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(Circulation. 1998;98:1943-1945.)
© 1998 American Heart Association, Inc.

Images in Cardiovascular Medicine

Right Ventricular Dysplasia With Biventricular Involvement

Bruno Pinamonti, MD; Lorenzo Pagnan, MD; Rossana Bussani, MD; Claudio Ricci, MD; Furio Silvestri, MD; ; Fulvio Camerini, MD

From the Departments of Cardiology (B.P., F.C.), Radiology (L.P., C.R.), and Pathology (R.B., F.S.), Ospedale Maggiore and University, Trieste, Italy.

Correspondence to Bruno Pinamonti, MD, Divisione di Cardiologia, Ospedale Maggiore, 34129 Trieste, Italy.

A23-year-old man was admitted to the hospital with severe heart failure and cachexia. Ventricular arrhythmias and progressive heart failure (predominantly right heart) had been observed in the previous 3 years. Physical examination was unremarkable except for a widely split second heart sound, a systolic left precordial lift, third and fourth heart sounds, and signs of increased venous pressure. Chest radiography showed significant cardiomegaly. The ECG was characterized by right atrial enlargement, low QRS voltages, wide complexes in the right precordial leads (epsilon waves?), and negative T waves. Nonsustained ventricular tachycardia with polymorphic configuration was observed at ambulatory ECG monitoring.

The echocardiogram (Figure 1) disclosed severe right ventricular enlargement, with aneurysm at the level of the outflow, severe depression of systolic pump function, and multiple wall bulges. The left ventricle was not dilated but showed severe diffuse hypokinesis and depression of the ejection fraction (20%).

View larger version (103K): [in this window] [in a new window]   Figure 1. Top, Two-dimensional echocardiography. Left, Parasternal short-axis view at basal level. Right, Modified apical 4-chamber view. Both frames in systole. Severe right ventricular (RV) enlargement with aneurysmal dilatation of outflow tract (RVOT) and multiple wall bulges (right) are present. PA indicates pulmonary artery; RA, right atrium; LV, left ventricle; and LA, left atrium. Bottom, M-mode echocardiographic tracing at ventricular level. RV is severely enlarged; LV is not enlarged, but severe hypokinesis of both septum (IVS) and posterior wall (PW) is evident.

Considering the severity of clinical symptoms refractory to medical treatment and the echocardiographic findings, a cardiac transplantation was scheduled; unfortunately, the young patient . . . [Full Text of this Article]

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