CARDIAC PATHOLOGY

dr. Budiana Tanurahardja,Sp.PA

dr. Lisnawati, SpPA

DEPARTEMEN PATOLOGI ANATOMIK

FAKULTAS KEDOKTERAN UNIVERSITAS INDONESIA
PUSAT SUMBER PEMBELAJARAN PATOLOGI
CARDIAC PATHOLOGY
 Valvular heart disease
 Myocardial diseases
 Tumors of the heart
 Corpulmonale
Normal heart
Normal heart : mitral valve
Normal Heart : Aortic valve
Normal heart : Tricuspid valve
Normal heart : pulmonary valve
VALVULAR HEART
DISEASE
 Valvular heart disease

Valvular involvement by disease causes:

 Stenosis: the failure of a valve to open completely
 impeding forward flow.
 Insufficiency/regurgitation/incompetence: failure of
a valve to close completely  allowing reversed
flow.
 Both stenosis and insufficiency coexist in the same
valve, but one usually predominates.
Dysfunction may affect a single valve or multiple
valves.
 Valvular heart disease
 Functional regurgitation: a valve becomes
incompetent owing to ventricular dilation.
 Abnormalities of flow often produce abnormal heart
sounds known as murmurs.
 Valvular dysfunction can vary in degree from slight
to severe and rapidly fatal.
 Valvular abnormalities may be caused by:
 1. congenital disorders.
 2. acquired diseases.
 Most frequent are acquired stenosis of mitral and
aortic valves ( 2/3 of valves lesions).
 Valvular heart disease

 Major causes of acquired heart valve disease

 Post-inflammatory scarring (Rheumatic heart
disease/ RHD) can cause mitral stenosis,
aortic stenosis, mitral and aortic
regurgitation.
 Insufficiency : intrinsic disease & damage to
supporting structures (aorta, mitral annulus,
tendinous cords, papillary muscle)
 Stenosis: primary cuspal abnormality chronic
process
 Valvular heart disease
 Most frequent chronic causes:
 Mitral stenosis  rheumatic heart disease
 Mitral insuff  myxomatous degeneration
(mitral valve prolapse)
 Aortic stenosis  calcification of
anatomically normal/ congenital bicuspid
aortic valve.
 Aortic insuff  dilation of ascending aorta
related to hypertension and aging.
 Valvular degeneration caused
by calcification
 Valves are subjected to high repetitive
mechanical stresses:
 > 40 million cardiac cycle/year.

 Substantial tissue deformation at each

cycle
 Transvalvular gradient in the closed phase

120 mmHg for mitral & 80 mmHg for aortic \

valve deposition of calcium phosphate
mineral.
Calcific valvular disease

 The most frequent :

- Calcific aortic stenosis.
- Calcification of a congenitally bicuspid
aortic valve.
- Mitral annular calcification.
Calcific aortic stenosis

 Most common of all valvular diseases.

 Congenital or acquired.
 Acquired: consequences of calcification
induced by wear and tear of congenitally
bicuspid/unicuspid valves or of normal
anatomic valves in aged individuals.
 Incidence increasing with the rising average
age of the population.
 Senile calcific aortic stenosis  70es-80es
normal anatomic valves.
Senile calcific aortic stenosis
 Calcific aortic stenosis
 Morphology: calcified masses protrude
through the outlflow surface into the
sinuses of Valsava.
 Primarily at the bases, free cuspal edges
are not involved.
 Usually comissural fusion is absent in
degeneratif aortic stenosis, however the
cusps may be fibrosed and thickened 
comissures fused.
 Calcific aortic stenosis
 Mitral valve is generally normal, in contrast
the patients with rheumatic aortic stenosis
have concomitant abnormality of the mitral
valve.
 CLINICAL FEATURES:
 obstruction to the left ventricular outflow 
 pressure gradient 75-100 mmHg, left
ventricular pressure 200mmHg  left
ventricular hypertrophy.
 Calcific aortic stenosis
 As the stenosis worsens :
 Angina  impaired microcirculatory
perfusion of the hypertrophied myocardium.
 Syncope  poorly understood.
 Congestive heart failure  the exhaustion
of compensatory cardiac hyperfunction 
poor prognosis if not treated by surgery 
death in 50 % patients within 3 yrs.
 Calcification of congenitally bicuspid
aortic valve
 1-2 % of the population.
 The 2 cusps : usually unequal size, larger
cusp have midline raphe  resulting from
incomplete embryologic separation.
 The raphe is the major site of calcific
deposits.
 Bicuspid aortic valve  incompetent as a
result of aortic dilation or cusp prolapse 
infective endocarditis.
Calcification of bicuspid aortic
valve
Mitral annular calcification

