Cardiovascular

Pathology
Objectives
1. Describe the normal function of the heart and the effects of heart failure.
2. Describe the key processes in atherosclerosis and hypertension.
3. Describe the spectrum of ischaemic heart disease and its complications.
4. Briefly describe the key causes and effects of valvular heart disease and endocarditis.
5. Briefly describe the key causes and effects of vasculitis.
CARDIAC
FUNCTION
The main function of the heart is
to provide the pumping action in
a closed circulation.
Comparison with a simple
mechanical system is useful and
valid, provided that it is
appreciated that the human
heart is infinitely more
sophisticated, with delicate
built-in controls and balances
which influence the inotropic
(intrinsic contractility) activity of
the heart.
HEART
FAILURE
ACUTE AND
CHRONIC
HEART
FAILURE
LEFT, RIGHT AND COMBINED VENTRICULAR FAILURE

Pulmonary circulation Systemic Circulation

o Low pressure system o Higher pressure system
o Systolic arterial pressure = 24 mmHg o Systolic arterial pressure = 120 mmHg
o Pressure gradient, artery/vein = 8 mmHg o Pressure gradient, artery/vein = 90
mmHg
COMPENSATORY
MECHANISMS
The onset of heart failure is preceded by
compensatory changes:
1. Increased rate of pumping
2. Dilatation
3. Hypertrophy
FAILURE OF
COMPENSATO
RY
MECHANISMS-
Heart Failure

The increased
efficiency derived from
all three mechanisms
is limited, and beyond
this limit heart failure
develops.
EFFECTS OF
HEART
FAILURE
LV is the initiating event in
most cases of combined
failure.
The main effects are the
results of:
(a) Low output
(b) ‘Backward’ pressure.*
Cyanosis is the result of
excess reduced
haemoglobin in capillaries
and venules.
Left Sided Failure
•Low output vs. congestion
•Lungs
– pulmonary congestion and edema
– heart failure cells
•Kidneys
– pre-renal azotemia
– salt and fluid retention
• renin-aldosterone activation
• natriuretic peptides
•Brain: Irritability, decreased attention, stupor🡪coma
Left Sided Failure
•Dyspnea
– on exertion
– at rest

•Orthopnea
– redistribution of peripheral edema fluid
– graded by number of pillows needed

•Paroxysmal Nocturnal Dyspnea (PND)

LEFT Heart Failure
Dyspnea
Orthopnea
PND (Paroxysmal Nocturnal
Dyspnea)
Blood tinged sputum
Cyanosis
Elevated pulmonary
“WEDGE” pressure (PCWP)
ACUTE
PULMONARY
EDEMA
In acute left
ventricular failure,
accumulation of fluid
results in dyspnoea
and hypoxaemia.
 CHRONIC
PULMONAR
Y EDEMA
CHRONIC VENOUS
CONGESTION OF THE LUNGS

oThe results of chronic combined

(congestive) failure and/or
chronic left ventricular failure:

oThe lungs are bulky, congested

and brownish in colour.
 Right Sided
Heart Failure
•Etiology
– left heart failure
– cor pulmonale
•Symptoms and signs
– Liver and spleen
• passive congestion (nutmeg liver)
• congestive splenomegaly
• ascites
– Kidneys
– Pleura/Pericardium
• pleural and pericardial effusions
• transudates
– Peripheral tissues
RIGHT Heart Failure
FATIGUE
“Dependent” edema
JVD
Hepatomegaly (congestion)
ASCITES, PLEURAL EFFUSION
GI
Cyanosis
Increased peripheral venous
pressure (CVP)
CONGESTIV
E CARDIAC
FAILURE
Congestion of the
systemic venous
circulation is a result of
right heart failure
whether secondary to
left heart failure or
lung disease (cor
pulmonale).
CONGESTIVE CARDIAC
FAILURE
•TRIAD
– 1) TACHYCARDIA
– 2) DYSPNEA
– 3) EDEMA
•FAILURE of Frank Starling mechanism
•HUMORAL FACTORS
– Catecholamines (nor-epinephrine)
– ReninAngiotensionAldosterone
– Atrial Natriuretic Polypeptide (ANP)
•HYPERTROPHY and DILATATION
CONGESTIVE
CARDIAC FAILURE
•Findings:
•Cardiomegaly
•Chamber Dilatation
•Hypertrophy of myocardial fibers,
•BOXCAR nuclei
 CONGESTIV
E CARDIAC
FAILURE
🡪The liver shows striking
changes:
🡪distension of the veins causes
enlargement of the liver so that
it can be felt below the costal
margin.
🡪The parenchymal cells furthest
from the arterial blood supply,
i.e. around the hepatic venules,
undergo degeneration with
atrophy and ultimately
disappear.
🡪Cells nearer the arteries show
an accumulation of fat.
LOW AND
HIGH OUTPUT
FAILURE
By definition, in cardiac failure output is low with
respect to body requirements, and in most cases
this output is lower than the normal output. Such
cases are called low output type.

In a few conditions, the cardiac failure

complicates a pre-existing state in which the
output before failure was greater than normal.
◦ In these cases, the output is not sufficient to
meet the body requirements but may still be
higher than the normal.

Such cases are called high output type.

DISEASES OF Arterial diseases are very common and are important because of their serious effects,
especially on the heart and brain.

ARTERIES – Normal age-related vascular changes:

ARTERIOSCLER ARTERIOSCLEROSIS (hardening of the arteries)

◦ This is a generalised degeneration of the specialised muscle and elastic tissue of

OSIS the media of the vessel wall, and replacement by fibrous tissue, proteoglycan and
calcium salts.
◦ There is also proliferation of the inner layer of the vessel (intima).
DISEASES OF ARTERIES –
ARTERIOSCLEROSIS
HYALINE ARTERIOLOSCLEROSIS affects the
small branches of the arterial system (arterioles)
– especially in the kidney.
This is due to entry of blood plasma under the
endothelium with protein deposition; gradual
replacement by collagen occurs. The process is
called plasmatic vasculosis.
In a variant of arteriosclerosis (Monckeberg),
seen in the very elderly, calcium salts are very
heavily deposited in the media particularly of the
leg arteries.
ATHEROMA
(ATHEROSCLEROSIS
)
Atheroma is a common disease in Westernised
countries; it is of gradual onset but is
progressive.
It is commonly seen in the middle-aged and
elderly people, but the earliest changes are
present in adolescents.
It is often asymptomatic but it may cause serious
disease or death from complicating thrombosis.
The lesions represent patchy deposition of lipid
within plaques deep in the intima.
ATHEROMA –
COMPLICATIONS
Fibrosis in the intima and media weaken the wall and may lead to aneurysm
formation, but the most important complication of atheroma is thrombosis.

