIA of the posterior circulation can cause syncope. Vertebral/basilar circulation is synonymous with the posterior or brainstorm circulation. There is no place in the circulation of the middle cerebral artery that can cause syncope. CCS Tip: On further management, if the diagnosis is not clear after you move the clock forward to obtain the results of initial tests, order the following: • • • • Holter monitor on outpatients Telemetry monitoring for inpatients Repeat check of CK-MB and troponin levels 4 hours later Urine and blood toxicology screens CCS Tip: On further management, particularly if the etiology is not clear, order the following: • • Tilt table testing to diagnose neurocardiogenic (vasovagal) syncope Electrophysiological testing Exclude cardiac cases of syncope. More than 80% of mortality from syncope is from cardiac causes. The Holter monitor is a 24- to 72-hour continuous ambulatory EKG. This is routine for most patients with syncope requiring admission. Treatment Treatment of syncope is based on the etiology. The majority of cases never get a specific diagnosis. The most important thing to do in syncope is to exclude a cardiology etiology, such as an arrhythmia. The majority (> 80 percent) of mortality from syncope involves a cardiac etiology. If a ventricular dysrhythmias diagnosed as the etiology of syncope, an implantable cardioverter/defibrillator is indicated. CCS Tip: Botton line, order the following for syncope: • • EKG Enzyme: Troponin/CKMB • • Echocardiogram Head CT