 Calcific deposits in the ring of the mitral

valve  irregular,stony hard, ulcerated
nodules (2-5 cm in thickness).
 Generally doesnot affect valvular
function.
 Unusual:it may lead to regurgitation 
systolic contraction of mitral valve ring.
 to stenosis impairing opening of mitral
leaflets.
Mitral annular calcification
 Mitral annular calcification
 To arrhythmias and ocassionally sudden
death  deposit penetrating deeply on AV
conducting system.
 Ulcerated : thrombi  emboli  risk of
stroke.
 Ulcerated nodule : infective endocarditis.
 Is common in women over 60 yrs old, patient
with myxomatous mitral valve or elevated left
ventricular pressure.
 Myxomatous degeneration of
the mitral valve
 Mitral valve prolapse : >3 % of adults
(young women) in USA.
 One or both leaflets: enlarged, hooded,
redundant/floppy and prolapse( balloon
back into the left atrium during systole).
 Incidental finding on physical exam.
 May lead to serious complication in a small
minority of cases.
Floppy Mitral Valve

 Synonyms :
 Mitral valve prolapse
 Barlow’s syndrome
 Balloon deformity
 Myxoid degeneration
 Myxomatous degeneration
 Late systolic murmur / click syndrome
Floppy mitral valve
 Mitral valve prolapse
 Morphology: Intercordal ballooning (hooding) of
mitral leaflets.
 Affected leaflets are thick and rubbery.
 Corda tendineus are elongated, thinned and
ocassionally ruptured.
 Annular dilation is characteristic.
 20%-40% of cases : tricuspid valve involvement.
 Commisural fusion is absent.
 Pathogenesis : unknown, favored is development
anomaly of connective tissue, like: Marfan
syndroma.
Mitral valve prolapse
 Mitral valve prolapse

 Clinical feature: most are asymptomatic.

 Auscultation: mid systolic click.
 3% develop serious complications:
 1.infective endocarditis.
 2.mitral insufficiency e.c. leaflet deformity,dilation of mitral
annulus,cordal lengthening, .and cordal rupture.
 3.Stroke/ systemic infarct,e.c. embolism of leaflet or atrial
thrombi.
 4. Arrhythmia, ventricular or atrial, sudden death is
uncommon.
 The risk is higher in men, older patients and those with
arrhythmia, mitral insuff  holosystolic murmurs, left
enlargement.
Floppy mitral valve & ruptured
chordae tendineae
Infective endocarditis

Is colonization/invasion of the heart valves,

mural endocardium,or other
cardiovascular site by a microbiologic
agent leading to the formation of bulky,
friable vegetations composed of
thrombotic debris and organisms 
bacterial endocarditis.
Classified into acute and subacute
endocarditis.
 Acute endocarditis

 Is a destructive infection with a highly virulent

organism to previously normal valves death
within days to weeks of > 50% patients.
 Tend to produce necrotizing, ulcerative, invasive
valvular infections.
 Clinical features: rapid developing fever, chills,
weakness and lassitude. Complications begin
within the 1st week. Murmur is common. Ring
abscess is frequent. The vegetation are likely to
embolize.
 Subacute endocarditis

 Low virulent organism in previously

abnormal heart (deformed valves).
 Insidiously onset, even untreated 
weeks or months. Recover after
appropriate therapy.
 Less destructive, vegetations often show
evidence of healing.
 Subacute endocarditis