Local factors at the site of the atheromatous plaque are important:

◦ 1. Intimal roughening or distortion with eddying of flow.
◦ 2. Thin, easily damaged intimal surface.
◦ 3. Rupture of new capillaries in plaque with haemorrhage into the plaque

In addition, general factors influencing blood coagulability are important, e.g.

smoking affecting vessel contractility and platelet function.
ATHEROMA – ETIOLOGY

FIXED

SEX – male > female

HEREDITY

(i) Family history of premature heart disease

(ii) Familial hyperlipidaemias

◦ Increased plasma cholesterol is an important risk factor and the ratio of low-density lipoprotein (LDL)
to high density lipoprotein (HDL) especially, so: LDL increases risk; HDL decreases risk
ATHEROMA – ETIOLOGY

MODIFIABLE
ENVIRONMENTAL
(a) SMOKING
(b) DIET – rich in saturated fats and cholesterol and low in fruit and
vegetables
(c) Lack of exercise
ATHEROMA – ETIOLOGY

DISEASES
(a) HYPERTENSION
(b) Hyperlipidaemia
(c) Diabetes mellitus
(d) Obesity
(e) Increased fibrinogen levels
Atherosclerosis
Atherosclerosis is a focal intimal disease - each
discrete lesion being called
a plaque.

Lipid is an essential component of the

atherosclerotic disease process.
PATHOGENESIS OF ENDOTHELIAL DAMAGE

ATHEROSCLEROSIS
 PATHOGENESIS OF
ATHEROSCLEROSIS
Endothelial injury: increased vascular permeability, leukocyte adhesion, thrombosis
Accumulation of lipoproteins: LDL
Monocyte adhesion to the endothelium, followed by migration into the intima and
transformation into macrophages and foam cells
Platelet adhesion
Factor release from activated platelets, macrophages, and vascular wall cells
Smooth muscle cell proliferation and ECM production
Lipid accumulation both extracellularly and within cells
The major components of a
well-developed intimal atheromatous
plaque overlying an intact media
EPIDEMIOLOGY
Virtually ubiquitous among most developed nations
prevalence and severity of atherosclerosis and IHD
◦ related to several risk factors
◦ constitutional (and therefore less controllable)
◦ acquired or related to behaviors that are potentially amenable to intervention
 MAJOR RISK FACTORS FOR
ATHEROSCLEROSIS
NONMODIFIABLE
Increasing age Family History

Male gender Genetic abnormalities

MODIFIABLE
Hyperlipidemia Diabetes

Hypertension C-reactive protein

Cigarette snoking
 Atherosclerotic plaques are
susceptible to the following clinically
important changes
Rupture, ulceration, or erosion
◦exposes the blood to highly thrombogenic substances and induces
thrombosis
◦can partially or completely occlude the lumen and lead to downstream
ischemia

Hemorrhage into a plaque

◦Rupture of the overlying fibrous cap, or of the thin-walled vessels in the
areas of neovascularization
◦ a contained hematoma may expand the plaque or induce plaque rupture.
Atherosclerotic plaques are
susceptible to the following clinically
important changes
Atheroembolism
◦ microemboli
Aneurysm formation
◦ Atherosclerosis-induced pressure or ischemic atrophy of the
underlying media
◦ vessel weaknes from loss of elastic tissue
◦aneurysmal dilation and potential rupture
CONSEQUENCES OF
ATHEROSCLEROTIC DISEASE
MAJOR TARGETS OF ATHEROSCLEROSIS
◦ Large elastic arteries : aorta, carotid, iliac arteries
◦ Large and medium sized muscular arteries : coronary, popliteal
arteries
MAJOR CONSEQUENCES OF ATHEROSCLEROSIS
◦MI - aortic aneurysm
◦Cerebral Infarct - peripheral vascular disease
The principal outcomes depend on:
◦ the size of the involved vessels
◦ the relative stability of the plaque itself
◦degree of degeneration of the underlying arterial wall

❑Smaller vessels can become occluded, compromising distal tissue perfusion.

❑ Ruptured plaque can embolize atherosclerotic debris and cause distal vessel
obstruction, or can lead to acute (and frequently catastrophic) vascular thrombosis.
❑ Destruction of the underlying vessel wall can lead to aneurysm formation, with
secondary rupture and/or thrombosis.
HYPERTENSION
ESSENTIAL
HYPERTENSI
ON
In 95% of cases of hypertension no
obvious cause is found – this is
ESSENTIAL hypertension – (a diagnosis
of exclusion).

A number of aetiological factors may be

implicated:

1. Genetic predisposition – polygenic

inheritance.

2. Racial, e.g. black > white.

3. Sodium homeostasis: increased salt

intake.

4. Lack of exercise.
 Five percent of hypertension is a complication of other
diseases. They are broadly classified as:
(a) Kidney diseases, e.g. chronic renal failure, renal
artery stenosis, polycystic kidneys. The mechanism is
usually renal ischaemia with activation of the
renin-angiotensin system.
SECONDARY (b) Endocrine disorders – Cushing syndrome –
HYPERTENSI corticosteroid excess; Conn syndrome – aldosterone
excess; Phaeochromocytoma – catecholamine
ON excess.
(c) Coarctation of aorta – aortic narrowing → renal
perfusion impaired → renin-angiotensin activation.
(d) Eclampsia and pre-eclampsia in pregnancy.
(e) Drugs, e.g. steroids, oral contraceptives.
CLASSIFICATION –
BENIGN AND
MALIGNANT
 generic designation for a group of pathophysiologically
ISCHEMI related syndromes resulting from myocardial ischemia
◦ imbalance between the supply (perfusion) and demand of the

C HEART heart for oxygenated blood

◦ reduces the availability of nutrients and the removal of
DISEASE metabolites
CLINICAL SYNDROME OF IHD
Myocardial infarction, the most important form of IHD, in which ischemia causes
the death of heart muscle.
• Angina pectoris, in which the ischemia is of insufficient severity to cause
infarction, but may be a harbinger of MI.
• Chronic IHD with heart failure.
• Sudden cardiac death
EPIDEMIOLOGY
leading cause of death for both males and females in the United States and other industrialized
nations
overall death rate from IHD has fallen in the United States by approximately 50%

◦ (1) prevention, achieved by modification of important risk factors, such as smoking, elevated blood cholesterol,
and hypertension

