Restrictive cardiomyopathy
Restrictive cardiomyopathy is impaired ventricular filling from a stiff, noncompliant myocardium with preserved (or near-preserved) systolic function and non-dilated ventricles, most often from infiltration such as amyloidosis. It presents as predominantly right-sided heart failure, and diagnosis centers on echocardiography and cardiac MRI plus a search for the underlying cause, distinguishing it from constrictive pericarditis; management treats the underlying cause with cautious diuresis.
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What this article covers
- Overview
Definition: restrictive cardiomyopathy is impaired ventricular filling from an abnormally stiff, noncompliant myocardium, with preserved or near-preserved systolic function and non-dilated ventricles.
- Epidemiology
The least common cardiomyopathy overall. Cause varies by region: amyloidosis and other infiltration in developed countries; endomyocardial fibrosis in tropical regions.
- Risk factors
Infiltrative and storage diseases: amyloidosis, sarcoidosis, and hemochromatosis.
- Pathophysiology
Increased myocardial stiffness: infiltration or fibrosis reduces compliance, so ventricles fill poorly at high pressures.
- Clinical features
Right-sided congestion: elevated jugular venous pressure, hepatomegaly, ascites, and peripheral edema.
- Differential diagnosis
Constrictive pericarditis: the key mimic, surgically treatable, distinguished by pericardial thickening or calcification and respiratory ventricular interdependence, absent in restriction (constrictive pericarditis).
- Diagnosis
Establish with echocardiography showing restrictive filling with preserved EF and non-dilated ventricles, then identify the cause and exclude constriction: the diagnostic basis is imaging…
- Management
First-line, treat the underlying cause: disease-specific therapy such as tafamidis 61 mg PO daily for transthyretin amyloidosis, chemotherapy for light-chain amyloidosis, phlebotomy or chelation for…
- Complications
Progressive heart failure and low-output state.
- Prognosis
Depends on the cause: light-chain amyloidosis has a poor prognosis, whereas treated transthyretin amyloidosis and hemochromatosis fare better.
- Prevention
Early diagnosis and cause-specific therapy (for example tafamidis in transthyretin amyloid) to slow progression.
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Related articles
- Cardiac amyloidosis
Cardiac amyloidosis is myocardial infiltration by misfolded amyloid fibrils, chiefly transthyretin (ATTR) or light-chain (AL) type, causing a restrictive, thick-walled cardiomyopathy with heart failure, conduction disease, and low-voltage ECG despite increased wall thickness. Diagnosis distinguishes AL (light chains plus biopsy) from ATTR (a positive bone-tracer scan without a plasma cell disorder), and treatment is type-specific plus supportive heart-failure care.
- Constrictive pericarditis
Constrictive pericarditis is a rigid, often calcified pericardium that impairs diastolic filling, causing predominantly right-sided heart failure. It follows pericardial injury (post-surgical, tuberculous, radiation, or idiopathic), is distinguished from restrictive cardiomyopathy by hemodynamic and imaging features, and definitive treatment is pericardiectomy.
- Heart failure
Heart failure is a clinical syndrome of impaired cardiac filling or ejection causing congestion and reduced perfusion, classified by ejection fraction. Diagnosis integrates symptoms, signs, natriuretic peptides, and echocardiography, and treatment of reduced-ejection-fraction disease is quadruple guideline-directed therapy, with device therapy and cause-specific management.
- Pericardial effusion
Pericardial effusion is accumulation of fluid in the pericardial space, ranging from an incidental finding to life-threatening tamponade. Its hemodynamic effect depends on the rate of accumulation rather than volume alone, and management ranges from observation and treating the cause to pericardiocentesis for tamponade or large symptomatic effusions.