Myocardial and Pericardial Disease Flashcards
Myocarditis
inflammatory reaction within the myocardium
Clinical, functional and pathologic patterns of Myocardial disease
dilated (90%)
hypertrophic
restrictive (least common)
Diagnosis of myocardial disease depends on
endomyocardial biopsy; small pieces of tissue removed from right ventricular septum
Dilated cardiomyopathy
gradual development of cardiac failure with 4-chamber hypertrophy and dilatation of heart of unknown etiology
Suspected etiologies of dilated cardiomyopathy
alcohol or other toxicities; previousmyocarditis; pregnancy assocaited with nutritional deficiency or immunologic
Clinical picture of dilated cardiomyopathy
slowly developing CHF which is progressive and unremitting; may have precipitous decompensation; 50% die within 2 years
treatment for dilated cardiomyopathy
transplant
Gross morphology of dilated cardiomyopathy
large, dilated hearts 2-3X normal size; all chambers involved; wall thickness may be normal; mural thrombi common; functional mitral regurgitation
Histology of dilated cardiomyopathy
most myofibers hypertrophied stretched; interstitial and endocardial fibrosis; replacement fibrosis
Two kinds of hypertrophic cardiomyopathy
idiopathic hypertrophic subaortic stenosis (IHSS)
hypertrophic obstructive cardiomyopathy
Clinical associations of hypertrophic cardiomyopathy
heaving muscular hypercontracting heart
Heart failure with hypertrophic cardiomyopathy is due to
decrease in chamber size, poor compliance with decreased stroke volume (diastolic failure)
What must hypertrophic cardiomyopathy be differentiated from
amyloidosis and hypertensive heart disease
Hypertrophic cardiomyopathy is what percent genetic
100% genertic causes
genetic causes of hypertrophic cardiomyopathy
sarcomere proteins with direct sarcomeric dysfunction; defect in energy transfer from mitochondria
Problems resulting from hypertrophic cardiomyopathy
atrial fibrillation with mural thrombus formation; infective endocarditis on mitral valve; intractable CF; sudden death
Morphology of IHSS
disproportionate thickening of ventricular septum compared to free wall
Morphology of hypertrophic cardiomyopathy
endocardial thickening or mural plaque formation of left ventricular outflow tract; thickening of anterior mitral leaflet secondary to contact of anterior mitral leaflets with septum during ventricular systole
Histology of hypertrophic cardiomyopathy
extensive myocyte hypertrophy; myofiber disarray; interstitial and replacement fibrosis
Restrictive cardiomyopathy
diastolic disorder; diastolic relaxation and left ventricular filling impeded by inability of myocardium to expand; contractile (systolic) function of ventricle usually unaffected
Restrictive cardiomyopathy results from
infiltrative diseases such as amyloidosis, hemochromatosis, leukemia, or storage disease; also radiation fibrosis, constrictive pericarditis
endocardial fibrosis
child, young adults in Africa and tropical countries; fibrosis or ventricular endocardium and subendocardium; extends from apex to inflow tract of rt/lt ventricles; may involve mitral, tricuspid valves; ventricular mural thrombi
Loeffler’s endomyocarditis
similar to endocardial fibrosis but unrestricted to specific geographic area; eosinophils, leukocytosis; involvement of other organs; eosinophils appear to be functionally abnormal
Endocardial fibroelastosis
focal or diffuse; cartilage-like fibroelastic thickening; first 2 years; often associated with congenital abnormalities (aortic valve obstruction)
Toxic, metabolic, and other specific causes of myocardial disease
- alcohol
- adriamycin and other drugs
- catecholamines
- peripartum state
- amyloidosis
- iron overload
- hyperthyroidism and hypothyroidism
Alcohol and myocardial disease
ETOH or its metabolites have direct toxic effect; may be complicated by thiamin deficiency (beriberi heart disease)
Adriamycin and other drugs and myocardial disease
anthracycline chemotherapeutic agents doxorubicin, daunorubicin, lithiu, phenothiazines, cocaine; attributed to lipid peroxidation of myofiber membranes; myofiber swelling and vacuolization; resolves with dissolution of the drug
Catecholamines and myocardial disease
pheochromocytoma - contraction band necrosis; dopamine, cocaine
Peripartum state and myocardial disease
globally dialted heart; due to hypertension, volume overload; nutritional deficiency
Amyloidosis
may be part of systemic amyloidosis or only affect heart, particiarly with senile isolated cardiac amyloidosis; two forms: ventricular (transthyretin) and atrial
Iron overload (hemochromatosis, hemosiderosis) and myocardial disease
more prominent in ventricles than atria; interferes with metal-dependent enzyme systems; accumulation of hemosiderin within cardiac myocytes
Hyperthyroidism and myocardial disease
tachycardia, palpitations, cardiomegaly, supraventricular arrhythmias; direct and indirect effect of thyroid hormones on the cells of the heart; alter plasma membrane function, responsiveness to sympathetic stimulation
Hypothyroidism and myocardial disease
decreased cardiac output with reduced stroke volume and rate; increased peripheral resistance (cold sensations); myxedema; heart is flabby, enlarged, dilated; myofiber swelling with loss of striation and basophilic degeneration, interstitial mucopolysaccharide-rich edema fluid
Myocarditis