◦ (2) diagnostic modalities

(3) therapeutic advances
◦ new medications
◦ percutaneous transluminal coronary angioplasty
◦ coronary artery bypass graft (CABG) surgery
◦ risk reduction: maintenance of normal blood glucose levels in diabetic patients, control of obesity, and
prophylactic anticoagulation of middle-aged men with aspirin
◦ coronary care units
◦ Thrombolysis for MI
◦ Endovasclar stents
PATHOGENESIS
insufficient coronary perfusion relative to myocardial demand
chronic, progressive atherosclerotic narrowing of the epicardial coronary arteries
variable degrees of superimposed acute plaque change
Thrombosis
vasospasm
CHRONIC ATHEROSCLEROSIS
90% with IHD have ATHEROSCLEROSIS
Progressive narrowing of the lumen: stenosis or acute plaque disruption with thrombosis →
compromise blood flow
≥ 75% obstruction → symptomatic ischemia with exercise
90% obstruction → inadequate blood flow even at rest
Role of collateral vessels
LAD, LCX, RCA and major secondary epicardial branches
 ACUTE PLAQUE CHANGE
Number
Distribution
Structure
Degree of obstruction

❑ spectrum of acute alterations in atherosclerotic lesions

(plaque disruption or plaque change)
CONSEQUENCES OF MYOCARDIAL ISCHEMIA
Stable angina
◦ increases in myocardial oxygen demand that outstrip the ability of stenosed coronary arteries to increase
oxygen delivery
◦ not associated with plaque disruption

Unstable angina
◦ plaque rupture complicated by partially occlusive thrombosis and vasoconstriction
◦ Microinfarct: thromboemboli

Myocardial Infarction
Sudden cardiac death
◦ atherosclerotic lesion with disrupted plaque → regional myocardial ischemia → fatal ventricular arrhythmia
 Coronary Artery Pathology in Ischemic Heart Disease
Plaque
Syndrome Stenoses Disruption Plaque-Associated Thrombus
Stable angina >75% No No
Unstable angina Variable Frequent Nonocclusive, often with thromboemboli
Transmural Variable Frequent Occlusive
myocardial infarction
Subendocardial Variable Variable Widely variable, may be absent,
myocardial infarction partial/complete, or lysed
Sudden death Usually Frequent Often small platelet aggregates or thrombi
severe and/or thromboemboli
ANGINA PECTORIS
Chest pain
paroxysmal and usually recurrent attacks of substernal or precordial chest
discomfort (constricting, squeezing, choking, knifelike)
caused by transient (15 seconds to 15 minutes) myocardial ischemia that falls short
of inducing myocyte necrosis
PATTERNS OF ANGINA PECTORIS
STABLE ANGINA
most common form; “typical angina pectoris”
imbalance in coronary perfusion (due to chronic stenosing coronary
atherosclerosis) relative to myocardial demand
Relieved by rest and nitroglycerine
PRINZMETAL ANGINA
Uncommon; caused by coronary artery spasm
Attacks unrelated to physical activity, heart rate or blood pressure
Responds to vasodilator: nitroglycerine & calcium channel blockers
UNSTABLE OR CRESCENDO ANGINA
Pattern of increasingly frequent pain, prolonged duration
Precipitated by low level activity / rest
disruption of an atherosclerotic plaque; superimposed partial (mural) thrombosis,
embolization or vasospasm
warning of an imminent acute MI “preinfarction angina”
MYOCARDIAL INFARCTION
Heart attack; cardiac muscle death due to prolonged severe ischemia
Most important form of IHD
Atherosclerosis
Incidence rises with age
M>F
10%: Transmural MI occurs in the absence of typical coronary pathology
◦ Vasospasm
◦ Emboli
◦ Ischemia without atherosclerosis/thrombosis
Coronary arterial obstruction →
compromised blood supply to a region of
myocardium → ischemia, myocardial
dysfunction, and potentially myocyte death.
“AREA AT RISK” : anatomic region
supplied by an artery
The outcome depends predominantly on the
severity and duration of flow deprivation
 MYOCARDIAL RESPONSE
Feature Time
Onset of ATP depletion Seconds
Loss of contractility <2 min
ATP reduced
to 50% of normal 10 min
to 10% of normal 40 min
Irreversible cell injury 20–40 min
Microvascular injury >1 hr
PROGRESSION OF NECROSIS
Consequences of myocardial
ischemia followed by reperfusion
FREQUENCIES OF INVOLVEMENT OF EACH OF THE THREE
MAIN ARTERIAL TRUNKS AND THE CORRESPONDING SITES
OF MYOCARDIAL LESIONS RESULTING IN INFARCTION

Left anterior descending coronary artery (40% to 50%): anterior wall of left
ventricle near the apex; the anterior portion of ventricular septum; and the apex
circumferentially
Right coronary artery (30% to 40%): inferior/posterior wall of left ventricle;
posterior portion of ventricular septum; and the inferior/posterior right ventricular
free wall in some cases
Left circumflex coronary artery (15% to 20%): lateral wall of left ventricle except
at the apex
TIMING of Gross and Microscopic Findings
½–4 hr None Usually none; variable waviness of fibers at border

4–12 hr Occasionally dark mottling Beginning coagulation necrosis; edema; hemorrhage

12–24 hr Dark mottling Ongoing coagulation necrosis; pyknosis of nuclei;

myocyte hypereosinophilia; marginal contraction
band necrosis; beginning neutrophilic infiltrate
1–3 days Mottling with yellow-tan Coagulation necrosis, with loss of nuclei and striations;
infarct center interstitial infiltrate of neutrophils
3–7 days Hyperemic border; central Beginning disintegration of dead myofibers, with dying
yellow-tan softening neutrophils; early phagocytosis of dead cells by
macrophages at infarct border
7–10 Maximally yellow-tan and Well-developed phagocytosis of dead cells; early
days soft, with depressed formation of fibrovascular granulation tissue at
red-tan margins margins
10–14 Red-gray depressed infarct Well-established granulation tissue with new blood
days borders vessels and collagen deposition

2–8 wk Gray-white scar, progressive Increased collagen deposition, with decreased cellularity
from border toward core
of infarct
>2 mo Scarring complete Dense collagenous scar
 Acute myocardial infarct,
predominantly of the
posterolateral left
ventricle
Microscopic features of myocardial
infarction and its repair
INFARCT MODIFICATION BY REPERFUSION
most effective way to “rescue” ischemic myocardium threatened by infarction is to restore
myocardial blood flow as rapidly as possible
may trigger deleterious complications:
◦ arrhythmias
◦ myocardial hemorrhage with contraction bands
◦ irreversible cell damage superimposed on the original ischemic injury (reperfusion injury)
◦ microvascular injury
◦ prolonged ischemic dysfunction (myocardial stunning)
Coronary intervention:
◦ Thrombolysis
◦ Angioplasty
◦ stent placement
◦ coronary artery bypass graft [CABG] surgery

dissolve, mechanically alter, or bypass the lesion that initiated the acute MI
AMI DIAGNOSIS
CLINICAL SYMPTOMS
◦ rapid, weak pulse and profuse sweating (diaphoresis)
◦ Dyspnea due to impaired contractility of the ischemic myocardium
◦ pulmonary congestion and edema
◦ “silent” MI: asymptomatic detected by ECG changes
LABORATORY EVALUATION
◦ Blood levels of proteins
◦ Myoglobulin
◦ Cardiac troponins T and I: most sensitive and specific
◦ Creatine kinase (MB): sensitive but not specific
◦ Lactate dehydrogenase

