Etiology

Mnemonic

“PLEASe Help!”: Causes of restrictive cardiomyopathy include Postradiation/Postsurgery fibrosis, Löffler endocarditis, Endocardial fibroelastosis, Amyloidosis, Sarcoidosis, and Hemochromatosis.

Infiltrative cardiomyopathy

  • Amyloidosis: caused by an accumulation of abnormal proteins in the myocardium
    • Most common cause of restrictive cardiomyopathy in high-income countries.
    • See ATTRmt amyloidosis
  • Sarcoidosis: secondary to deposition of granulomas in the myocardium

Pathophysiology

Infiltration (e.g., abnormal proteins, glycogen, eosinophils, granulomas, iron) or proliferation of connective or fibrotic tissue → ↓ elasticity of myocardium → ↓ ventricular compliance (severe diastolic dysfunction)


Clinical features


Diagnostics

Differ from Hypertrophic cardiomyopathy by EF = 25~50%, while restrictive cardiomyopathy > 60%


Treatment