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Restrictive Cardiomyopathy (RCMP)

Clinical Subject Page

Restrictive Cardiomyopathy (RCMP)

ICD-10

I42.5

Specialty

Cardiology

Onset

Chronic

Reviewed

June 2026

On This Page

Overview

Restrictive cardiomyopathy (RCMP) is a myocardial disease characterized by impaired ventricular filling due to increased ventricular stiffness, while systolic function is usually preserved in the early stages.

Etiology & Risk Factors

Etiology of Restrictive Cardiomyopathy :

  • Idiopathic
  • Cardiac amyloidosis (most common)
  • Sarcoidosis
  • Hemochromatosis
  • Endomyocardial fibrosis
  • Radiation-induced heart disease
  • Fabry disease
  • Storage diseases

Risk Factors for Restrictive Cardiomyopathy :

  • Older age
  • Family history of cardiomyopathy
  • Amyloidosis
  • Hemochromatosis
  • Autoimmune disease
  • Prior chest radiation
  • Infiltrative or storage disorders

Pathophysiology

Infiltrative / Fibrotic myocardial disease
↓
↑ Ventricular stiffness
↓
Impaired ventricular relaxation
↓
↓ Ventricular filling (Diastolic dysfunction)
↓
↑ Filling pressures
↓
Biatrial enlargement
↓
Pulmonary congestion + Right-sided heart failure

Clinical Presentation

Symptoms of Restrictive Cardiomyopathy :

  • Progressive exertional dyspnea
  • Fatigue
  • Peripheral edema
  • Ascites
  • Orthopnea
  • Palpitations
  • Syncope (occasionally)

Signs of Restrictive Cardiomyopathy :

  • Elevated JVP
  • Peripheral edema
  • Hepatomegaly
  • Ascites
  • S4 heart sound
  • Kussmaul sign
  • Pleural effusions

History Taking

Key Questions of Restrictive Cardiomyopathy :

  • Progressive dyspnea?
  • Leg swelling?
  • Orthopnea or PND?
  • Palpitations?
  • Syncope?
  • Family history of cardiomyopathy?
  • History of amyloidosis or hemochromatosis?
  • Previous chest radiation?
  • Autoimmune disease?

Red Flags for Restrictive Cardiomyopathy :

  • Syncope
  • Rapidly progressive heart failure
  • Ventricular arrhythmias
  • Signs of systemic amyloidosis

Investigations

Laboratory

  • CBC
  • Renal & liver function
  • BNP/NT-proBNP
  • Troponin
  • Iron studies
  • Serum/urine protein electrophoresis
  • Serum free light chains

ECG

  • Low-voltage QRS (amyloidosis)
  • Atrial fibrillation
  • Conduction abnormalities
  • Nonspecific ST-T changes

Imaging

  • Chest X-ray
  • Echocardiography (first-line)
    • Normal or small ventricles
    • Biatrial enlargement
    • Preserved EF
    • Severe diastolic dysfunction
  • Cardiac MRI
    • Infiltrative disease
    • Myocardial fibrosis

Additional Tests

  • Endomyocardial biopsy (selected cases)
  • Genetic testing
  • Nuclear imaging for cardiac amyloidosis

Diagnosis

Diagnosis is based on:

  • Clinical features of heart failure
  • Echocardiographic evidence of restrictive filling
  • Cardiac MRI findings
  • Identification of the underlying cause
  • Endomyocardial biopsy when indicated
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Management

General Measures

  • Sodium restriction
  • Fluid restriction (if congested)
  • Treat the underlying cause

Medications

  • Loop diuretics (symptom relief)
  • Mineralocorticoid receptor antagonists (selected patients)
  • Anticoagulation for atrial fibrillation
  • Disease-specific therapy (e.g., tafamidis for ATTR amyloidosis, chemotherapy for AL amyloidosis)

Advanced Therapy

  • Pacemaker (if conduction disease)
  • Heart transplantation (selected patients)

Complications

  • Chronic heart failure
  • Atrial fibrillation
  • Ventricular arrhythmias
  • Thromboembolism
  • Pulmonary hypertension
  • Sudden cardiac death

Prognosis

  • Depends on the underlying cause.
  • Cardiac amyloidosis generally has the poorest prognosis.
  • Early diagnosis and disease-specific therapy improve outcomes.
  • Advanced disease may require heart transplantation.

Key Points / Clinical Pearls

  • RCMP primarily causes diastolic dysfunction with preserved EF.
  • Amyloidosis is the most common infiltrative cause.
  • Biatrial enlargement is a classic echocardiographic finding.
  • Cardiac MRI helps identify infiltrative myocardial disease.
  • Treatment focuses on managing the underlying cause and relieving congestion.
  • Differentiate RCMP from constrictive pericarditis, as management differs significantly.