Cover image: Restrictive cardiomyopathy results from fibrosis or infiltrative disease of the myocardium, with the most frequent cause being amyloidosis | Medcomic/Jorge Muniz

Introduction

Restrictive cardiomyopathy (RCM) is a form of cardiomyopathy in which the walls are rigid, and the heart is restricted from stretching and filling with blood properly.

• Diastolic dysfunction
• L/C cardiomyopathy

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Aetiology

• PRIMARY
  1. Löffler’s endocarditis
     • Eosinophils in the heart
  2. Endocardial fibroelastosis (children)
     • Fibrosis of endocardium + subendocardium
• SECONDARY
  • Infiltitative:
    1. Cardiac amyloidosis
       • Familial amyloid cardiomyopathy
         • TTR (transthyretin) mutation
       • Senile cardiac amyloidosis (elderly)
         • Wild-type TTR deposition in heart
    2. Sarcoidosis
       • Granulomas
    3. Hemochromatosis
  • Interstitial:
    • Radiation → reactive oxygen species → Fibrosis

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Pathophysiology

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Diagnosis

ECG

• Low amplitude QRS

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Management

• Treat underlying cause