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Alain Serraf
Stefano Conte
François Lacour-Gayet
Claude Planché
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Ann Thorac Surg 1995;60:970-977
© 1995 The Society of Thoracic Surgeons

Articles

Systemic obstruction in univentricular hearts: Surgical options for neonates

MD Alain Serraf*, MD Stefano Conte, MD François Lacour-Gayet, MD Jacqueline Bruniaux, MD Miguel Sousa-Uva, MD Régine Roussin, MD Claude Planché

Department of Pediatric Cardiac Surgery, Marie Lannelongue Hospital, Le Plessis-Robinson, France

* Address reprint requests to Dr Serraf, Department of Pediatric Cardiac Surgery, Marie Lannelongue Hospital, 133 avenue de la Résistance, 92350 Le Plessis-Robinson, France.

Background.: The surgical management for bridging patients with univentricular heart and systemic obstruction to a Fontan procedure remains controversial.

Methods.: Twenty-seven of 96 patients with univentricular heart and unobstructed pulmonary blood flow referred for surgical palliation were seen with systemic obstruction. Twenty-six were neonates with coarctation of the aorta in 21 and subaortic stenosis in 5. In 8 other patients, subaortic stenosis developed after initial pulmonary artery banding. Four different palliative procedures were performed: coarctation repair with pulmonary artery banding (group I, n = 15); Norwood or Damus-Kaye-Stansel or arterial switch operation (group II, n = 9); coarctation repair with pulmonary artery banding and bulboventricular foramen enlargement (group III, n = 2); and orthotopic heart transplantation with coarctation repair (group IV, n = 1).

Results.: The mortality 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 patients (8 in group I and 1 in group II) had development of subaortic stenosis and underwent a subsequent procedure: Damus-Kaye-Stansel operation in 5, arterial switch operation in 3, and bulboventricular foramen enlargement in 1. Three had a concomitant or subsequent Fontan procedure and 2, a bidirectional Glenn procedure. In group II, 1 patient underwent a subsequent Fontan procedure and another, a bidirectional Glenn anastomosis. Six of the 8 patients with subaortic stenosis after initial pulmonary artery banding underwent a second stage consisting of a Damus-Kaye-Stansel procedure (n = 3), bulboventricular foramen enlargement (n = 2), or creation of an aortopulmonary window (n = 1). Three had a concomitant Fontan procedure and 2, a bidirectional Glenn procedure. Actuarial 4-year survival was 65.5% ± 8.4% (70% confidence limits) 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 patients 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.




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