Restrictive Cardiomyopathy Flashcards
most common causes of RCM
- infiltrative diseases (amyloidosis, sarcoidosis, hemochromatosis)
- lysosomal storage diseases (Fabry disease)
- endomyocardial processes (endomycardial fibrosis, HES, carcinoid, radiation therapy, anthracycline toxicity)
- noninfiltrative diseases (scleroderma)
RCM causes abnormally rigid ventricular walls causing?
diastolic dysfunction WITHOUT systolic dysfunction
in RCM, diastolic dysfunction WITHOUT systolic dysfunction manifests as?
- impaired ventricular filling
- elevated diastolic ventricular pressures
pulmonary manifestations of RCM
- pulmonary venous congestion
- PH
- RVHF
JVD on inspiration
Kussmaul sign
cardiac catheterization results in RCM
elevated LVEDP and RVEDP
clues to underlying systemic diseases causing RCM:
- amyloidosis
- neuropathy
- proteinuria
- hepatomegaly
- periorbital ecchymosis
- bruising
- low-voltage ECG
confirmation of dx of amyloidosis
abdominal fat pad aspiration
clues to underlying systemic diseases causing RCM:
- sarcoidosis
- B/L HLAD
- skin, joint, or eye lesions
- arrhythmias, conduction blocks, or HF
clues to underlying systemic diseases causing RCM:
- hemochromatosis
- elevated transaminases
- OA
- DM
- ED
- HF
- elevated serum ferritin and transferrin saturation levels
clues to underlying systemic diseases causing RCM:
- Fabry disease
- XR
- consider in males w/ HF, urinary concentrating defects, proteinuria, CKD, premature CAD, painful neuropathy, angiokeratomas
confirmation of dx of Fabry disease
low a-galactosidase A level
what must RCM be differentiated from?
constrictive pericarditis
tx for RCM
tx underlying disease
tx for dyspnea and peripheral edema in RCM
loop diuretics
