title: Systemic obstruction in univentricular hearts: surgical options for neonates.
abstract: 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.
authors.affiliation: Department of Pediatric Cardiac Surgery, Marie Lannelongue Hospital, Le Plessis-Robinson, France.
authors.forename: A
authors.initials: A
authors.lastname: Serraf
authors.forename: S
authors.initials: S
authors.lastname: Conte
authors.forename: F
authors.initials: F
authors.lastname: Lacour-Gayet
authors.forename: J
authors.initials: J
authors.lastname: Bruniaux
authors.forename: M
authors.initials: M
authors.lastname: Sousa-Uva
authors.forename: R
authors.initials: R
authors.lastname: Roussin
authors.forename: C
authors.initials: C
authors.lastname: Planché
mesh_terms: D001017:Aortic Coarctation / Q000150:complications / Q000401:mortality / Q000601:surgery*; D001020:Aortic Stenosis, Subvalvular / Q000150:complications / Q000401:mortality / Q000601:surgery*; D006348:Cardiac Surgical Procedures / Q000379:methods; D018729:Fontan Procedure; D005602:France / Q000453:epidemiology; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans; D007231:Infant, Newborn; D010166:Palliative Care / Q000379:methods*; D015996:Survival Rate; D016896:Treatment Outcome