Myocarditis Flashcards
dilated cardiomyopathy: clinical associated
-gradual development of cardiac failure
-4-chamber hypertrophy and dilatation
-20% -50% genetic, familial
-myocardial degeneration - defect in force generation,
force transmission and/or signaling
-common end pathway for a variety of myocardial insults
-suspected etiologies:
-alcohol or other toxicities
-previous myocarditis
-pregnancy associated with nutritional deficiency or immunologic
primary abnormality
impairment of left ventricular function; end
stage ejection fraction <25% (systolic failure)
dilated cardiomyopathy characteristics
- may occur at any stage age, usually 20-60
- clinical picture: slowly developing CHF
- may have precipitous decomposition, 50% die in 2 years
- need transplant
etiologist for dilated cardiomyopathy
- alcohol, chemotherapeutic agents (doxorubicin/Adriamycin)
- Familial (25-35%): muscular dystrophy, cytoskeletal
morphology of dilated cardiomyopathy
large,”globular” dilated hearts 2-3X normal size
-all chambers involved; wall thickness may be = normal
-mural thrombi common, especially near apex of
ventricles, atria
-functional mitral or tricuspid regurgitation
-left ventricle - patchy subendocardial scars; ?ischemic
-histology - most myofibers hypertrophied, stretched,
vacuolization
-interstitial and endocardial fibrosis; REPLACEMENT
fibrosis
alcohol
-most common cause of dilated cardiomyopathy
-ETOH or its metabolites (acetaldehyde) has direct toxic
effect
-may be complicated by thiamin deficiency (beriberi heart
disease)
toxic, metabolic, and other
alcohol hyperthyroidism, hypothyroidism peripartum state (pregnancy) adriamycin and other drugs catecholamines
pregnancy
- globally dilated heart
- ?due to hypertension, volume overload
- nutritional deficiency
adriamycin and other drugs
-anthracycline chemotherapeutic agents doxorubicin,
daunorubicin - dose dependent
-also lithium, phenothiazines, cocaine
-attributed to lipid peroxidation of myofiber membranes
-myofiber swelling and vacuolization
-usually resolves on discontinuation of drug
catecholamines
- pheochromocytoma - contraction band necrosis
- also dopamine, cocaine
where else are contraction bands necrosis
seen with reperfusion injury
cocaine
catecholamines
hypertrophic cardiomyopathy
-Idiopathic hypertrophic subaortic stenosis -IHSS
-Hypertrophic obstructive cardiomyopathy
-non-hypertensive heaving muscular hyper-contracting
heart
-100% genetic – defects in sarcomeric proteins
-Autosomal dominant with variable penetrance
-Missense mutations
-sometimes try surgical reduction of septum
problems hypertrophic cardiomyopathy
atrial fibrillation, endocarditis, sudden death
what causes the heart failure seen with hypertrophic cardiomyopathy
-heart failure due to decrease in chamber size, poor
compliance with decreased stroke volume (diastolic failure)
what is seen with hypertrophic cardiomyopathy
-massive myocardial hypertrophy
-IHSS: disproportionate thickening of ventricular septum;
asymmetric hypertrophy (10% symmetric)
-jet lesions
-endocardial thickening/mural plaque formation of left
ventricular outflow tract; thickening of anterior mitral
leaflet 2° to contact with septum during systole
histology of hypertrophic cardiomyopathy
-extensive myocyte hypertrophy; myocyte diameter >40 um
(normal 15 um)
-myofiber disarray; interstitial and replacement fibrosis
glycogen storage disease
pompe’s disease
pompe’s disease
- acid alpha-glucosidase deficiency
- accumulation of normal glycogen in cells - heart failure in infants
- also juvenile, adult forms - restrictive heart disease
- left ventricular hypertrophy with outflow obstruction
- death from heart failure by age 1
restrictive cardiomyopathy: clinical associations
-also a diastolic disorder
-diastolic relaxation and left ventricular filling impeded by
inability of myocardium to expand
-contractile (systolic) function of ventricle usually unaffected
-results from infiltrative diseases such as amyloidosis,
hemachromatosis, leukemia, or storage diseases
-also radiation fibrosis, constrictive pericarditis
-endocardial fibrosis