Restrictive cardiomyopathy (RCM) is the least common among cardiomyopathies. It can be idiopathic, familial, or secondary\nto systematic disorders. Marked increase in left and/or right ventricular filling pressures causes symptoms and signs of\ncongestive heart failure. Electrocardiographic findings are nonspecific and include atrioventricular conduction and QRS complex\nabnormalities and supraventricular and ventricular arrhythmias. Echocardiography and cardiac magnetic resonance (CMR) play\na major role in diagnosis. Echocardiography reveals normal or hypertrophied ventricles, preserved systolic function, marked\nbiatrial enlargement, and impaired diastolic function, often with restrictive filling pattern. CMR offering a higher spatial\nresolution than echocardiography can provide detailed information about anatomic structures, perfusion, ventricular function,\nand tissue characterization. CMR with late gadolinium enhancement (LGE) and novel approaches (myocardial mapping) can\ndirect the diagnosis to specific subtypes of RCM, depending on the pattern of scar formation. When noninvasive studies\nhave failed, endomyocardial biopsy is required. Differentiation between RCM and constrictive pericarditis (CP), nowadays by\nechocardiography, is important since both present as heart failure with normal-sized ventricles and preserved ejection fraction\nbut CP can be treated by means of anti-inflammatory and surgical treatment, while the treatment options of RCM are dictated by\nthe underlying condition. Prognosis is generally poor despite optimal medical treatment.
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