Cardiomyopathies Flashcards
final common pathway of cardiomyopathy
CHF, arrhythmia, death
compensatory mechanisms of cardiomyopathy
- left ventricular enlargement
- Frank starling mechanism
eventually these are exceeded and cardiac output decreases = heart failure
diagnostic tools used to evaluate cardiomyopathy
clinical suspicion (FH, exam) + echocardiogram + cardiac MRI
test of choice for all cardiomyopathies
ECHOcardiogram
normal thickness of LV = <15 mm or 1.5 cm
types of primary cardiomyopathies
genetic
mixed (genetic v. non genetic)
acquired
types of genetic cardiomyopathies
hypertrophic cardiomyopathy
arrhythmogenic right ventricular cardiomyopathies
hypertrophic cardiomyopathy
genetic mutation
disorder of myocardium caused by mutations of sarcomere or sarcomere-associated proteins
autosomal dominant
Pathophys of hypertrophic cardiomyopathy
Septal hypertrophy = thickening of left ventricular septum
impaired relaxation, secondary to ventricular wall thickness, and loss of elasticity = DIASTOLIC issue
hard to fill the heart
abnormalities of hypertrophic cardiomyopathy
myocardial ischemia
mitral regurgitation
arrhythmias
MC CAUSE OF DEATH IN YOUNG ATHLETES
clinical features of hypertrophic cardiomyopathy
syncope, SOB, chest pain
S4 heart sound (atrial contraction against no complaint vent)
ventricular arrhythmias
sudden death
s4 heart sound
atrial diastolic gallop
“Tah” Lub Dub
found before S1
diagnostic eval of hypertrophic cardiomyopathy
clinical suspicion + ECG+ echocardiogram *** TOC + cardiac MRI
can genetic test
treatment of hypertrophic cardiomyopathy
activity restriction
Anti-arrhythmics
surgical tx
pharm treatment of hypertrophic cardiomyopathy
beta blockers - Metoprolol/Lopressor (sluggish)
avoid inotropes (digoxin, epinephrine, dopamine)
avoid vasodilators (CCB, nitrates, hydralazine)
hypertrophic cardiomyopathy surgical treatments
surgical septal myectomy
alcohol septal ablation
implantable cardio defibrillator
arrhythmogenic right ventricular cardiomyopathy pathophys
fibro-fatty infiltration of RIGHT ventricle
causes r. sided heart failure and various rhythm disturbances
precipitated by catecholamine release during exercise
second mc cause of death in young athletes
arrhythmogenic right ventricular cardiomyopathy
phases of presentation
- Concealed phase (asymptomatic)
- Electrical phase- palpitations or syncope
- diffuse phase - right sided heart failure
diagnostics of arrhythmogenic right ventricular cardiomyopathy
EKG - suggestive
Echocardiogram (test of choice)
cardiac MRI
arrhythmogenic right ventricular cardiomyopathy treatment
activity restriction
anti-arrhythmic drugs
implantable cardio defibrillator
athletic ventricular hypertrophy
NOT cardiomyopathy
endurance training, increased thickness
can cause thickness but <14mm
types of mixed cardiomyopathies
restrictive
dilated
restrictive cardiomyopathies
rarest form
excessive rigidity of ventricle walls
diastolic dysfunction
restrictive cardiomyopathies etiologies
infiltrative disease (amyloidosis, sarcoidosis)
inflammatory
clinical features of restrictive cardiomyopathies
HF with normal sized heart
SOB (orthopnea, PND)
R. heart failure symptoms (JVD, periperhal edema, ascites, S4 heart sound)
SMALL STIFF heart
pathogenic process of restrictive cardiomyopathy
BACKING UP ISSUE
one side backs up causes back up on other
i.e. pulmonary congestion causes RV to back up, then RA, and systemic congestion occurs
restrictive cardiomyopathy
diagnostic
clinical suspicion + ECG + echocardiogram + cardiac MRI + Endomyocardial biopsy
restrictive cardiomyopathy
treatment
no treatment of restrictive issue
can treat HF, arrhythmia
dilated cardiomyopathy
poor contraction
low ejection fraction
heart dilates to compensate
MC form of cardiomyopathy
etiologies of dilated cardiomyopathy
ETOH (mc cause)
genetics (chronic A Fib, DM, SLE, muscular dystrophy)
drugs/toxins (chemotherapy, anti=rheumatic)
clinical presentation fo dilated cardiomyopathy
heart failure (SOB, crackles, S3)
mitral regurge, arrhythmia, death
peripheral edema
S3 heart sound
ventricular diastolic gallop
found shortly after S2
dilated cardiomyopathy diagnostics
CXR (infiltrates)
ECG
Echocardiogram (left vent. remodeling, ejection fraction <25%)
dilated cardiomyopathy treatment
eliminate reversible causes
treat HF, thrombosis risk, or dysrhythmia
pharm tx of dilated cardiomyopathy
beta blockers (decrease HR)
vast dilators (ace inhibitors, decrease after load + nitroglycerin, preload and after load decrease)
diuretics
antigoaculants
DIGOXIN
types of acquired cardiomyopathies
myocarditis
permpartum cardiomyopathy
stress related, “Takotsubo”
myocarditis
inflammatory process with necrosis of myocardium
can be asymptomatic, fever symptoms, to acute HF and death
etiologies of myocarditis (BROAD)
viruses bacteria fungus parasties protozoan autoimmune conditions
BASICALLY EVERYTHIng
pathophysiology myocarditis
typically caused by viral infection
histologic hallmark - focal patchy, diffuse inflammatory infiltrate with adjacent myocyte injury
myocarditis clinical presentation
asymptomatic
overshadowed or masked by symptoms of illness
typically come in after acute flu like syndrome
myocarditis diagnostics
ECG
positive cardiac enzymes** (troponin elevated)
echocardiogram
endomyocardial biopsy
