Restrictive cardiomyopathy: Clinical assessment and imaging in diagnosis and patient management

Abstract

The main difference between restrictive cardiomyopathy (RCM) and the other cardiomyopathies (CMP) is that its diagnosis and definition depend on functional rather than morphologic criteria. All causes of diastolic dysfunction are included in the differential diagnosis of RCM, in particular, infiltrative/storage CMP and constrictive pericarditis (CP). The recognition of CP is clinically important, as it is potentially curable with surgery, whereas RCM has no specific therapy. Rare forms characterized by endomyocardial involvement with severe fibrosis, with or without eosinophilic infiltration, are included within the spectrum of RCM disease. It must be noted that, although RCM is the least common CMP and extremely rarely encountered in clinical practice, its diagnosis must be an exclusion diagnosis, and it is important to exclude all the other causes of restrictive filling in order to manage these patients with the most appropriate and possibly specific therapy.