Page 10 - Heart Failure Clinical Guidelines

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III. RESTRICTIVE CARDIOMYOPATHY

A. Initial Evaluation for Patient with Restrictive Cardiomyopathy
1. Additional Tests
MRI with contrast (evaluate function, fibrosis, doppler respiratory flow changes, pericardium)
Laboratories
1. CK
2. Acyl Carnitine Profile
3. Urine Analysis with Micro
4. Urine Organic Acids
5. Serum Amino Acids
6. Restrictive Cardiomyopathy Gene Panel (Include Desmin, RSK2, Lamin A/C)
Other Laboratories to Consider Based on History & Physical
1. Serum ACE level (older teenagers)
2. Chromosomal Microarray
3. Hemoglobin Electrophoresis
4. Selenium level
5. Cardiac Catheterization to asses PA pressure, LVEDP and RVEDP difference

B. Medical Management for Restrictive Cardiomyopathy

1. Carvedilol/Beta Blockers
Indication
-treatment of symptomatic left ventricular diastolic or systolic dysfunction

2. Diuretics
Indication
-Congestive symptoms

3. Anticoagulation
Indications
a) indwelling central venous catheter
b) previous thrombus or embolic event
c) persistent or uncontrolled atrial fibrillation or atrial flutter

C. Long-Term Follow-up Schedule for Restrictive Cardiomyopathy

1. Asymptomatic with Normal Function
1. F/U q6 months (q3 months if <3yo)
2. Echo q6 months
3. CBC, CMP, BNP q 6 months
4. EKG q6 months
5. Holter/Event Monitor q1-2 years
6. Exercise with Metabolic q3 years
7. Catheterization for PVR q1-2 years