ELECTROCARDIOGRAM
 COMPLICATIONS
Wall motion abnormalities
Arrhythmias
Rupture (3 - 7 days) / ( 4-5 days) / (range 1-10 days
Pericarditis
RV infarction
Infarct extension, expansion
Mural thrombus
Ventricular aneurysm
Papillary muscle dysfunction (regurgitation)
Progressive late heart failure
Complications of myocardial infarction
CHRONIC ISCHEMIC HEART
DISEASE (IHD)
progressive heart failure as a consequence of ischemic myocardial damage
“ischemic cardiomyopathy”
has been prior MI and sometimes previous coronary arterial interventions and/or
bypass surgery
appears postinfarction due to the functional decompensation of hypertrophied
noninfarcted myocardium
Morphology
enlarged and heavy heart
Left ventricular hypertrophy and dilatation
obstructive coronary atherosclerosis
Discrete scars
mural endocardium may have patchy, fibrous thickenings, and mural thrombi may be present
Microscopic findings:
◦ myocardial hypertrophy
◦ diffuse subendocardial vacuolization
◦ fibrosis.
SUDDEN CARDIAC DEATH
unexpected death from cardiac causes in individuals without symptomatic heart
disease or early after symptom onset (usually within 1 hour)
consequence of a lethal arrhythmia (e.g., asystole, ventricular fibrillation)
Acute myocardial ischemia is the most common trigger for fatal arrhythmias
Nonatherosclerotic conditions associated with SCD include
◦ Congenital structural or coronary arterial abnormalities
◦ Aortic valve stenosis
◦ Mitral valve prolapse
◦ Myocarditis
◦ Dilated or hypertrophic cardiomyopathy
◦ Pulmonary hypertension
◦ Hereditary or acquired cardiac arrhythmias
◦ Cardiac hypertrophy of any cause (e.g., hypertension)
◦ Other miscellaneous causes, such as systemic metabolic and hemodynamic alterations, catecholamines, and
drugs of abuse, particularly cocaine and methamphetamine.
Morphology
80-90 % SCD: marked coronary atherosclerosis 75% stenosis ( 1 or more major vessels)
10-20%: nonatherosclerotic origin
most SCD is not associated with acute MI
most of these deaths are thought to result from myocardial ischemia–induced irritability that
initiates malignant ventricular arrhythmias
Scars of previous infarcts and subendocardial myocyte vacuolization indicative of severe
chronic ischemia
Constitutional risk factors in IHD
AGE
◦ dominant influence
◦ progressive
◦ 40 to 60 y/o: MI
◦ Deaths from IHD

GENDER
◦ Premenopausal women

GENETICS
◦ family history : most significant risk factor for atherosclerosis
◦ mendelian disorders associated with atherosclerosis
 Modifiable risk factors in IHD
HYPERLIPIDEMIA
◦ hypercholesterolemia: major risk factor for atherosclerosis
◦ HDL vs LDL

DIETARY AND PHARMACOLOGIC APPROACHES THAT LOWER

LDL OR TOTAL SERUM CHOLESTEROL, AND/OR RAISE SERUM
HDL
◦ Cholesterol / saturated fats vs polyunsaturated fats / omega-3 FA
◦ Exercise, moderate consumption of ethanol : raise HDL
◦ Obesity, smoking
◦ Statins: inhibiting hydroxymethylglutaryl coenzyme A (HMG-CoA) reductase, the
rate-limiting enzyme in hepatic cholesterol biosynthesis
Modifiable risk factors in IHD
◦HYPERTENSION
◦Increase risk of IHD by 60%
◦Left ventricular hypertrophy
◦CIGARETTE SMOKING
◦Prolonged (years) smoking of one pack of cigarettes or more daily doubles
the death rate from IHD
◦DIABETES MELLITUS
◦Hypercholesterolemia
◦increased risk of strokes and a 100-fold increased risk of
atherosclerosis-induced gangrene of the lower extremities
Additional risk factors
INFLAMMATION
◦ present during all stages of atherogenesis and is intimately linked with atherosclerotic plaque
formation and rupture
◦ C-reactive protein (CRP)
HYPERHOMOCYSTINEMIA
METABOLIC SYNDROME
LIPOPROTEIN
FACTORS AFFECTING HEMOSTASIS
◦ plasminogen activator inhibitor 1
◦ Thrombin, PDF
OTHER FACTORS: lifestyle, personality
Cardiomyopathies
Causes can be primary (i.e., predominantly affecting heart) or secondary (i.e., part of a
larger systemic disorder):
◦ Infections (e.g., viral, bacterial, fungal, protozoal)
◦ Toxic exposures (e.g., alcohol, cobalt, chemotherapeutic agents)
◦ Metabolic disorders (e.g., hyperthyroidism, nutritional deficiency)
◦ Genetic abnormalities in cardiomyocytes (e.g., storage disorders, muscular dystrophies)
◦ Infiltrative lesions (e.g., sarcoid, carcinoma, radiation-induced fibrosis)
◦ Immunologic disorders (e.g., autoimmune myocarditis, rejection)

Cardiomyopathy is divided into three main functional and pathologic patterns: dilated,
hypertrophic, and restrictive
Dilated Cardiomyopathy
characterized by gradual fourchamberhypertrophy and dilation; there is systolic
dysfunction with hypocontraction
presents as indolent, progressive CHF.
only 25% of patients survive more than 5 years after symptom onset.
◦ Although the cause is frequently unknown (idiopathic DCM), certain pathologic mechanisms can
contribute
Dilated Cardiomyopathy
Genetic influences:
◦ 20% to 50% of DCM is familial; autosomal dominant inheritance is most common.

Alcohol toxicity:
◦ DCM is attributed to direct toxicity of alcohol or a metabolite (especially acetaldehyde) on the
myocardium.

Peripartum cardiomyopathy:
◦ DCM is discovered within several months before or after delivery

Myocarditis
Dilated Cardiomyopathy
Grossly, the heart is flabby with
cardiomegaly (i.e., up to 900 g); wall
thickness may not reflect the degree of
hypertrophy due to chamber dilation.
Poor contractile function and stasis
predispose to mural thrombi.
Valves and coronary arteries are generally
normal.
Microscopic changes in DCM are often
subtle and entirely nonspecific; most
commonly there is diffuse myocyte
hypertrophy and variable interstitial fibrosis.
Dilated Cardiomyopathy
Generalized enlargement of the heart is seen upon normal chest X-ray.
◦ Pleural effusion may also be noticed, which is due to pulmonary venous hypertension.

