title: Primary repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a useful approach.
abstract: BACKGROUND
  The ultimate goal of surgical therapy for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is to create unobstructed and separate in series pulmonary and systemic circuits. Our preference has been a 1-stage complete unifocalization technique, avoiding collateral anastomosis with either the native pulmonary arteries or other aortopulmonary collateral vessels.
  
  
  RESULTS
  Since 1998, 5 patients (median age 29.6 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have undergone surgical correction, consisting of (1) exclusion of a descending thoracic aortic segment from which all major aortopulmonary collateral arteries originate, and (2) connection of this aortic segment to the native pulmonary artery using an interposition polytetrafluoroethylene conduit. The ventricular septal defect was closed in all patients, and the right ventricle was connected to the unifocalized pulmonary artery with a valved conduit. All patients survived the operation. Two patients required reexploration for postoperative bleeding. One patient remained on mechanical ventilation for 17 days due to a pulmonary infection. During follow-up (12-21 months), no patient required additional interventions. The postoperative right ventricular/left ventricular pressure ratio was 0.55 median. No significant stenosis within the reconstructed pulmonary circuit was identified. All patients remain free of symptoms, requiring no medications.
  
  
  CONCLUSIONS
  Intracardiac repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries can be accomplished by a midline 1-stage repair including complete unifocalization of all pulmonary blood supply without individual collateral anastomosis in selected patients. This approach offers a convenient and satisfactory surgical option.
authors.affiliation: Division of Cardiovascular Surgery, Hospital San Donato, San Donato Milanese, Italy. r_abella@hotmail.com
authors.forename: Raul F
authors.initials: RF
authors.lastname: Abella
authors.forename: Teresa
authors.initials: T
authors.lastname: De La Torre
authors.forename: Gerardo
authors.initials: G
authors.lastname: Mastropietro
authors.forename: Nuccia
authors.initials: N
authors.lastname: Morici
authors.forename: Adriano
authors.initials: A
authors.lastname: Cipriani
authors.forename: Carlo
authors.initials: C
authors.lastname: Marcelletti
mesh_terms: D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000601:surgery*; D000714:Anastomosis, Surgical; D000792:Angiography; D002315:Cardiopulmonary Bypass / Q000379:methods*; D002675:Child, Preschool; D003097:Collateral Circulation; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000601:surgery; D006334:Heart Function Tests; D006345:Heart Septal Defects, Ventricular / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D018633:Pulmonary Atresia / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D012189:Retrospective Studies; D018570:Risk Assessment; D012494:Sampling Studies; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000379:methods*