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PATHOLOGY

DISEASES OF THE HEART

Dra. Reyes
October 2015

Predominant cause of morbidity and mortality in


industrialized nations
80% of cardiac death are attributable to ischemic
heart disease

3.
4.

Compensation ultimately constitute further burden on


cardiac function
o Combine with the primary cardiac disease
o Secondary hypertrophy to induce dilation in
excess of the optimal tension-generating
point leading to progressive chronic heart
failure

Abnormal circulatory function usually results from:


1.
2.

3.

4.

5.

Disruption of continuity of circulation with significant


loss of blood volume (rupture of aorta)
Disorder of cardiac conduction (heart block) or
Arrhythmia (ventricular fibrillation)
o Leads to uncoordinated contractions of
muscular walls
Lesion preventing valve opening or narrowing of the
lumen of a vessel (aortic valve stenosis or coarction)
o Obstructs blood flow
o Overworks the pump behind the obstruction
Regurgitant flow (mitral or aortic regurgitation)
o Some of the output be directed backward
o Pump repeatedly expels the same blood
Failure of the pump itself (chronic heart failure)
o Potential common endpoint
I. CONGESTIVE HEART FAILURE

A. LEFT-SIDED HEART FAILURE

The pathophysiologic state resulting from impaired


cardiac function
Renders the heart unable to maintain an output
sufficient for the metabolic requirements of the body

Mostly due to:


Systolic dysfunction
o Damaged muscles contracts weakly
o Chambers cannot empty properly
Diastolic dysfunction
o Inability of the heart chambers to relax
sufficiently during diastole
o Unable to fill the ventricles properly

Forward failure
o Diminished cardiac output
Backward failure
o Damming back of blood in the venous
system

Compensatory changes
1. Pressure or volume stress induces hypertrophyinitially adaptive
2. Ventricular dilation

Major causes:
o Ischemic heart disease
o Hypertension
o Aortic and mitral valve disease
o Myocardial disease
Manifested by:
o Pulmonary congestion
o Edema
Reduced cardiac output causes reduced renal
perfusion, leading to:
o Further salt and water retention
o Ischemic ATN??
o Impairment of waste excretion

Prerenal azotemia
CNS perfusion is reduced
o Hypoxic encephalopathy
o Irritability to coma
B. RIGHT-SIDED HEART FAILURE

Characterized by:

Improve contraction by stretching of


myofibers
Blood volume expansion
o Salt and water retention
Tachycardia
o

HEART DISEASE

Consequence of left-sided heart failure


Pure right-sided heart failure may be caused by:
o Intrinsic disease of the lungs or pulmonary
vasculature functional right ventricular
outfluw obstruction (cor pulmonale)
o Tricuspid or pulmonary valvular disease
Manifestations:
o Portal, systemic and dependent peripheral
congestion and edema and effusions (pleural
and peritoneal)
o Hepatomegaly with carotid pulse curve
(nutmeg liver)
o Congestive splenomegaly
o Renal congestion with acute tubular necrosis

Jonelle B. || Page 1 of 16

DISEASES OF THE HEART

II. ISCHEMIC HEART DISEASE

Group of closely related syndromes resulting from


ischemia
o Imbalance between the supply and demand
of the heart for oxygenated blood
o Reduced availability of nutrient substrates
o Inadequate removal of meatabolites
Mechanisms:

Reduced coronary blood flow (>90%)


o Coronary atherosclerosis
o Vasospasm
o Thrombosis
o Uncommon causes:

Arteritis

Emboli

Cocaine-induced vasospasm

Shock with systemic hypotension

Increased myocardial demand exceeding vascular


supply
o Tachycardia
o Hypertrophy
4 ISCHEMIC SYNDROMES:
A. ANGINA PECTORIS

3.

Precipitated
by
Relieved by

Prinzmetal variant

Uncommon

Caused by coronary artery spasm

Occurs at rest
Unstable/Crescendo

Caused by plaque disruption with


superimposed mural thrombosis

Occurs with progressively increasing


frequency

Precipitated by rest

More prolonged duration

Prinzmetal
Uncomon
Coronary
artery
spasm

Effort

Rest

Unstable
Plaque
disruption with
superimposed
mural
thrombosis
Rest

Rest

B. MYOCARDIAL INFARCTION

Marked by death of cardiac muscle cells


Caused by acute plaque change with total thrombotic
occlusion

C. CHRONIC ISCHEMIC HEART DISEASE

Marked by paroxysmal substernal or precordial pain


or discomfort
Ischemia without frank infarction

3 Patterns:
1. Stable/Typical

Most common form

Caused by chronic stenosing coronary


atherosclesoris

Precipitated by effort

Relieved by rest
2.

Caused by

Stable
Most common
Chronic
stenosing
coronary
atherosclerosis

Seen typically in elderly patients with multivessel


coronary atherosclerosis
Insidiously develop into chronic heart failure
May result from:
o Postinfarction cardiac decompensation
o Slow ischemic myocyte degeneration
Death may be precipitated by:
o Slowly progressive chronic heart failure
o Superimposed acute myocardial infarction
o Arrhythmia
o Secondary to unrelated causes

D. SUDDEN CARDIAC DEATH

Unexpected cardiac death within 1 hour of symptom


onset
Caused by disrupted plaque and partial thrombus with
regional myocardial ischemia
o Induces fatal arrhythmia (asystole or
ventricular fibrillation)
Acute Coronary Syndromes

Share a common pathophysiologic basis in coronary


atherosclerotic plaque disruption and intraluminal
thrombus formation
o Unstable angina
o Acute myocardial infarct
o Sudden cardiac death

Jonelle B. || Page 2 of 16

DISEASES OF THE HEART

III. MYOCARDIAL INFARCTION


2 TYPES:
A. TRANSMURAL INFARCT

Full thickness of ventricular wall


Caused by severe coronary atherosclerosis worsened
by acute plaque change disrurption and
superimposed occlusive thrombosis (90%)

Significant plaques typically occur in:


o Proximal 2 cm of the left anterior descending
artery (40-50%)
o Left circumflex artery (15-20%)
o Proximal and distal thirds of the right
coronary artery (30-40%)
Nearly all affect the left ventricle
o 15%- Left and right ventricle

Particularly in the posterior-inferior


left ventricle infarcts
o 1-3%- Isolated right ventricle infarction

Schematic representation of sequential progression of


coronary artery lesion morphology
Beginning with stable chronic plaque responsible for
typical angina and leading to various acute coronary
syndromes
CORONARY ARTERY PATHOLOGY IN ISCHEMIC HEART
DISEASE
Stenoses

Plaque
Disruption

Stable angina

>75%

No

Unstable
angina

Variable

Frequent

Transmural
myocardial
infarction

Variable

Frequent

Syndrome

Subendocardial
myocardial
infarction

Sudden death

Variable

Usually
severe

Variable

Frequent

PlaqueAssociated
Thrombus

No
Nonocclusive,
often with
thromboemboli
Occlusive
Widely
variable, may
be absent,
partial/
complete or
lysed
Often small
platelet
aggregates or
thrombi and/or
thromboemboli

Initial event:
o Erosion, ulceration, fissuring rupture or
hemorrhagic expansion of partially stenosing
atheroma (acute plaque change or
disruption)
o Followed by thrombosis
Absence of sudden death
Thrombi may lyse
Fibrinolytic treatment or
spontaneously
vasospasm may relax

Re-establish flow and spare some myocardium from


necrosis

Reperfusion of precariously injured cells

Restore viability
Cells poorly contractile (stunned) for 1-2 days
B. SUBENDOCARDIAL INFARCT

Limited to inner 1/3 to 1/3 of the ventricular wall, often


multifoca
Caused by:

Diffuse coronary atherosclerosis and global borderline


perfusion without superimposed thrombosis made
transiently critical by:
o Increased demand
o Vasospasm
o Hypotension
Disrupted plaque overlying thrombus
o Spontaneously lyses
o Limiting the extent of myocardial injury

Jonelle B. || Page 3 of 16

DISEASES OF THE HEART

Evolution of Morphologic Changes in Myocardial Infarction


Gross
Features
Reversible Injury
Time

0-1/2
hr

None

Light Microscope

Electron
Microscope
Relaxation of
myofibrils,
glycogen loss,
mitochondrial
swelling

None

Irreversible Injury

Schematic representation of progression of myocardial necrosis


after coronary artery occlusion.
Necrosis begins in a small zone of myocardium beneath the
endocardial surface in the center of ischemic zone. This entire
region of myocardium (dashed outline) depends on the occluded
vessel for perfusion and is the area at risk.
Note that a very narrow zone of myocardium immediately beneath
the endocardium is spared from necrosis because it can be
oxygenated by diffusion from the ventricle

Approximate Time of Onset of Key Events in Ischemic


Cardiac Myocytes
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
>1hr

- 4 hr

None

4-12 hr

Ocassionally
dark mottling

12-24
hr

Dark
mottling

1-3
days

Mottling with
yellow-tan
infarct center

3-7
days

Hyperemic
border;
central
yellow-tan
softening

7-10
days

10-14
days

2-8
weeks

>2 mos

Maximally
yellow-tan
and soft,
with
depressed
red-tan
margins
Red-gray
depressed
infarct
borders
Gray-white
scar,
progressive
from border
toward core
of infarct
Scarring
complete

Usually none;
variable waviness of
fibers at border
Beginning
coagulation necrosis;
edema; hemorrhage
Ongoing coagulation
necrosis; pyknosis of
nuclei; myocyte
hypereosinophilia;
marginal contraction
band necrosis;
beginning
neutrophilic infiltrate
Coagulation necrosis
with loss of nuclei
and striations;
interstitial infiltrate of
neutrophils
Beginning
disintegration of dead
myofibers, with dying
neutrophils; early
phagocytosis of cells
by macrophages at
intact border

Sarcolemmal
disruption,
mitochondrial
amorphous
densities

Well-developed
phagocytosis of dead
cells, early formation
of fibrovascular
granulation tissue at
margins

Well-established
granulation tissue
with new blood
vessels and collagen
deposition with
decreased cellularity

Dense collagenous
scar

Jonelle B. || Page 4 of 16

DISEASES OF THE HEART

Morphology of Myocardial Infarction

Area of necrosis can be demonstrated histochemically


after 2-3 hours using TTC (triphenlytetrazolium
chloride)
Infarcted area is revealed as unstained pale zone
o Because dehydrogenases are depleted in
this area
o Staining defect is due to the enzyme leakage
that follows cell death
Dye imparts a brick-red color to intact, noninfarcted
myocardium

Within first 10 days


o Ventricular rupture of the free wall causing
pericardial hemorrhage and tamponade
rupture of the septum producing left to
right shunt right-sided heart volume
overload

