Restrictive cardiomyopathy

Background

• Muscle is stiff from fibrosis or infiltrating process
• EF is usually normal
• Form of diastolic dysfunction (difficulty filling)
• Must distinguish from constrictive pericarditis

Differential Diagnosis

Cardiomyopathy

• Dilated cardiomyopathy
• Hypertrophic cardiomyopathy
• Restrictive cardiomyopathy
• Peripartum cardiomyopathy
• Takotsubo cardiomyopathy
• Arrhythmogenic right ventricular dysplasia
• Restrictive cardiomyopathy ddx
  • Idiopathic
  • Familial noninfiltrative disease
  • Infiltrative disease (amyloidosis, sarcoidosis, Gaucher, Hurler)
  • Storage diseases (hemachromatosis, Fabry, glycogen/lysosomal storage diseases)
  • Diabetes
  • Scleroderma
  • Endomyocardial fibrosis (hypereosinophilic syndrome, medication toxicity)
  • Radiation, chemotherapy (doxorubicin)
  • Metastatic disease, carcinoid syndrome

Evaluation

• Consider if CHF but no evidence of cardiomegaly or systolic dysfunction

Management

• Symptom directed (diuretics and ACEI)

See Also

• Cardiomyopathy (Main)