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Biventricular diverticula in a patient with restrictive cardiomyopathy

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The simultaneous occurrence of both right and left ventricular diverticula is extremely rare and usually associated with midline thoracic congenital defects. The association of biventricular diverticula with restrictive cardiomyopathy, however, has not been described so far.

A 16 year old male underwent evaluation for heart transplantation because of progressive dyspnoea, dizziness, and palpitations caused by restrictive cardiomyopathy of unknown aetiology which was unresponsive to conventional medical treatment. Idiopathic restrictive cardiomyopathy had been diagnosed at the age of less than 1 year and was associated with growth retardation, but no other overt congenital defects. During childhood and early adolescence the boy remained limited in his physical performance compared to his schoolmates. Since the autumn of 1997 progressive deterioration with dyspnoea and symptomatic arrhythmia such as atrial re-entry tachycardia or atrial flutter occurred and made further attendance of school almost impossible. Medical treatment was ineffective and, after clinical reassessment, the patient was put on the waiting list for heart transplantation in early 1998.

Echocardiographic examination at this time revealed the presence of biventricular diverticula located beneath the subtricuspid lateral right ventricular wall and submitral lateral left ventricular wall, respectively (LV, left ventricle; RV, right ventricle, IVC, inferior vena cava). Restrictive cardiomyopathy with massive enlargement of both atria, an increase in left ventricular wall thickness, and a reduced ejection fraction of 40% was confirmed.

The patient underwent successful heart transplantation in the summer of 1999. Pathologic anatomic examination of the explanted heart confirmed the diagnosis of idiopathic restrictive cardiomyopathy, and also the presence of a right and left ventricular basal subanular diverticula (arrows).