 Clinical features:fever may be slight or absent

(in the elderly), nonspesific fatique, loss of
weight, flulike syndrome. Murmurs in 90% of
patients with left sided lesions but may relate to
preexisting cardiac abnormality.
 Petechiae, subungual hemorrhages, Roth spots
in the eyes(secondary to retinal microemboli)
become uncommon because of shortened
clinical course as a result of antibiotic therapy.
 Cause and pathogenesis
 In years past: RHD was the major contributor.
 Now: myxomatous mitral valve, degenerative calcific
valvular stenosis, bicuspid aortic valve(calcified or
not), artificial valves and vascular grafts.
 Host predisposing factors :neutropenia,
immunodeficiency, therapeutic immunosupression,
diabetes mellitus, alcohol and intravenous drug
abuse,
 platelet-fibrin deposits caused by preexisting cardiac
disease and indwelling vascular catheters.
 Causative organisms
 50-60 % of SBE : alpha hemolytic viridans
streptococci.
 10-20 % of AE and SBE :Staphylococcus aureus,
more virulent, commonly found on the skin  major
in intravenous drugs abusers.
 Enterococci and HACEK group( Haemophilus,
Actinobacillus, Cardiobacterium, Eikenella and
Kingella) all comensal in the oral cavity.
 Coagulase negative staphylococcus
(staphylococcus epidermidis) in prosthetic valves.
 Gram negative bacilli and fungi.
 10% of endocarditis : no organisms.
 Morphology

 AE&SBE: have a friable, bulky,

destructive vegetations containing fibrin,
inflammatory cells, bacteria/other
organisms on the heart valves.
 Most common sites : aortic and mitral.
 Tricuspid may be involved particularly in
intravenous drug abusers.
 Morphology

 Vegetations: single or multiple, if erode

into myocard ring abscess; influenced
by the type of the causative organisms,
the degree of host reaction and previous
antibiotic therapy.
 Microscopy: SBE: granulation tissue at
their base;
 Fibrosis,calcification,chronic
inflammatory infiltrate may develop.
Morphology
Morphology
 Complications
Valvular insuffiency/stenosis with cardiac failure.
Myocardial ring abscess.
Suppurative pericarditis.

Embolic complications:
left sided lesions : cerebral infarcts/abscess/ meningitis,
myocard infarct, abscess of spleen, abscess of the
kidney.
right sided lesion: infarct/abscess/pneumonia of the lung.

Renal complications: infarction, focal and diffuse

glomerulonephritis, multiple abscesses.
 Nonbacterial thrombotic
endocarditis

 Valvular lesions of NBTE are sterile (no organisms)

and loosely attached.
 Often encounter in debilitated patients( cancer/
sepsis)  marantic endocarditis.
 Morphology: deposition of fibrin/other blood elements
on the previously normal valves.
 Sterile, nondestructive, non inflammatory, small(1-5
mm), singly or multiply.
 Microscopy: composed of bland thrombus without
inflammatory reaction and valve damage.
Nonbacterial thrombotic endocarditis
 Pathogenesis

 Occur concomitantly with venous thromboses and

pulmonary embolism relation with hypercoagulable
state e.g: DIC
 Related with cancer, particularly mucin-producing
adenocarcinoma procoagulant effects of circulating
mucin. Occasionally non mucin producing malignancy
e.g: acute promyelocytic leukemia.
 Other diseases promoting hypercoagulability:
hyperestrogenism, extensive burns, sepsis.
 Other conditions: trauma to endocardium
catheterization.
 Endocarditis of SLE
( Libman-Sacks endocarditis)

 Vegetations: small(1-4mm),sterile, pink, granular,

frequently located on undersurfaces of mitral valve,
maybe on other valves, mural endocardium, cords.
 Microscopy: consist of finely granular,fibtrinous
eosinophilic material that may contain hematoxylin
bodies, intense inflammatory reaction, fibrinoid
necrosis.
 Subsequent fibrosis and serious deformity can
result resemble chronic rheumatic heart disease
and require surgery.
Libman-Sacks endocarditis
Prosthetic Cardiac Valve

Two types :
1. Bioprosthetic valves :
- Glutaraldehyde fixed porcine
- Bovine tissue
- Cryo preserved human valves
2. Synthetic (mechanical)
Mechanical prosthetic valve
 Complications

1. Mechanical deterioration :
- All biologic valve undergo some degree of
stiffening  stenosis
- Calcification  stenosis
- Perforation / tear  insufficiency
- Mechanical valve is less susceptible than
biologic one
Complications

2. Thrombus :
Any type  troublesome in mechanical valve
3. Infective endocarditis :
Any type 
mechanical valve : suture line, perivalvular
tissue
The valve leaflet become infected 
perforation  regurgitation
Complications

4. Para valvular leaks : any type

Infection is important cause.

