Restrictive cardiomyopathy (RCM) is the least common among cardiomyopathies, it can be idiopathic, familial (autosomal or X-linked, dominant or recessive) or secondary to systematic disorders. Patients commonly present with symptoms and signs of congestive heart failure (HF). Electrocardiographic findings are non-specific and include supraventricular and ventricular arrhythmias, atrioventricular conduction and QRS complex abnormalities. Echocardiography and cardiac magnetic resonance (CMR) play a major role in diagnosis, while cardiac computed tomography (CT) and nuclear imaging assist in specific conditions. Echocardiography reveals normal-sized or hypertrophied ventricles, atrial enlargement and preserved systolic and impaired diastolic function, often with restrictive filling pattern. CMR with a higher spatial resolution than echocardiography provides detailed information about anatomy, perfusion, ventricular function and tissue characterization. Also CMR with late gadolinium enhancement (LGE) and myocardial mapping can direct the diagnosis to specific subtypes of RCM, depending on the pattern of scar formation. Endomyocardial biopsy may be required if non-invasive studies fail to pose diagnosis. Differentiation between RCM and constrictive pericarditis (CP), nowadays by echocardiophy, is important since both present as HF with preserved ejection fraction but CP can be treated, while the treatment options of RCM depend on the underlying condition. Prognosis is generally poor despite optimal medical treatment.

Author(s) Details

Aidonis Rammos,
2nd Department of Cardiology, University Hospital of Ioannina, Greece.

Aris Bechlioulis
2nd Department of Cardiology, University Hospital of Ioannina, Greece.

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