ECG often shows sinus tachycardia or atrial fibrillation, ventricular arrhythmias, left
atrial enlargement, and sometimes intraventricular conduction defects and low
voltage.
Genetic testing can be important, since one study has shown that gene mutations in
the TTN gene (which codes for a protein called titin) are responsible for
"approximately 25% of familial cases of idiopathic dilated cardiomyopathy and 18%
of sporadic cases.
Dilated Cardiomyopathy
Standard therapy may include salt restriction, ACE inhibitors, diuretics, and beta
blockers.
◦ Anticoagulants may also be used for antithrombotic therapy

Artificial pacemakers may be used in patients with intraventricular conduction delay,

and implantable cardioverter-defibrillators in those at risk of arrhythmia
Arrhythmogenic Right Ventricular
Cardiomyopathy
Recently recognized cardiomyopathy with distinct presentation and morphology.
an autosomal dominant disorder (with variable penetrance) characterized by
predominantly right-sided failure and arrhythmia.
◦ most commonly caused by defective adhesive molecules in desmosomes.

Morphologically, the right ventricular wall is severely thinned with myocyte loss and
profound fatty infiltration.
Death occurs secondary to progressive CHF or fatal arrhythmias.
Hypertrophic Cardiomyopathy
is characterized by heavy, muscular, hypercontractile, poorly compliant hearts with
poor diastolic relaxation;
◦ in a third of cases there is also ventricular outflow obstruction.
Hypertrophic Cardiomyopathy
is predominantly caused by mutations of sarcomeric proteins (b-myosin heavy chain
mutations being most common);
◦ most are autosomal dominant mutations with variable penetrance.

Prognosis varies widely depending on the specific mutations.

Hypertrophic Cardiomyopathy
The major feature is reduced stroke volume due to a combination of impaired
diastolic filling and left ventricular outflow tract obstruction.
Focal myocardial ischemia is common due to increased ventricular pressures,
massive myocyte hypertrophy, diminished stroke volume
Can be entirely asymptomatic.
Symptomatic disease usually presents in young adults with dyspnea, angina, and/or
syncope.
Hypertrophic Cardiomyopathy
The clinical course can be highly variable; major complications include atrial
fibrillation with mural thrombus and embolization, IE, CHF, and SCD.
HCM is one of the most common causes of sudden death in young athletes.
Hypertrophic Cardiomyopathy
there is disproportionate thickening of the
interventricular septum (asymmetric septal
hypertrophy
The left ventricular cavity is compressed into a
banana-like configuration by the asymmetric
bulging of the septum.
Septal thickening at the level of the mitral valve
compromises left ventricular systolic outflow by
contact of the anterior mitral leaflet with the
septum (systolic anterior motion);
◦ this causes hypertrophic obstructive cardiomyopathy,
reflected by a fibrous plaque on the septum.
Hypertrophic Cardiomyopathy
significant number of people with hypertrophic cardiomyopathy do not have any
symptoms and will have normal life expectancies,
◦ although they should avoid particularly strenuous activities or competitive athletics, and should be
screened for risk factors for sudden cardiac death

primary goal of medications is to relieve symptoms such as chest pain, shortness of

breath, and palpitations.
◦ Beta blockers are considered first-line agents, as they can slow down the heart rate and decrease
the likelihood of ectopic beats.
Hypertrophic Cardiomyopathy
Surgical septal myectomy is an open-heart operation done to relieve symptoms in
people who remain severely symptomatic despite medical therapy.
Prognosis and Consequences
Restrictive Cardiomyopathy
Relatively rare and with multiple etiologies, this entity is marked by a restriction of
ventricular filling leading to reduced cardiac output.
Contractile function is usually normal.
V entricle size is normal, although there is typically biatrial dilation.
Non-specific interstitial myocardial fibrosis is usually present; biopsy frequently
reveals a specific etiology.
Restrictive Cardiomyopathy
Causes
Endomyocardial fibrosis
◦ disease mainly of African children and young adults; the cause is unknown.

Loeffler endocarditis
◦ morphologically similar to endomyocardial fibrosis but is classically associated with peripheral eosinophilia
and eosinophilic infiltration of multiple organs.

Endocardial fibroelastosis
◦ an uncommon disorder of obscure etiology (and possibly the end point of different injuries), characterized
by focal to diffuse, fibroelastic thickening of the endocardium, and left ventricle greater than right.
Myocarditis
Infectious etiologies or primary autoimmune responses underlie myocarditis.
The clinical spectrum is broad, from entirely asymptomatic to abrupt onset of
arrhythmia, CHF, or SCD; most patients recover quickly and without sequelae,
although DCM can occur
Myocarditis
Most cases in the United States are viral in origin (e.g., Coxsackievirus A and B,
echovirus).
Cardiac involvement occurs days to weeks after a primary viral infection;
◦ cardiac involvement can be due to a direct infection or secondary to immunologic cross-reactivity
between pathogen and myocardium.
Myocarditis
Trypanosoma cruzi (causal organism in Chagas disease) causes myocarditis in the
majority of infected individuals with 10% dying acutely and others progressing to
cardiac failure over 10 to 20 years.
Toxin released by Corynebacterium diptheriae is responsible for the myocardial
injury in diptheria.
Myocarditis
Myocarditis occurs in 5% of patients with Lyme disease (Borrelia burgdorferi).
Lyme myocarditis is usually mild and reversible but occasionally requires a
temporary pacemaker for atrioventricular block.
Myocarditis
Myocarditis in acquired immunodeficiency syndrome (AIDS) patients results from
inflammation and damage without a clear etiologic agent
Noninfectious myocarditis may be immune mediated (e.g., associated with rheumatic
fever, systemic lupus erythematosus, or drug allergies).
In some cases, the cause is unknown (e.g., sarcoidosis, giant cell myocarditis) or the
microbe is unidentifiable.
Myocardits Microscopic
Chagasic myocarditis,
microscopic
Myocarditis
As with most viral infections, symptomatic treatment is the only form of therapy for most
forms of myocarditis
In people with symptoms, digoxin and diuretics may help.
◦ For people with moderate to severe dysfunction, cardiac function can be supported by use
of inotropes such as milrinone in the acute phase, followed by oral therapy with ACE inhibitors when
tolerated.
Systemic corticosteroids may have beneficial effects in people with proven myocarditis
People who do not respond to conventional therapy may be candidates for bridge
therapy with left ventricular assist devices.
Heart transplantation is reserved for people who fail to improve with conventional therapy
PERICARDITIS
SEROUS: Rheum. Fever (RF), SLE, scleroderma,
tumors, uremia
FIBRINOUS: MI (Dressler), uremia, radiation, RF, SLE,
s/p open heart surgery
PURULENT: infective, bacterial
HEMORRHAGIC: Malignancy, TB
CASEOUS: TB
CHRONIC: (ADHESIVE, CONSTRICTIVE)
Valvular HD
Opening problems: Stenosis
Closing problems: Regurgitation or Incompetence
70% of all VHD:
AS
◦ Calcification of a deformed valve
◦ “Senile” calcific AS
◦ Rheum, Heart Dis.
MS
◦ Rheumatic Heart Disease
AORTIC STENOSIS
2X gradient pressure
LVH, ischemia
Cardiac decompensation, angina, CHF
50% die in 5 years if angina present
50% die in 2 years if CHF present
RHEUMATIC FEVER

In rheumatic fever (acute rheumatism), a disease of children and young adults, inflammation
affects the connective tissues at many sites, of which the heart is the most important.
The incidence in Western countries has fallen dramatically, but it is common in parts of Africa and
Asia.
RHEUMATIC FEVER

1. General manifestations include:

Fever with sweating and malaise: raised Erythrocyte sedimentation rate and C-reactive protein:
neutrophil leucocytosis.
2. Localised inflammation
(a) Joints and adjacent musculofascial tissues – causing POLYARTHRITIS with effusion, muscle
pains and weakness.
(b) Serous membranes – pericardial and sometimes pleural effusion.
(c) Skin (i) a rash (erythema marginatum).
(ii) small subcutaneous palpable nodules over bony prominences subject to pressure.
 CARDIAC MANIFESTATIONS
The inflammation is widespread throughout the
heart (PANCARDITIS) – affecting the
pericardium, myocardium and endocardium.
1. Pericarditis occurs during the acute phase of
RHEUMAT the illness and is an important cause of
IC FEVER pericardial effusion.
2. Myocarditis is common during the acute
phase and is usually mild: it is rarely severe
enough to cause cardiac failure. The histological
picture is striking and the presence of the Aschoff
body is characteristic.
 3. Endocarditis.
Although there is a widespread histological
inflammation, gross changes are seen usually on
the endocardial sites subject to the greatest
RHEUMAT pressures and traumas
IC FEVER ◦ i.e. in the left side of the heart at the points of valve
closure and at any sites of jet effect in the blood flow.

The inflammation is complicated by ulceration of

the valve surface followed by platelet and fibrin
thrombosis in the form of small ‘vegetations’.
ACUTE:
-Inflammation
-Aschoff bodies
-Anitschkow cells
-Pancarditis
-Vegetations on
chordae tendinae at
leaflet junction

CHRONIC:
THICKENED VALVES
COMMISURAL FUSION
THICK, SHORT,
CHORDAE TENDINAE
CLINICAL
FEATURES
Migratory Polyarthritis
Myocarditis
Subcutaneous nodules
Erythema marginatum
Sydenham chorea
 REGURGITATIONS
AR
◦ Rheumatic
◦ Infectious
◦ Aortic dilatations
◦ Syphilis
◦ Rheumatoid Arthritis
◦ Marfan

MR
◦MVP
◦ Infectious
◦ Papillary muscles, chordae tendinae
◦ Calcification of mitral ring (annulus)
Mitral Valve Prolapse (MVP)
MYXOMATOUS degeneration of the mitral valve
Associated with connective tissue disorders
“Floppy” valve
3% incidence, F>>M
Easily seen on echocardiogram
 MVP: CLINICAL FEATURES
Usually asymptomatic
Mid-systolic “click”
Holosystolic murmur if regurg. present
Occasional chest pain, dyspnea
97% NO untoward effects
3% Infective endocarditis, mitral insufficiency, arrythmias, sudden death
INFECTIVE
ENDOCARDITIS
VEGETATIONS
INFECTIVE >5mm
NON-Infective <5mm
 DIAGNOSIS

MAJOR
Positive blood culture(s) indicating characteristic
organism or persistence of unusual organism
Echocardiographic findings, including valve-related or
implant-related mass or abscess, or partial separation of
artificial valve
New valvular regurgitation
 DIAGNOSIS

minor
Predisposing heart lesion or intravenous drug use
Fever
Vascular lesions, including arterial petechiae, subungual/splinter
hemorrhages, emboli, septic infarcts, mycotic aneurysm, intracranial
hemorrhage, Janeway lesions
Immunologic phenomena, including glomerulonephritis, Osler nodes, Roth
spots, rheumatoid factor
Microbiologic evidence, including single culture showing uncharacteristic
organism
Echocardiographic findings consistent with but not diagnostic of
endocarditis, including new valvular regurgitation, pericarditis
 MARANTIC ENDOCARDITIS – in debilitated
patients (often with disseminated malignancy),
thrombus may form on the closure line of the
valve cusps.
LIBMAN-SACKS DISEASE – thrombotic
Non vegetations can occur in systemic lupus
infective erythematosus (SLE) and cause embolic
complications
Endocarditis CARCINOID SYNDROME – excess
5-hydroxytrptamine secretion for metastatic
carcinoid tumours can result in right heart
endocardial fibrosis leading to stenosis or
incompetence of the tricuspid valve.
 Developmental abnormalities of the heart are
relatively common; clinically significant defects
CONGENIT occur in 7–11 per 1000 live births and the rate is
significantly higher in stillbirths.
AL HEART The severity varies from minor aberrations to
DISEASE complex distortions incompatible with life.
Many patients now live normal lives after cardiac
surgery.
CONGENITAL HEART
DISEASE

This is often obscure. Hereditary factors are of limited importance, Down

syndrome often causes septal defects. Possible environmental factors are
complex and numerous.
Examples include: virus infection, e.g. rubella; teratogen, e.g. thalidomide
(historically); altitude at birth (patent ductus arteriosus is commoner at high
altitudes).
The initial damage, which is sustained during the first trimester of pregnancy, is
modified by later fetal and postnatal growth and development.
Single anatomical abnormalities occur, but often there are multiple defects which
are associated in groups of which the more common are given specific names.
 Congenital Heart Disease
•Faulty embryogenesis (week 3-8)
•Usually a SINGLE lesion (ASD, VSD, hypo-RV, hypo-LV)
•May not be evident until adult life (Coarctation, ASD)
•Overall incidence 1% of USA births
•INCREASED simple early detection via non invasive methods,
e.g., US, MRI, CT, etc.
 Incidence per Million Live
Malformation Births %

Ventricular septal defect 4482 42

1043 10
Atrial septal defect
Pulmonary stenosis 836 8
781 7
Patent ductus arteriosus
577 5
Tetralogy of Fallot
Coarctation of aorta 492 5

Atrioventricular septal defect

396 4
Aortic stenosis

Transposition of great arteries 388 4

Truncus arteriosus 388 4
Total anomalous pulmonary venous connection 136 1
Tricuspid atresia 120 1
Congenital Heart
Disease
•L🡪R SHUNTS: all “D’s” in their names
– NO cyanosis
– Pulmonary hypertension
– SIGNIFICANT pulmonary hypertension is IRREVERSIBLE

•R🡪L SHUNTS: all “T’s” in their names

– CYANOSIS
– VENOUS EMBOLI become SYSTEMIC

•OBSTRUCTIONS
Left to Right NON CYANOTIC
•ASD
IRREVERSIBLE:
•VSD PULMONARY
HYPERTENSION
•ASVD IS THE MOST
FEARED
•PDA CONSEQUENCE
Atrial Septal Defect
•Non patent foramen ovale
•Usually asymptomatic until adulthood
•SECUNDUM (90%): Defective fossa ovalis
•PRIMUM (5%): Next to AV valves, mitral cleft

•SINUS VENOSUS (5%): Next to SVC with anomalous pulmonary

veins draining to SVC or RA
Ventricular Septal
Defect
•By far, most common CHD defect
•Only 30% are isolated
•Often with TETRALOGY of FALLOT
•90% involve the membranous septum
•If muscular septum is involved, likely to have multiple holes
•SMALL ones often close spontaneously
•LARGE ones progress to pulmonary hypertension
Patent Ductus
Arteriosus
•90% isolated
•HARSH, machinery-like murmur
•L🡪R, possibly R🡪L as pulmonary hypertension approaches
systemic pressure
•Closing the defect may be life saving
•Keeping it open may be life saving (Prostaglandin E). Why?
Atrio-Ventricular
Septal Defect
•Associated with defective, inadequate AV valves
•Can be partial, or COMPLETE (ALL 4 CHAMBERS FREELY
COMMUNICATE)
 Right to Left Shunts
•Tetralogy of Fallot
•Transposition of great arteries
•Truncus arteriosus
•Total anomalous pulmonary venous connection
•Tricuspid atresia
Right to Left Shunts
•TETRALOGY of FALLOT most COMMON
– 1) VSD, large
– 2) OBSTRUCTION to RV flow
– 3) Aorta OVERRIDES the VSD
– 4) RVH
– SURVIVAL DEPENDS on SEVERITY of SUBPULMONIC STENOSIS
– Can be a “PINK”tetralogy if pulmonic obstruction is small, but the greater the obstruction, the
greater is the R🡪L shunt
TGA (Transposition
of Great Arteries)
•NEEDS a SHUNT for survival
– PDA or PFO (65%), “unstable” shunt
– VSD (35%), “stable” shunt
– RV>LV in thickness
– Fatal in first few months
– Surgical “switching”
Truncus Arteriosus
Tricuspid Atresia
•Hypoplastic RV
•Needs a shunt, ASD, VSD, or PDA
•High mortality
Total Anomalous
Pulmonary Venous Connection
•PULMONARY VEINS do NOT go into LA, but into L.
innominate v. or coronary sinus
•Needs a PFO or a VSD
•HYPOPLASTIC LA
Obstructive Congenital
Health Disease
•COARCTATION of aorta
•Pulmonary stenosis/atresia
•Aortic stenosis/atresia
Coarctation of the Aorta
•M>F
•INFANTILE FORM (proximal to PDA) (SERIOUS)
•ADULT FORM (CLOSED DUCTUS)
•Bicuspid aortic valve 50% of the time
CARDIAC
TUMOURS
Primary tumours in the heart are very rare.

Secondary tumours, especially from the lungs, affecting the pericardium by

direct spread and the heart by blood spread are fairly common in advanced
malignant disease.

Atrial ‘myxoma’ is a rare benign tumour affecting usually the left atrium in
which a smooth, pale, soft, rounded mass is attached by a pedicle to the
endocardium.

It can cause serious effects by occluding the mitral valve opening or by

embolisation.

Mesothelioma may present primarily in the pericardium.

Rhabdomyomas are occasionally seen in childhood and are associated

with tuberous sclerosis.
CARDIAC
TUMOURS
90% benign “mesenchymal”, i.e., stromal
◦MYXOMAS (LEFT ATRIUM
MOST COMMON)
◦ FIBROMAS
◦ LIPOMAS
◦ FIBROELASTOMAS
◦ RHABDOMYOMA (Most common cardiac tumor
in children)
10% SARCOMAS
MYXOMA
Cardiac effects of NON-cardiac tumors
Direct Consequences of Tumor
◦ Pericardial and myocardial metastases
◦ Large vessel obstruction
◦ Pulmonary tumor emboli

Indirect Consequences of Tumor (Complications of

Circulating Mediators)
◦ Nonbacterial thrombotic endocarditis (NBTE)
◦ Carcinoid heart disease
◦ Pheochromocytoma-associated heart disease
◦ Myeloma-associated amyloidosis

Effects of Tumor Therapy

◦ Chemotherapy
◦ Radiation therapy
LARGE VESSEL VASCULITIS
LARGE VESSEL VASCULITIS

Takayasu disease
This rare disease, typically affects young females (20–30 years).
The patchy arteritis is more severe and affects the aorta and its primary
branches, leading to ischaemia of brain (cerebral arteries), eyes
(ophthalmic arteries), face (carotid arteries), arms (‘pulseless’ disease),
heart (coronary orifices), and kidneys (causing HYPERTENSION) in
varying combinations.
MEDIUM
VESSEL
VASCULITIS
MEDIUM VESSEL VASCULITIS

Kawasaki disease
This is a disease of infants which affects the main aortic branches particularly the coronary
arteries.
Also called mucocutaneous lymph node syndrome
Febrile disorder of unknown etiology usually affecting children
High incidence (39 per 100K children below age 5) in Hong Kong
Fever, pharyngitis and conjunctivitis, erythematous skin rashes, cervical adenopathy (25%);
also arthritis (40%), coronary arteritis with persistent damage (15 - 25%) which may cause
death
 Anti-neutrophil cytoplasmic antibodies
(ANCA) associated

SMALL ANCA occur in various forms of vasculitis.

VESSEL A perinuclear pattern of staining (p-ANCA) is

seen in microscopic polyarteritis and about 50%
VASCULI of cases of eosinophilic granulomatosis with
polyangiitis (Churg-Strauss syndrome).
TIS Cytoplasmic staining (c-ANCA) is seen in
granulomatosis with polyangiitis (Wegener
granulomatosis).
 In microscopic polyarteritis, there is a form of
glomerulonephritis due to involvement of afferent
arterioles as well as pulmonary capillaritis.
SMALL In granulomatosis with polyangiitis destructive
VESSEL lesions of the nasal mucosa, the lungs and the
kidneys are seen.
VASCULI In eosinophilic granulomatosis with
polyangiitis there is vasculitis with peripheral
TIS eosinophilia, asthma and sinusitis sometimes
associated with peripheral neuropathy.
SMALL VESSEL VASCULITIS
Immune complex associated
The best known example of this is IgA vasculitis (Henoch-Schonlein purpura).
This is characterized by deposition of immune complexes containing IgA which results in purpura,
arthritis and abdominal pain due to intestinal involvement.
Renal involvement may also occur.
ANEURYSMS
Aneurysms
Dissecting aneurysm
Classically begins in the arch of aorta.
Predisposing causes:
(a) HYPERTENSION (common)
(b) Atheroma
(c) Medial degeneration (rare) seen in
Marfan syndrome due to mutation of the
fibrillin gene.
Aneurysms
‘Berry’ aneurysm
Occurs in medium-sized
vessels at the base of the
brain, e.g. on Circle of
Willis.
Associated with congenital
deficiency of arterial
media.
Rupture causes
subarachnoid
haemorrhage.
Aneurysms
Microaneurysm –
associated with
hypertension and affects
smaller arteries and
arterioles, especially in the
brain.
Raynaud Phenomenon
• Primary Raynaud phenomenon occurs in 3%
to 5% of the general population and most
commonly affects young women;
It reflects exaggerated vasomotor responses to
cold or emotion.
The clinical course is usually benign.
• Secondary Raynaud phenomenon is
vascular insufficiency due toarterial narrowing
induced by other conditions (e.g.,
atherosclerosis, systemic lupus erythematosus,
systemic sclerosis [scleroderma], or Buerger
disease).
Varicose Veins
These are typically superficial lower extremity
veins that are dilated and tortuous due to
chronically elevated intraluminal pressure.
They occur in 10% to 20% of men, and 25% to
33% of women; the increased frequency in
women is due to the venous stasis occurring in
pregnancy.
Other causes include hereditary venous
defects, obesity, prolonged dependent leg
position, proximal thrombosis, and compressive
tumor masses.
Thrombophlebitis and
Phlebothrombosis

These are largely interchangeable terms for venous thrombosis and

inflammation.
Predisposing factors for deep vein thrombosis (DVT) include congestive
heart failure, prolonged immobilization, local infection, or systemic
hypercoagulability (e.g., neoplasia, pregnancy, or the postoperative
state).
Migratory thrombophlebitis (Trousseau syndrome) is a
malignancyassociated hypercoagulability due to procoagulant
elaboration; it is characterized by sporadic thrombosis at various sites.
Lymphangitis denotes the inflammation occurring when infections
spread into lymphatics; b-hemolytic streptococci are a common cause.
Lymphangitis presents as painful subcutaneous red streaks, often with
tender regional lymphadenopathy (lymphadenitis).
Dilated lymphatics are filled with neutrophils and macrophages;
inflammation can extend into adjacent tissues with cellulitis orabscess
formation.
Lymphedema is due to lymphatic obstruction and dilation, with
associated increases in interstitial fluid.

Lymphangitis and Lymphedema

 Lymphangitis and
Lymphedema
Primary hereditary causes include Milroy disease (primary
lymphatic agenesis). Common secondary causes of
lymphedema are:
• Malignancy
• Surgical resection of regional lymph nodes
• Post-radiation fibrosis
• Filariasis
• Postinflammatory thrombosis with lymphatic scarring.
Prolonged lymphedema causes interstitial fibrosis, and in
cutaneous tissues it causes a peau d’orange (orange peel)
appearance, with associated ulcers and brawny induration.
Rupture of obstructed lymphatics in a body cavity leads to
milky chylous accumulations.
VASCULAR TUMOURS
PYOGENIC GRANULOMA
This lesion appears on the skin at a site
subject to trauma and infection, e.g.
around the finger nails, the nostrils or
the tongue.
It commonly occurs during pregnancy.
It grows over several days to form a
small red nodule up to about 1 cm in
diameter and is usually ulcerated.
VASCULAR TUMOURS
TELANGIECTASIS
Small localised dilatations of small blood
vessels. There are two main types: congenital
and acquired.
(a) Congenital. In the rare hereditary
haemorrhagic telangiectasia, the small lesions
occur in the skin and mucous membranes and
may be associated with bleeding (especially
from the nose).
(b) Acquired. The small lesions, affecting the
face and neck, are known as spider naevi.
VASCULAR TUMOURS
LYMPHANGIOMA
Lymphangiomas are much rarer than
haemangiomas and are usually without
serious pathological significance.
Occasionally the abnormal lymphatic vessels
become distended and form fluctuant swellings
with local pressure effects, e.g. ‘cystic
hygroma’ of neck of babies and children.
 GLOMANGIOMA

VASCUL (Glomus tumour) – arises from a specialised type

of arteriolovenous junction which includes a rich

AR neural component. A common site is the fingertip,

perhaps under the nail; a very small but often

TUMOUR painful tumour.

Microscopically it consists of a complex of blood
S vessels lined by endothelium but cuffed by round
clear cells with contractile properties (myoid
cells). It is rich in nerve fibres
 ANGIOSARCOMAS
These are uncommon malignant tumours of
endothelial cells. They may affect the skin.
VASCUL Lesions of the scalp occur in the elderly. They
appear as spreading ‘bruise-like’ areas often with
AR a central nodule. Chronic sun exposure is
thought to be important.
TUMOUR Lesions often arise in the limbs in areas of

S
lymphoedema, e.g. in the past following
irradiation and lymph node clearance for
carcinoma of breast.
Angiosarcoma of the liver is associated with
exposure to vinyl chloride monomer.
VASCULAR TUMOURS
KAPOSI SARCOMA
This tumour has increased in incidence with the spread of AIDS and HIV infection.
It is a tumour of endothelial cells due to infection by Herpes virus type 8, often occurring in immunocompromised
patients.
It occurs in four groups of patients.
(a) AIDS associated.
(b) Sporadic (classic) – affecting elderly males (especially Ashkenazi Jews) living in the Mediterranean littoral.
(c) Endemic – mainly in central Africa, typically affecting young adults.
(d) Iatrogenic – in immunosuppression, e.g. transplant patients.
The disease presents as dark coloured (due to high blood content) subcutaneous nodules or plaques resembling
bruises.