Hemolysis occur in minority of patients with

prosthetic valve particularly mechanical
aortic valve  shearing effect
Infective endocarditis
Infective endocarditis
MYOCARDIAL
DISEASE
Myocardial disease

 Myocarditis : inflammatory reaction.

 Cardiomyopathy : non
inflammatory.
 primary : unknown cause.

 secondary: known cause.

Myocarditis

 Definition (Dallas criteria) : a process

characterized by an inflammatory
infiltrate of the myocardium with necrosis
and/or degeneration of adjacent
myocytes not typical of the ischaemic
damage associated with coronary artery
disease.
 Occur at any age, usually sudden onset
acute cardiac failure or sudden death
by arrhythmia.
Myocarditis

 In many instances the prognosis is good.

 Endomyocardial biopsy  broaden
understanding of nature and course of
carditis.
Myocarditis

 Major causes : virus(Coxsackie A,B)

 Chlamydia psittaci ;Rickettsia typhi –
tsutsugamushi.
 Bacteria: C.diphteria,
Salmonella,mycobactrium, streptococci,
neisseria,leptospira, borrelia.
 Fungi and protozoa: Trypanosoma (chagas
disease),aspergillus, blastomyces,
crytoccocus,candida, coccidioidomyces.
 Metazoa: Echinococcus, Trichinella.
Myocarditis

 Major causes : virus(Coxsackie A,B)

 Chlamydia psittaci ;Rickettsia typhi –
tsutsugamushi.
 Bacteria: C.diphteria,
Salmonella,mycobactrium, streptococci,
neisseria,leptospira, borrelia.
 Fungi and protozoa: Trypanosoma (chagas
disease),aspergillus, blastomyces,
crytoccocus,candida, coccidioidomyces.
 Metazoa: Echinococcus, Trichinella.
Myocarditis

 Immune –mediated reaction: post-

streptococcal (RF), SLE, Systemic
sclerosis, Methyldopa,Sulfonamides,
penicillin, PAS, Streptomycin,Transplant
rejection
 Physical agents: radiation, heat stroke.
 Unknown : sarcoidosis, giant cell
myocarditis (Fiedler’s), Kawasaki’s
disease.
Myocarditis
Myocarditis
Primary cardiomyopathy
 1. Dilated (congestive) CMP
 2. Hypertrophic CMP.
 3. Restrictive (obliterative) CMP.

 Dilated CMP:
 Characterized by gradual development of
cardiac failure associated with four-chamber
dilatation of the heart of unknown cause.
Suspected: alcohol toxicity, pregnancy-
associated nutritional deficiency, genetic defect,
post-viral myocarditis.
Dilated cardiomyopathy

 Peripartum CMP : discovered with the

month before/ after delivery, most often
occurs in frequent multiple pregnancies,
nutritional deficiency, hypertension,
volume overload, metabolic
derangement.
 Half of the patient disappear within
months after delivery, other half  fail to
recover.
 Dilated cardiomyopathy
 Macroscopy: increase weight (900gr), dilatation of
ventricles, mural thrombi because of poor cardiac
contraction and stasis of the blood in chambers. Valve
normal. Coronary artery normal or minimal-moderate
non obstructive atherosclerosis.
 Microscopy: non specific changes, hypertrophy and
atrophy of myocytes, interstitial fibrosis, mild to
moderate thickening of endocardium, foci of
mononuclear inflammatory cells.
 Occur at any ages developing cardiac failure.
 Cardiac transplantation is sometimes recommended.
Primary Cardiomyopathy
(Congestive Type)
 Hypertrophic cardiomyopathy
 Asymetrical septal hypertrophy (ASH), idiopathic
hypertrophic subaortic stenosis (IHSS) and
hypertrophic obstructive CMP.
 Macros : cardiomegaly with myocardial hypertrophy,
left ventricle usually more involved than right. Atria
slightly hypertrophy.
 Micros: disorganization of myofibre haphazard
pattern., disarrays of myofilament within
sarcomeres, interstitial fibrosis. This change is not
uniform.
 > ½ : autosomal dominant inheritance, occur in
young adults but sometimes in elderly.
Restrictive/obliterative
cardiomyopathy
 Restriction of ventricular filling.
 Rare.
 Induced by: cardiac amyloidosis,
sarcoidosis; endocardial fibroelastosis;
endomyocardial fibrosis; Loeffler’s
endocarditis.The last two also called
fibroplatic parietal endocarditis with
blood eosinophilia.
 Senile amyloidosis50% over 70 yrs.
 Endomyocardial fibrosis: children &
young adults in africa.
 Restrictive cardiomyopathy

