Late assessment after biventricular repair for isomerism heart

Although biventricular repair is theoretically ideal for the treatment of isomerism heart, the long-term outcome is unknown. We assessed the outcome of biventricular repair for atrial isomerism.

From 1984 to 2002 in our surgical database, 10 of 67 patients with atrial isomerism received biventricular repair. The age at operation was 7.7 +/- 9.1 years. Preoperative ventricular volume was normal in all patients. Left ventricular ejection fraction was 62% +/- 8%. Intra-atrial rerouting was required in 8. The atrioventricular septal defect with double-outlet right ventricle was closed using comma-shaped intraventricular conduit in 8. Other procedures included pulmonary valvotomy and infundibulectomy in 2, transannular patch in 1 and right ventricle to pulmonary artery conduit in 1.

There was one early death due to hemolytic phagocytic syndrome. The other 9 patients are in New York Heart Association class I at 12.7 +/- 5.3 years postoperatively. There was no atrial baffle stenosis except in 1 patient; it was successfully treated by stent placement 10 years after the operation. There was no incidence of left ventricular outflow obstruction. Mitral replacement (4 months postoperatively) or repair (15 years postoperatively) was done in 2 patients. The other 5 patients with atrioventricular septal defect showed trivial to mild regurgitation in the long-term period. Arrhythmia was observed in 5 (left 4, right 1). Catheter ablation was needed in a patient with atrial flutter. Three of 9 patients require diuretics or digitalization, or both.

The long-term outcome of the biventricular repair for atrial isomerism was excellent. Late development of mitral regurgitation and arrhythmia could be managed adequately.