SYSTEMIC OBSTRUCTION IN UNIVENTRICULAR HEARTS - SURGICAL OPTIONS FOR NEONATES

Background. The surgical management for bridging patients with univent ricular heart and systemic obstruction to a Fontan procedure remains c ontroversial. Methods. Twenty-seven of 96 patients with univentricular heart and unobstructed pulmonary blood now referred for surgical pall iation were seen with systemic obstruction. Twenty-six were neonates w ith coarctation of the aorta in 21 and subaortic stenosis in 5. In 8 o ther patients, subaortic stenosis developed after initial pulmonary ar tery banding. Four different palliative procedures were performed: coa rctation repair with pulmonary artery banding (group I, n = 15); Norwo od or Damus-Kaye-Stansel or arterial switch operation (group II, n = 9 ); coarctation repair with pulmonary artery banding and bulboventricul ar foramen enlargement (group III, n = 2); and orthotopic heart transp lantation with coarctation repair (group IV, n = 1). Results. The mort ality rate was 34.3% (n = 12) for all patients, 53.3% in group I, 33.3 % in group II (p = 0.003 versus group I), and 50% in group III. Nine p atients (8 in group I and 1 in group II) had development of subaortic stenosis and underwent a subsequent procedure: Damus-Kaye-Stansel oper ation in 5, arterial switch operation in 3, and bulboventricular foram en enlargement in 1. Three had a concomitant or subsequent Fontan proc edure and 2, a bidirectional Glenn procedure. In group II, 1 patient u nderwent a subsequent Fontan procedure and another, a bidirectional Gl enn anastomosis. Six of the 8 patients with subaortic stenosis after i nitial pulmonary artery banding underwent a second stage consisting of a Damus-Kaye-Stansel procedure (n = 3), bulboventricular foramen enla rgement (n = 2), or creation of an aortopulmonary window (n = 1). Thre e had a concomitant Fontan procedure and 2, a bidirectional Glenn proc edure. Actuarial 4-year survival was 65.5% +/- 8.4% (70% confidence li mits) for all patients; it was 40% +/- 13.3% in group I and 66.6% +/- 16.3% in group II (p < 0.05). Conclusions. Initial management of patie nts with univentricular heart and systemic obstruction by Norwood-like procedures provides a better outcome. Success of the Fontan operation relies on the ability to provide timely relief of subaortic stenosis.