8: Cardiomyopathies Flashcards
Dilated cardiomyopathy- dx
Echocardiogram (MC)*
Radionuclide ventriculography- Ejection fraction
-EF is best prognostic indicator
Dilated cardiomyopathy
Autosomal dominant
Peripartum cardiomyopathy (PPCM)
Development of CHF in the last month of pregnancy or within 5 months after delivery*
DCM
Takotsubo cardiomyopathy
stress induced
DCM
Hypertrophic cardiomyopathy (HCM)
autosomal dominant
sudden death in young athletes
Apical or Concentric hypertrophy
Obstructive or non obstructive
- obstructive is due to systolic anterior movement of MV anterior leaflet (SAM)+ Asymmetric septal hypertrophy (ASH)
Echocardiography + Doppler studies- dx (Hallmarks: SAM, ASH, MR)
Restrictive cardiomyopathy (RCM)
Endomyocardial fibrosis
Eosinophilic/ Loeffler endomyocarditis
Amyloidosis, Hemochromatosis, Sarcoidosis, Glycogen storage disease
Pericardial constriction and RCM may be indistinguishable**
-Kussmaul sign: JVP normally fails to fall during inspiration and may actually rise in constrictive pericarditis (less so in RCM)
Ddx- CP vs RCM
CP: Pericardial knock, high frequency sound. No murmurs. Pericardial calcification on x-ray. Normal sized ventricles and atria. Respiratory changes during inspiration. Equal right and left filling pressure.
RCM: peripheral stigmata of systemic disease. Presence of loud diastolic filling sound S3, low frequency sound. Murmurs of mitral+tricuspid insufficiency. Normal chest x-ray. Marked dilatation of both atria. Greater elevation in the left sided filling pressure. Bx: shows abnormal myocardium.
*pericardial thickening is not reliably observed on echocardiography; MRI is suggested for exclusion of a thick pericardium
Echocardiogram
DCM: LV dilatation+dysfunction
HCM: ASH, SAM
RCM: increased LV wall thickness
Arrhythmogenic right ventricular cardiomyopathy (ARVC)
Autosomal dominant
Palmoplantar keratoderma and wooly hair
Dilated thin RV