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The number of adult patients with a systemic right ventricle (RV) is steadily increasing. Survival is relatively good in these patients, but deterioration of the systemic RV seems inevitable. Although therapeutic options for patients with LV failure are well established, their role in patients with systemic RV failure is often undefined. To appreciate the potency of LV failure therapy in patients with a systemic RV, insight into pathophysiology of systemic RV failure and into recent developments in therapeutic research are indispensible. This review provides these insights, and will facilitate and ameliorate therapeutic decision making in patients with a systemic RV.

The number of adult patients with a systemic right ventricle (RV) is steadily increasing. Survival is relatively good in these patients, but deterioration of the systemic RV seems inevitable. Although therapeutic options for patients with LV failure are well established, their role in patients with systemic RV failure is often undefined. To appreciate the potency of LV failure therapy in patients with a systemic RV, insight into pathophysiology of systemic RV failure and into recent developments in therapeutic research are indispensible. This review provides these insights, and will facilitate and ameliorate therapeutic decision making in patients with a systemic RV.

OBJECTIVES--A combined atrial and arterial switch procedure was performed in selected patients with congenitally corrected transposition to establish the morphological left ventricle as the systemic ventricle. Immediate and early follow up results are presented. BACKGROUND--Progressive right ventricular dysfunction and tricuspid regurgitation are common in patients with congenitally corrected transposition who undergo repair of associated lesions. A surgical procedure which re-establishes the left ventricle as the systemic ventricle should improve functional results. METHODS--Four symptomatic children aged from 9 months to 3 years 1 month (mean 2 years 3 months) with congenitally corrected transposition and ventricular septal defect underwent both an atrial and arterial switch procedure and were followed up for a mean of 12 months (range 6-21 months). RESULTS--There were no early or late deaths. Conduction abnormalities worsened in two patients. Hospital stay ranged from 8 to 17 days (mean 13 days). The cardiothoracic ratio decreased from a mean (range) of 0.65 (0.6 to 0.71) to 0.58 (0.52 to 0.6). Currently, three patients are in functional class I and one child is in functional class II. CONCLUSIONS--The combination of an atrial and an arterial switch procedure in symptomatic children with congenitally corrected transposition establishes the left ventricle as the systemic ventricle. The initial experience is encouraging with excellent immediate and early follow up results.

We report an 82-day-old infant with a rare combination of anomalies: pulmonary atresia associated with corrected transposition of the great arteries and intact ventricular septum; this is the second such recorded case. By using echocardiography, chest radiography, and electrocardiography the correct anatomical diagnosis was obtained before invasive investigation and necropsy.

A case is reported concerning echocardiographic findings in a patient with congenital corrected transposition of the great vessels and Ebstein's anomaly of the tricuspid valve. This presented an unusual opportunity to study atrioventricular valve closure in a patient in whom the Ebstein's malformation involved the systemic atrioventricular valve. The mitral to tricuspid valve closure interval was 68 milliseconds and represented significant delay in tricuspid valve closure. This closure interval is similar to closure intervals previously reported for patients with Ebstein's anomaly without ventricular inversion.