Papillary muscle infarction mitral valve dysfunction


Fibrinous to hemorrhagic pericarditis
Mural thrombosis in noncontractile area,
o Risk of peripheral embolization
Infarct expansion
o Deformation of a large area of infarction
o May heal into ventricular aneurysm
o Prone to mural thrombosis
Infarct extension
o Repetitive infarction

Clinical Features

Diagnosis is based on:


o Symptoms

Chest pain

Nausea

Diaphoresis

Dsypnea
o ECG changes
o Serum elevation of CKMB
o Troponin I
o Troponin T
o Myoglobin
o Adjunctive for diagnosis

Angiography

Echocardiography

Perfusion scintigraphy
25% of patients experience sudden death secondary
to fatal arrhythmia
o Patients who survive the event, 80-90%
develop complications
Early restoration of flow through the occluded vessel
generates better prognosis via:
o Thrombolysis
o Balloon angioplasty
st
Overall mortality in the 1 year is 30% and thereafter
5-10% per year
Complications depend on:
o Size
o Location of necrosis
o Reserve of functional myocardium

Complications:

Seen with >40% of left ventricle infarct


o Arrhythmia
o Chronic heart failure
o Cardiogenic shock

IV. HYPERTENSIVE HEART DISEASE


A. SYSTEMIC (LEFT-SIDED) HYPERTENSIVE HEART
DISEASE
Diagnostic Criteria

Left ventricular hyperplasia


o Typically concentric
History of extracardiac anatomic evidence of
hypertension
Absence of other lesions that might induce cardiac
atrophy
o Aortic valve stenosis
o Coarction of aorta

Clinical Features

Causes of death
o Chronic heart failure (1/3)
o Renal disease
o Stroke
o Unrelated causes
Hypertensive heart disease increases the risk of
sudden cardiac death

Pathogenesis
Myocyte hypertrophy as a response to increased work

Thickened myocardium reduces left ventricle compliance

Impaired diastolic filling


Increase in oxygen demand
Increases the distance for oxygen and nutrient diffusion
from adjacent capillaries

Accompanying coronary atherosclerosis adds the element


of ischemia

Jonelle B. || Page 5 of 16

DISEASES OF THE HEART


c.
Morphology:

Thickened left ventricle wall (usually >2cm)


Increased heart weight (>500 gm)
Myocytes and nuclei are enlarged
In the long term
o Diffuse interstitial fibrosis
o Focal myocyte atrophy and degeneration
o Left ventricle chamber dilatation and wall
thinning
B. PULMONARY (RIGHT-SIDED) HYPERTENSIVE
HEART DISEASE

d.
e.
3.

Disorders affecting chest movement


a. Kyphoscoliosis
b. Marked obesity (pickwickian syndrome)
c. Neuromuscular diseases

4.

Disorders inducing pulmonary arterial constriction


a. Metabolic acidosis
b. Hypoxemia
c. Chronic altitude sickness
d. Obstruction to major airways

Cor pulmonale
Pulmonary disorders pulmonary hypertension
right ventricular hyperplasia or dilatation

V. VALVULAR HEART DISEASE

1. Acute cor pulmonale

Right ventricle dilation after massive pulmonary


embolization

2. Chronic cor pulmonale

Chronic right ventricle pressure overload


Pulmonary vasoconstriction due to
hypoxemia/acidosis exacerbates baseline pulmonary
hypertension

A. DEGENERATIVE CALCIFIC AORTIC VALVE


STENOSIS

Age-related lesions clinically important in patients in


their 70s and 80s
Morphology:

Morphology:

Right ventricle hyperplasia >1cm thickness, dilation or


both
Pulmonary arteriolar wall thickening
Pulmonary arterial atherosclerosis
Left side of heart essentially normal

Responsible for some cases of cardiac


decompensation
Cardiac symptoms masked by underlying lung
disease

Need for surgical intervention is heralded by:


o Angina
o Syncope
o Chronic heart failure

Degenerative, noninflammatory, calcific deposits


within the mitral annulus
Usually in the elderly
May be present:
o Regurgitation
o Stenosis
o Arrhythmia
Rarely a focus of infective endocarditis

B. MITRAL ANNULAR CALCIFICATION

Disorders Predisposing to cor Pulmonale


1.

2.

Diseases of the pulmonary parenchyma


a. Chronic obstructive pulmonary disease
b. Diffuse pulmonary interstitial fibrosis
c. Pneumoconioses
d. Cystic fibrosis
e. Bronchiectasis
Diseases of pulmonary vessels
a. Recurrent pulmonary thromboembolism
b. Primary pulmonary hypertension

Subendothelial rigid calcific masses with thickening


and immobility of the valve cusps
Narrowing of orifice but no commissural fusion
Concentric left ventricle hyperplasia
o From chronic pressure overload

Clinical Features

Clinical Features:

Extensive pulmonary arteritis (Wegener


granulomatosis)
Drug, toxin or radiation-induced vascular
obstruction
Extensive pulmonary tumor microembolism

C. MITRAL VALVE PROLAPSE

Myxotamous degeneration of the mitral valve


Myxomatous, enlarged and floppy mitral valves that
balloon back (prolapse) into left atrium during systole
20-40 years of age
Uncertain etiology
Common in Marfan syndrome

Jonelle B. || Page 6 of 16

DISEASES OF THE HEART

Clinical Features:

1. Acute rheumatic fever

Generally asymptomatic
Discovered only as midsystolic click
Manifestations:
o Atypical chest pain
o Dsypnea
o Fatigue
o Psychiatric manifestations
Increased risk
o Infective endocarditis
o Mitral valve insufficiency
o Atrial and ventricular arrhythmia
o Sudden cardiac death

Morphology:

Interchordal ballooning >4mm of the mitral valve


leaflets
Elongated, attenuated occasionally ruptured chordae
tendinae
No commissural fusion
Thinning and degeneration of the fibrosa layer
Myxomatous thickening of the spongiosa
Secondary changes:
o Fibrous thickening of valve leaflets
o Thickened left ventricle endocardium
o Atrial thrombosis behind the ballooning
cusps
o Calcification of the mitral annulus
D. RHEUMATIC FEVER AND RHEUMATIC HEART
DISEASE

Rheumatic fever
o Acute, recurrent inflammatory disease
o Typically occurs 1 to 5 weeks after group A,
-hemolytic streptococcal infection
st
Mainly 5-15 years; adults may suffer the 1 attack
Secondary to host antistreptococcal antibodies crossreactive to cardiac antigens
Diagnosis rests on the:
o Clinical history
o 2 out of 5 major Jones criteria

Major Jones Criteria (CASEC)


1. PanCarditis
2. Migratory large joint polyarthritis
3. Subcutaneous nodules
4. Erythema marginatum (bathing suit distribution)
5. Sydenham Chorea

Death is rare
Most frequently secondary to myocarditis
Valvular involvement leads to deformation, scarring
with permanent dysfunction

2. Chronic rheumatic heart disease

st

1 attack is in early childhood


st
1 bout of rheumatic fever is severe
With recurrent attacks

Clinical features:

Left atrial hypertrophy and enlargement


Chronic pulmonary congestive changes
Right ventricular hypertrophy
Chronic heart failure
Increased risk of infective endocarditis
Atrial fibrillation secondary to atrial dilatation

Morphology

Aschoff bodies
o Pathognomic foci of fibrinoid necrosis
o With lymphocytes, macrophages and plasma
cells
o Found in many sites, usually the
myocardium
o Slowly replaced by fibrous scar
Valvulitis with formation of beady fibrinous
vegetations (verrucae)
Healed valve shows
o Fibrous thickening of leaflets
o Calcification of fibrous leaflets
o Commisural fusion
o Fishmouth or buttonhole stenosis
o Thickened, fused and shortened mitral valve
chordae
MacCallum plaques
o Subendocardial collections of Aschoff
nodules
o Usually in left atrium
Solitary mitral involvement (65-75%)
Mitral or aortic involvement (20-25%)

Minor Jones Criteria


1. Fever
2. Arthralgia
3. Leukocytosis

Jonelle B. || Page 7 of 16

DISEASES OF THE HEART

Morphology

Friable, 0.5 to 2 cm, microbe-laden vegetations on


one or more valves
Acute infective endocarditis
o Cause erosions or perforations of leaets,
invading adjacent myocardium or aortic wall
to produce abscess cavities
Subacute infective endocarditis
o Rarely erode or penetrate the leaets
With prosthetic valves
o Ring abscess is almost always present
With IV drug abuse
o Vegetations are often acute and on right-
sided valves

Clinical Consequences

The pathogenic sequelae and key morphologic features of


rheumatic heart disease

VI. VEGETATIVE ENDOCARDITIS


A. INFECTIVE ENDOCARDITIS

Colonization of heart valves with microbiologic


organisms
Formation of friable, infected vegetations and valve
injury

Direct injury to valves


o Insuciency or stenosis with chronic heart
failure
o
myocardium and aorta
Embolization to spleen, kidneys, heart, and brain with
infarction or metastatic infection
Renal injury
o Embolic infarction or infection
o Antigen-antibody mediated acute
glomerulonephritis
Diagnosis conrmed by blood cultures (positive in 80
to 95%)

1. Acute Infective Endocarditis


Pathogenesis:

Bacteremia is prerequisite, derive from:


o Infection elsewhere in the body
o IV drug abuse
o Dental or surgical procedures
o Microinjuries to gut, urinary tract, oropharynx
or skin
Cardiac congenital anomalies are common subsoil
including
o Shunts (Ventricular septal defect)
o Stenoses
o Chronic rheumatic heart disease
o Mitral valve prolapse
o Prosthetic valves
o Indwelling catheters
Contributory conditions:
o Neutropenia
o Immunosuppressed

Caused by highly virulent organisms (S. aureus)


o Seeding a previously normal valve
o Produces a necrotizing, ulcerative and
invasive infection
Rapidly developing
o Fever with rigors
o Malaise
o Weakness
Larger vegetations often cause embolic complications
with splenomegaly
Even with treatment, death occurs in days to weeks
(50-60%)

2. Subacute Infective Endocarditis

Caused by organism of moderate to low virulence (S.


viridans)
o Seeding an abnormal or previously injured
valve
o Cause less valvular destruction
Insidious onset
o Non-specific malaise
o Low-grade fever

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DISEASES OF THE HEART

o Weight loss
o Flu-like syndrome
Smaller vegetations, less frequent embolic
complications
Protracted course even without treatment, less fatal
VII. NONBACTERIAL THROMBOTIC ENDOCARDITIS

Marantic endocarditis
Small (1 to 5 mm), sterile, bland brin and platelet
thrombi (vegetations) loosely adherent to valve
leaets (often mitral) along the lines of closure,
without signicant inammation or valve damage
Attributed to systemic hypercoagulability
May embolize to brain, heart, or elsewhere
Occurs in settings of cancer (visceral
adenocarcinomas) or prolonged debilitating illness,
attributed to DIC or hypercoagulability state
A. LIBMAN-SACKS ENDOCARDITIS

Endocarditis associated with systemic lupus


erythematous
Uncertain pathogenesis, also occur in
antiphospholipid antibody syndrome
Mitral and tricuspid valves
o Fibrinoid necrosis with mucoid degeneration
o Small sterile vegetations on either side of the
valve leaets
Valve deformations from healed vegetations

Morphology

Plaquelike intimal thickenings of the


o Tricuspid
o Right ventricle ow tract
o Pulmonic valve
Left side usually unaffected

Diagrammatic comparison of the lesions in the four major


forms of vegetative endocarditis.

The rheumatic fever phase of RHD (rheumatic heart


disease)
o Marked by a row of warty, small vegetations
along the lines of closure of the valve
leaflets.

IE (infective endocarditis)

Characterized by large, irregular masses on


the valve cusps that can extend onto the
cords
NBTE (nonbacterial thrombotic endocarditis)
o Typically exhibits small, bland vegetations,
usually attached at the line of closure.
o One or many may be present
LSE (Libman-Sacks endocarditis)
o has small or medium-sized vegetations on
either or both sides of the valve leaflets
o

MAJOR CAUSES OF ACQUIRED HEART VALVE


DISEASE
Mitral Valve Disease
Aortic Valve Disease
Mitral Stenosis
Aortic Stenosis
Post-inammatory scarring
Post-inammatory scarring
(rheumatic heart disease)
(rheumatic heart disease)
Senile calcic aortic
stenosis
Calcication of congenitally
deformed valve
Mitral Regurgitaion
Abnormalities of leaets
and commissures
Postinammatory
scarring
Infective endocarditis
Mitral valve prolapse
Abnormalities of tensor
apparatus
Rupture of papillary
muscle
Papillary muscle of
dysfunction (brosis)
Rupture of chordae
tendinae
Abnormalities of left
ventricular cavity and/or
annulus
Left ventricular
enlargement (myocarditis,
dilated CMP)
Calcication of mitral
annulus

Aortic Regurgitation
Intrinsic valvular disease
Postinammatory
scarring (rheumatic
disease)
Infective endocarditis
Aortic disease
Degenerative aortic
dilation
Syphilitic aortitis
Ankylosing spondylitis
Rheumatic arthritis
Marfan syndrome

Jonelle B. || Page 9 of 16

DISEASES OF THE HEART

VIII. MYOCARDIAL DISEASE

Myocardial dysfunction as the major cause of heart


disease
o Inammatory disorders (myocarditis)
o Immunologic and systemic metabolic
disorders
o Genetic abnormalities in myocytes

A. CARDIOMYOPATHY

Primary cardiac abnormality localized to myocardium


Diagnosed by endomyocardial biopsy
Three types:
o Dilated,
o Hypertrophic,
o Restrictive

1. Dilated Cardiomyopathy

gradual four-chamber hypertrophy and dilation


occur as slow, progressive chronic heart failure

Morphology

Cardiomegaly up to 900 gm

Mural thrombi secondary to poor contractie function


and stasis

Valves and coronary arteries usual for age

Diuse myocyte hypertrophy and interstitial


myocardial brosis

Focal endocardial thickenings, especially in ventricles


2. Hypertrophic Cardiomyopathy

Idiopathic hypertrophic subaortic stenosis


Hypertrophic obstructive cardiomyopathy
heavy, muscular, hypercontractile, poorly compliant
heart with poor diastolic relaxation

Morphology

Marked cardiomegaly, LV>RV with atrial dilation


Asymmetric septal hypertrophy (ASH) in classical
cases
Banana-like conguraiton of left ventricle
Basal septum thickened at the level of mitral valve,

Left ventricle-systolic outflow may be


compromised
Patchy replacement brosis

3. Restrictive Cardiomyopathy

Restricted ventricular lling and reduced CO


Presence of interstitial myocardial brosis

Endomyocardial brosis

Ventricular subendocardial brosis with mural thrombi


AV valves may be involved
Reduced ventricular chamber volume

Loeffler endocarditis

Similar to endomyocardial brosis

Both have associated:


o Peripheral eosinophilia
o Eosinophilic inltration of multiple organs,
and rapidly fatal course

Endocardial fibroelastosis

Focal to diffuse cartilage like broelastic thickening of


the endocardium;
LV>RV
Most common <2 yrs of age
Congenital cardiac anomalies present in 1/3 of cases

Manifestations:

Dyspnea,
Angina,
Near-syncope,
Chronic heart failure
May be asymptomatic

Complications:

Atrial fibrillation with mural thrombi


Embolization
Infective endocarditis
Chronic heart failure
Sudden cardiac death

Jonelle B. || Page 10 of 16

DISEASES OF THE HEART

B. MYOCARDITIS

1.

Representation of the three distinctive clinical-pathologicfunctional forms of myocardial disease.


CARDIOMYOPATHY AND INDIRECT MYOCARDIAL
DYSFUNCTION: FUNCTIONAL PATTERNS AND CAUSES
Functional
Pattern
LV Ejection
fraction*
Mechanisms
of heart
failure

Causes

Dilated

Hypertrophic

Restrictive

<40%

50-80%

45-90%

Impairment of
contractility
(systolic
dysfunction)

Impairment of
compliance
(diastolic
dysfunction)

Impairment
of
compliance
(diastolic
dysfunction)
Idiopathic;
amyloidosis;
radiation
induced
fibrosis

Idiopathic;
alcohol;
peripartum;
genetic;
myocarditis;
hemochromatosis;
chronic
anemia;
doxorubicin
(Adriamycin;
sarcoidosis
Indirect
Ischemic
myocardial
heart
dysfunction
disease;
(not
valvular
cardiomyop
heart
athy)
disease;
hypertensive
heart
disease;
congenital
heart
disease
*Normal approximately 50-65%.

Idiopathic;
genetic;
Friedreich
ataxia;
storage
diseases;
infants
of diabetic
mothers

2.
3.
4.
5.
6.
7.
1.
2.
3.

1.
2.

Pericardial
constriction

MAJOR CAUSES OF MYOCARDITIS


Infections
Viruses (e.g., coxsackievirus, ECHO, inuenza, HIV,
cytomegalovirus)
Chlamydiae (e.g., C. psi3aci)
Rickettsiae (e.g., R. typhi typhus fever)
Bacteria (e.g., Corynebacterium diphtheria, Neisseria
meningococcus, Borrelia Lyme disease)
Fungi (e.g., Candida)
Protozoa (e.g., Trypanosoma Chagas disease,
toxoplasmosis)
Helminths (e.g., trichinosis)
Immune-Mediated Reactions
Postviral
Poststreptococcal (rheumatic fever) Systemic lupus
erythematosus
Drug hypersensitivity (e.g., methyldopa, sulfonamides)
Transplant rejection
Unknown
Sarcoidosis
Giant cell myocarditis

Morphology

Hypertensive
heart
disease;
aortic
stenosis

Broad clinical spectrum


o Asymptomatic to abrupt onset of arrhythmia,
o Chronic heart failure
o Sudden cardiac death
Infectious or immunologic
o Most cases probably of viral origin
o Trypanosoma cruzi (chagas disease) in 50%
of cases in s. America
o In many cases the cause is unknown, or the
microbe unidentiable

1.

abby ventricular myocardium, four chamber


dilatation and patchy diuse hemorrhagic mottling
with mural thrombi
Focal lesions, unaected endocardium and valves
Myocardial inammatory inltrate with myocyte
necrosis or degeneration
Viral Myocarditis

Isolated myober necrosis, interstitial edema,


mononuclear inltrate
Complete resolution with no residua or variable
interstitial and focal brosis
May present later as dilated cmp

2.

Hypersensitivity Reactions

Perivascular mononuclear and eosinophilic inltrates


Acute vasculitis and spotty myober necrosis
Often induced by therapeutic drugs

Jonelle B. || Page 11 of 16

DISEASES OF THE HEART

3.

Idiopathic giant cell myocarditis

Focal myocyte necrosis associated with


o Granulomatous inammation
o Multinucleated giant cells
Poor prognosis

VIII. PERICARDIAL DISEASE

Unusually primary
Generally, it is secondary to diseases of adjacent
structures or part of a systemic disorder.

o Viral infections
Lymphocytic inltration
Fuid resorbs if the disease remits

Fibrinous/serobrinous

Most common form


Seen with myocardial infarction
Associated with friction rub
May completely resolve or organize into adhesive
pericarditis

Hemorrhagic
A. PERICARDIAL EFFUSION

Normal pericardial sac contains 30 to 50 cc;


Accumulations rarely exceed 500 cc
Serous
o Most common form
o Slowly accumulating
o Caused by chronic heart failure and
hypoproteinemia
Serosanguineous
o Usually from blunt chest trauma
Chylous
o From lymphatic obstruction

Purulent (Suppurative)

B. HEMOPERICARDIUM

Blood in the pericardium w/o inammation


Due to:
o Trauma
o Rupture of myocardium or aorta
o Hemorrhage from an abscess
o Tumor metastases
Rapidly lling
o 200-300 ml may cause tamponade
C. PERICARDITIS

Mostly from disorders involving the heart or adjacent


mediastinal structures
Less frequently from systemic abnormalities
o Uremia
o Autoimmune diseases

1. Acute Pericarditis

Mostly viral

Serous

50-200 ml; slowly accumulating


Nonbacterial
o Renal failure
o Systemic lupus erythematous
o Tumors
o Uremia,

Gram positive staphylococci, streptococci, or


pneumcocci
400 to 500 ml thin to creamy pus
Fever, rigors and friction rub
Organizes to constrictive pericarditis or
mediastinopericarditis

Caseous

Secondary to
o Tuberculosis
o Less from mycotic infections

Most frequent antecedent to brocalcic constrictive


pericarditis
2. Chronic or Healed Pericarditis

Blood mixed with brinous to suppurative effusion


Secondary to
o Cardiac surgery,
o Tuberculosis
o Cancer
Usually organizes with or without calcication

Healing of acute lesions can lead to resolution or


epicardial brosis
Soldier's plaque nonadherent epicardial plaque
Usual antecedents are tuberculous or purulent
pericarditis

Adhesive Mediastinopericarditis

Obliterated pericardial sac, parietal layer is tethered to


mediastinal tissue
Heart contracts against all the surrounding attached
structures
Subsequent hypertrophy and dilatation

Constrictive Pericarditis

Thick dense brous obliteration with calcication of


the sac that encases the heart
Limits diastolic expansion and cardiac output
Tb most common cause

Jonelle B. || Page 12 of 16

DISEASES OF THE HEART

3. Rheumatoid Arthritis

Involves the heart in 20 to 40%


Pericarditis derived from pericardial rheumatoid
granulomas
May progress to potentially restrictive adhesions
CAUSES OF PERICARDITIS
Infections

Viruses
Pyogenic bacteria
Tuberculosis
Fungi
Other parasites
Presumably Immune-Mediated Reactions
Rheumatic fever
Systemic lupus erythematosus
Scleroderma
Postcardiotomy
Post-myocardial infarction (Dressler) syndrome
Drug hypersensitivity reaction
Miscellaneous
Myocardial infarction
Uremia
After cardiac surgery
Neoplasia
Trauma
Radiation
IX. TUMORS

Primary tumors are rare


Cardiac metastases occur in some patients who die of
cancer, usually involving the pericardium or penetrate
into the myocardium.
A. LIPOMAS

Probably hamartomas
Circumscribed but poorly encapsulated
Often subendocardial large polypoid accumulations of
adipose tissue
Usually in the
o Left ventricle,
o Right atrium
o Septum
Encroachment on valve function or conduction
pathways

L:R is 4:1
1 to 10 cm diameter, sessile to pedunculated;
Globular and hard to papillary or villous and myxoid
Stellate or globular mesenchymal myxoma cells, with
o Endothelial cells,
o Smooth muscle cells,
o Inammatory cells, in an acid mps matrix
Physical obstruction or wrecking ball trauma to the
atrioventricular valves or embolization
C. RHABDOMYOMAS

Most common primary cardiac tumor in children


Probably hamartomas;
Some associated with tuberous sclerosis
May cause valvular or outflow tract obstruction
Left-sided or right-sided gray-white ventricular wall
masses
Large, rounded, or polygonal cells rich in glycogen
and containing myobrils
Spider cells
o Cytoplasmic strands radiating from the
nucleus to plasma membrane
D. PAPILLARY FIBROELASTOMAS

May cause emboli, usually incidental nding at


autopsy
Found on right-sided valves in children
Left-sided valves in adults
Clusters of 2 to 5 mm hairlike projections,
laments have a core of myxoid tissue with smooth
muscle cells and broblast, covered by endothelium
Probably derived from organized thrombi
E. ANGIOSARCOMA AND RHABDOMYOSARCOMAS

Malignant neoplasms that resemble their counterparts


in other locations
X. CONGENITAL HEART DISEASE

Some cardiac abnormalities are incompatible with


intrauterine survival;
Others manifest shortly after birth as the circulation
changes from fetal to postnatal conguration;
Still others cause cardiac malfunction only in adult life
Some are entirely inconsequential.

Etiology
B. MYXOMAS

Most common primary cardiac tumor in adults


Hamartomatous origin
Usually single, 90% arise in the atria in the region of
fossa ovale

Unknown in 90%, likely multifactorial with genetic and


environmental inputs
2x to 10x increase in the incidence of chronic heart
disease in siblings of an affected child

Jonelle B. || Page 13 of 16

DISEASES OF THE HEART

5% associated with chromosomal abnormalities (e.g.,


down syndrome)
<1% environmental
o Rubella

Patent ductus arteriosus

Pulmonic and aortic stenosis

Tetralogy of fallot
o Thalidomide (sleeping pill)
o Excessive alcohol consumption and
cigarette smoking
o Most criticalembryologic development (3 to
8 weeks aog)
A. SHUNTS

Abnormal communication
o Between heart chambers,
o Between vessels,
o Between chambers and vessels
Depending on pressure relationships,

2 Types of Shunts

Right to left shunts


o Causes cyanosis from the outset as poorly
oxygenated blood passes into the systemic
circulation;
o Also permit paradoxical embolism
Left to right shunts
o Induce chronic right-sided heart overload
o
with 2 pulmonary hpn and rvh,
o Eventually right-sided pressure exceeds
leftsided pressure,
o Shunt becomes right to-left;
o Cyanosis appears late

RELATIVE FREQUENCY AND GENDER DISTRIBUTION


OF 12 MOST COMMONLY ENCOUNTERED
CONGENITAL CARDIAC MALFORMATIONS
Relative
Male-toMalformation
Frequency (%)
Female Ratio
Ventricular septal
32
1:1
defect
Atrial septal defect
8
1:2
Patent ductus
8
1:2
arteriosus
Tetralogy of Fallot
8
1:1
Pulmonary stenosis
8
1:1
Aortic stenosis or
8
3:1
atresia
Coarctation of aorta
6
2:1
Transposition of great
5
2:1
arteries
Atrioventricular septal 4
1:1
defect
Tricuspid atresia
2
1:1
Total anomalous
2
1:1
pulmonary
venous connection
1
1:1
Truncus arteriosus
8

Other
XI. LEFT-TO-RIGHT SHUNTS: LATE CYANOSIS
A. VENTRICULAR SEPTAL DEFECT

B. OBSTRUCTIONS

Typically
o Coarctation,
o Valvular stenoses,
o Atresias
Do not cause cyanosis

Abnormal opening in ventricular septum


Most common congenital cardiac anomaly
Frequently associated with tof but 30% isolated
90% are membranous; 10% muscular
Presents as:
o Fulminant chronic heart failure
o Late cyanosis,
o Asymptomatic holosystolic murmur
Spontaneous closure in 50% when < 0.5 cm in
diameter
Increased risk of infective endocarditis
Early surgical correction
B. ATRIAL SEPTAL DEFECT