inflammatory involvement of the heart muscle characterized by leukocytic infiltrate and resultant non-ischemic necrosis or degeneration of myocytes
Most cases of myocarditis are
viral in origin
Patients most vulnerable to myocarditis
infants, immunosuppressed, pregnant women
Viruses that cause myocarditis
Coxsackievirus A and B; ECHO virus; poliovirus; influenza A and B
Nonviral causes of myocarditis
- Chaga’s disease
- trinosis
- Corynebacterium diphtheriae
- Lyme disease
- AIDS
Chaga’s disease
Trypanosoma cruzi; endemic in South America; myocardial involvement found in 80%
Corynebacterium diphtheriae
patchy myocyte necrosis with only sparse lymphocytic infiltrate
AIDS patients and myocarditis
a. inflammation and myocyte damage without a clear etiologic agent
b. myocarditis caused directly by HIV or by opportunitistic pathogen
Non-infectious causes of myocarditis
hypersensitivities (drugs); rheumatic fever, SLE; sarcoidosis
Morphology of myocarditis
interstitial mononuclear, predominantly lymphocytic inflammatory infiltrate; focal necrosis
Giant cell myocarditis
widespread inflammatory cellular infiltrate containing multinucleate giant cells, interspersed with lymphocytes, eosinophils, plasma cells, and macrophages with necrosis
Pericardial disease almost always associated with
disease in othe rportions of the heart or surrounding structures
Normal fluid in pericardium
normal 30-50 ml of thin, clear, straw-colored translucent fluid
How do pericardial effusions usually accumulate
slowly and rarely exceed 500 ml
Hemopericardium
due to rupture of heart wall secondary to MI, traumatic performation, or rupture of intrapericardial aorta; leads to cardiac tamponade
Serous pericarditis
non-infectious inflammation (RF, SLE, scleroderma, tumors, uremia)
Most frequent type of pericarditis
Fibrinous and serofibrinous pericarditis
Fibrinous and serofibrinous pericarditis
serous fluid mixed with fibrinous exudate; MI, Dressler’s syndrome; uremia, chest irradiation, rheumatic fever, trauma
Dressler’s syndrome
autoimmune conditions which appear several weeks after MI
Most striking characteristic of fibrinous and serofibrinous pericarditis
loud pericardial friction rub
Purulent or suppurative pericarditis
invasion of organisms into pericardial space; organization may lead to restrictive pericarditis
Hemorrhagic pericarditis
exudate composed of blood mixed with a fibrinous or suppurative effusion; tuberculosis, direct malignant neoplastic involvement of the pericardial space
Types of pericarditis
- Serous pericarditis
- Fibrinous and serofibrinous pericarditis
- Purulent or suppurative pericarditis
- Hemorrhagic pericarditis
- Caseous pericarditis
- Chronic pericarditis
Chronic pericarditis
often denotes healed or organized effusions which may lead to fibrosis, adhesive percarditis; restrictive pericarditis
Myxoma
most common primary tumor of the heart in adults; 90% are located in the atria
Lipoma
may occur in subendocardium, subepicardium or within myocardium
Fibroelastoma
generally located on valves as Lambl’s excrescences; incidental lesions
Rhabdomyosarcoma
most frequent primary tumor of the heart in children; discovered in first years of life because of obstruction of the outflow tract or cardiac chamber
Noncardiac Neoplasms
- carcinoid, pheochromocytoma, myeloma-associated amyloidosis
- metastatic diseae
Morphology of dilated cardiomyopathy
four-chamber hypertrophy; associated with mural thrombi; patchy myocardial scars
Morphology of idiopathic hypertrophic subaortic stenosis
asymmetric septal hypertrophy; myofiber disarray; subaortic hypertrophy leads to outflow obstruction
Adhesive mediastinopericarditis
results when the pericardial sac is obliterated and the cardiac structures are adhered to surrounding structures, greatly increasing the workload of the heart; heart is free to contract but pulls on adjacent structures leading to hypertrophy and dilatation
Morphology of viral myocarditis caused by Coxsackie A and B
interstitial mononuclear lymphocytic infiltrate
Morphology of viral myocarditis caused by HIV
focal necrosis of myocytes
Morphology of viral myocarditis caused by ECHO, polio, and influenza virus
post-infectious fibrosis
Morphology of Chaga’s disease myocarditis
parasitism of myocytes with scattered inflammatory infiltrate
Morphology of Trinchinella myocarditis
encysted trinchinella with inflammatory infiltrate, eosinophils
Morphology of Corynebacterium diptheriae myocarditis
patchy myocyte necrosis with sparse lymphocyte infiltrate
Morphology of alcohol related myocardial disease
dilated myocardial disease
Morphology of Adriamycin (Doxorubicin, daunorubicin) related myocardial disease
myofiber swelling and vacuolization, fatty change, myocytolysis
Morphology of catecholamine-related myocardial disease
foci of myocardial necrosis with contraction bands; monocytic infiltrate; similar to reperfusion injury
Morphology of peripartum state myocardial disease
globally dilated heart
Morphology of iron overload associated myocardial disease
hemosiderin within cardiac myocytes; cellular degeneration and replacement fibrosis; dilated myocardial disease
Morphology of hyperthyroidism related myocardial disease
nonspecific hypertrophy
Morphology of hypothyroidism related myocardial disease
dilated myocardial disease with myxedema; deposition of mucopolysaccharide-rich edema fluid; myofiber swelling