 Etiology : unknown may be viral,

malnutrition,autoimmun, heavy consumtion of
bananas & plantain  serotonin.
 Loeffler’s endocarditis:large mural thrombi,
eosinophilic leukocytosis, infiltration of eosinophil
in various organ, rapidly fatal.
 Endocardial fibroelastosis: focal or diffuse,
cartilage-like fibroelastic thickening of the mural
endocardium. Most often in the left side but
sometimes both sides or only in the right side.
Primary Cardiomyopathy
(Obliterative Type)
 Secondary cardiomyopathy
 Known etiology or associated with well-defined
systemic disease.
 Major association of secondary CMP:
 Toxic :alcohol,cobalt,catecholamines,CO,
Li,hidrocarbon,As, Cyclophosphamide,
Doxorubicin(adriamycin),daunorubicin.
 Metabolic disease: hyper/hypothyroidism,
hyper/hypokalemia, nutritional deficiency,
hemochromatosis.
 Neuromuscular disease: Friedreich’s ataxia, muscular
dystrophy, congenital atrophies.
Secondary cardiomyopathy

 Storage disorder: Hunter-Hurler

syndrome, glycogen storage disease,
Fabry’s disease, Sandhoff’s disease.
 Infiltrative: leukemia, carcinomatosis,
sarcoidosis.
 Immunologic: post transplant.
 Tumors of the heart
 Primary tumors are extremely rare.
 Found in 1 : 1000 to 10.000 autopsies.
 Metastatic tumors occur in 5 % of patients dying
of cancer.
 The most common primary tumors are : Myxoma,
Lipoma, Papillary fibroelastoma, Rhabdomyoma,
Angiosarcoma, and Rhabdomyosarcoma.
 The first four  70% of all primary tumors.
 The last two 20%.
Myxoma
Rhabdomyoma
rhabdomyoma
Cardiac metastases

 In order of frequency:
 Carcinoma of the bronchus and
breast, Malignant melanoma,
Lymphoma, and Leukemia.
 Most involve pericardium 
asymptomatic/pericarditis. Or
penetrate myocardium  arrhythmia,
congestive failure, obstruction to the
major vessels  thrombosis.
 Corpulmonale
 Right ventricular enlargement secondary to pulmonary
hypertension caused by disorders that affect the
structure/function of the lungs, not caused by the
disease of the left side of the heart and congenital heart
disease.
 Right side counterpart of hypertensive heart disease.
 2 forms:
 Acute cor pulmonale: refer to right ventricular
dilatation that follows massive pulmonary
embolization.
 Chronic cor pulmonale: secondary to prolonged
pressure overload related to narrowing/obstruction of
major artery.
 Cor pulmonale / pulmonary heart
disease
 Etiology:
 Disease of the lung: COPD, diffuse pulmonary
interstitial fibrosis, cystic fibrosis.
 Disease of pulmonary vessels: embolism, vascular
sclerosis, arteritis (Wegener’s granulomatosis), drug-
toxin-radiation induced vascular sclerosis.
 Disorder affecting chest movement: kyphoscoliosis,
marked obesity , neuromuscular diseases.
 Disorder inducing pulmonary arteriolar constriction:
 Metabolic acidosis, hypoxemia ( chronic altitude
sickness, obstruction to major airways, idiopathic
alveolar hypoventilation.