Biventricular Repair for Right Atrial Isomerism

Background Biventricular repair is often difficult to accomplish in patients with right atrial isomerism because of complex anomalous structures. Methods Ten patients with right atrial isomerism underwent biventricular repair. Their ages ranged from 15 months to 21 years. The follow-up period ranged from 1 month to 21 years. The Fontan procedure was unsuitable in 7 patients. The atrioventricular valves were separated in 5 patients and common in 5. One patient who had severe right-sided atrioventricular valvular regurgitation required concomitant prosthetic valve replacement. Another patient with a hypoplastic intraventricular septum underwent ventricular septation. Nine patients had two balanced ventricles. A ventricular septal defect was enlarged for rerouting in 3 patients. All patients had anomalous venoatrial connections and required intra-atrial baffle rerouting. One with major aortopulmonary collateral arteries underwent staged unifocalization. Three had extracardiac conduit repair. The outcomes were compared with 97 patients who underwent the Fontan procedure. Results There were 3 early deaths and 1 late death. Six survivors are in New York Heart Association functional class I or II. Two are free from medications. Two required reoperation owing to infection or prosthetic valve failure. At 1 year, cardiac index and systemic venous pressure were 3.2 ± 0.9 L • min−1 • m−2 and 6.6 ± 1.6 mm Hg, respectively. There were no significant differences in survival, freedom from arrhythmia, freedom from reoperation, or exercise tolerance between biventricular repair and the Fontan procedure. Conclusions Biventricular repair provided good long-term outcomes in patients with right atrial isomerism. In selected circumstances, biventricular repair is an acceptable alternative to the Fontan procedure.