Abnormal opening in atrial septum


Most common congenital cardiac anomaly presenting
in adults

Three types:

Primum type (5%)


o Common in down's syndrome;
o Low in the atrial septum
Secundum type (90%)
o At foramen ovale

Jonelle B. || Page 14 of 16

DISEASES OF THE HEART

Sinus venosus type (5%)


o High in the septum near the svc entrance
Usually asymptomatic until adulthood
Right-sided failure gradual right-sided hypertrophy
and pulmonary hypertension Right to Left shunting
and cyanosis
Early surgical correction to prevent pulmonary
vascular changes and paradoxical embolism

The shunt is right to left (shunt reversal) and there is


volume and pressure hypertrophy of the right ventricle.
Arrows indicate the direction of blood flow.
XII. RIGHT-TO-LEFT SHUNTS: EARLY CYANOSIS
A. TETRALOGY OF FALLOT

C. PATENT DUCTUS ARTERIOSUS

In fetus, ductus arteriosus permits blood ow between


the aorta and pulmonary artery
At term, 02 and low PGE induces closure within 1-2
days of life
85-90% are isolated defects with associated left
ventricular hyperplasia and pulmonary artery dilation
Initially asymptomatic;
Machinery-like murmur
Long standing patent ductus arteriosus induces:
o Pulmonary hypertension
o Right ventricular hyperplasia
o R-l shunting
o Late cyanosis
Early closure is advocated

Embryologic anterosuperior displacement of


infundibular septum
Four components
o Ventricular septal defect
o dextroposed overriding aorta
o Pulmonary stenosis
o Right ventricular hyperplasia
Cyanosis from birth or soon after
Complete pulmonary obstruction, survival is permitted
by a patent ductus arteriosus or dilated bronchial
arteries
Pulmonary stenosis protects the lung from volume or
pressure overload
B. TRANSPOSITION OF GREAT ARTERIES

Aorta from the RV and the pulmonary artery from the


LV
Fetal life depends on patent ductus arteriosus and
patent foramen ovale
Postnatal life depends on
o Patent ductus arteriosus
o VSO
o Atrial septal defect
o Patent foramen ovale
Most children die within rst 6 months if untreated
Common in children with diabetic mothers
C. TRUNCUS ARTERIOSUS

Schematic diagram of congenital left-to right shunts.


A, Atrial septal defect.
B, Ventricular septal defect (VSD).

The shunt is left to right and the pressures are


the same in both ventricles. Pressure hypertrophy of the right ventricle and volume
hypertrophy of the left ventricle are present.
C. Patent ductus arteriosus.
D, Atrioventricular septal defect.
E, Large ventricular septal defect with irreversible
pulmonary hypertension

Failure of aorta and PA to separate


Results in an infundibular ventricular septal defect
RL shunting eventually L-R with RVH with
pulmonary HPN
Poor prognosis
D. TRICUSPID ATRESIA

Complete occlusion of tricuspid valve


Mitral valve larger than normal, hypoplastic right
ventricle
Circulation maintained by
o Asd,
o Patent fo,
o Vsd
Cyanosis from birth
High mortality from rst weeks or months of life

Jonelle B. || Page 15 of 16

DISEASES OF THE HEART

E. TOTAL ANOMALOUS PULMONARY VENOUS


RETURN

Pulmonary veins do not directly join the left atrium


Drains into left innominate vein or to coronary sinus
Patent FO or ASD always present
RAH and RVH with dilation, pulmonary trunk dilated,
hypoplastic left atrium

o Lower body cyanosis


o With fetal rvh and early right-sided failure
Death due to
o CHF
o Aortic dissection,
o Intracranial bleed,
o IE

Diagram showing coarctation of the aorta with and without


persistent ductus arteriosus (PDA).
B. PULMONARY VALVE STENOSIS OR ATRESIA WITH
INTACT IV SEPTUM

Schematic diagram of the most important right-to-left


shunts (cyanotic congestive heart disease).
A, Tetralogy of Fallot.

Diagrammatic representation of anatomic


variants, indicating that the direction of shunting
across the VSD depends on the severity of the
subpulmonary stenosis. Arrow indicates the
direction of the blood flow.
B, Transposition of the great vessels with and without YSD

C. AORTIC VALVE STENOSIS OR ATRESIA

XIII. OBSTRUCTIVE CONGENITAL ANOMALIES


A. COARCTION OF AORTA

Constriction of the aorta, 50% as isolated defect


Most with cardiomegaly
Mostly postductal coarctation (distal to ductus or
ligamentum artenosus)
Asymptomatic unless severe
Upper extremity hypertension
Lower extremity hypotension and low ow
Collateral ow develops with intercostal rib notching,
internal mammary and axillary artery dilation

Occurs in isolation or with other anomalies


Complete pulmonic atresia
o Hypoplastic RV
o ASD,
o PDA
Pulmonary outflow obstruction may be subvalvular or
supravalvular
Mild stenosis is asymptomatic
More severe stenosis cause early cyanosis

Congenital complete aortic atresia is rare and


incompatible with survival
Survival depends on severity
Aortic valvular stenosis may be valvular and
subvalvular
Complications are
o
IE,
o LVH,
o Poststenotic dilation of aortic root,
o Sudden death
Bicuspid aortic valve is common and prone to calcic
degeneration and IE

1. Preductal Coarction
o
o

Early manifestation, rapidly fatal


Survival depends on pda, to sustain blood
ow to the distal aorta

Jonelle B. || Page 16 of 16

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