title: Surgical management of pulmonary atresia with ventricular septal defect in late adolescence and adulthood.
abstract: OBJECTIVE
  In presence of adequate pulmonary blood flow, patients presenting with unoperated or palliated pulmonary atresia with ventricular septal defect (PA/VSD) can reach adult age. However, they remain symptomatic with a limited life expectancy.
  
  
  METHODS
  Since 1993, 27 patients underwent surgery for unrepaired PA/VSD. Median age was 20 (range: 15-43) years. Nineteen patients had 33 previous palliative procedures while eight were unoperated survivors. Major aortopulmonary collateral artery (MAPCA) had been observed in all but 2 and were still patent in 23. All bronchopulmonary segments were connected to the native pulmonary arteries (NPA) in 4 (type A), to both NPA and MAPCA in 18 (type B) and only to MAPCA in 5 (type C). The biventricular repair was performed in 17 patients: 3 type A, 12 type B and 2 type C. Ten patients underwent palliative procedure: eight aortopulmonary shunt, with unifocalisation in two and one right ventricle to NPA restrictive conduit.
  
  
  RESULTS
  One (4%) hospital death occurred following the failure of a palliative procedure. No clinical improvement was observed in seven patients including one repaired and six palliated survivors. Two late cardiac death occurred 1 and 7 years after repair. At last visit, 15 of 16 repaired survivors were in NYHA class I or II. Only one patient awaits septation, while eight other with subsequent palliation were considered not repairable.
  
  
  CONCLUSIONS
  The outcome was encouraging in patients who were eligible for completed biventricular repair. Although considered as unique alternative to cardiopulmonary transplantation, the justification for palliative surgery to improve pulmonary blood flow remains to be established.
authors.affiliation: Marie Lannelongue Hospital, Paris-Sud University, Le Plessis-robinson, France. ebelli@ccml.com
authors.forename: Emre
authors.initials: E
authors.lastname: Belli
authors.forename: Loïc
authors.initials: L
authors.lastname: Macé
authors.forename: Mohammed
authors.initials: M
authors.lastname: Ly
authors.forename: Patrice
authors.initials: P
authors.lastname: Dervanian
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Pineau
authors.forename: Régine
authors.initials: R
authors.lastname: Roussin
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Lebret
authors.forename: Alain
authors.initials: A
authors.lastname: Serraf
mesh_terms: D000293:Adolescent; D000328:Adult; D001028:Aortopulmonary Septal Defect / Q000000981:diagnostic imaging / Q000601:surgery; D006345:Heart Septal Defects, Ventricular / Q000000981:diagnostic imaging / Q000601:surgery*; D006801:Humans; D010166:Palliative Care / Q000379:methods; D018633:Pulmonary Atresia / Q000000981:diagnostic imaging / Q000601:surgery*; D012086:Reoperation; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome