title: Partial biventricular repair of pulmonary atresia with intact ventricular septum. Use of an adjustable atrial septal defect.
abstract: BACKGROUND
  Repair of pulmonary atresia with intact ventricular septum (PA-IVS) is one of the more challenging problems in congenital heart surgery. The hypertrophied but hypoplastic right ventricle is suddenly presented with an increased volume load as the right-to-left shunt is abolished by closure of the atrial septal defect (ASD) as part of definitive repair. We have proposed that a restrictive, adjustable ASD that allows a controlled right-to-left shunt may prevent excessive venous hypertension and allow adequate left-sided filling after biventricular repair of PA-IVS.
  
  
  RESULTS
  From 1982 through 1991, 39 patients presented for definitive repair of PA-IVS. Nineteen of these patients were thought to be suitable candidates for biventricular repair, and the other 20 underwent a modified Fontan procedure. An adjustable ASD was used in 12 of the 19 patients undergoing biventricular repair. Mean age was 24 months (age range, 5-66 months). Two patients had their ASD snared closed in the operating room after coming off bypass. In the other 10 patients, closure of the ASD could not be tolerated at the time of surgery as evidenced by elevation of right atrial pressures by > 15 mm Hg and decreased blood pressure when closure was attempted. Seven patients had their open ASDs closed by the snare device, and one patient had his ASD narrowed in the early postoperative period. One patient underwent delayed closure 16 months after surgery. There was one hospital death.
  
  
  CONCLUSIONS
  The snare-controlled adjustable ASD allows partial biventricular repair of PA-IVS, reducing the risk of low cardiac output and severe venous hypertension in the postoperative period.
authors.affiliation: Division of Cardiothoracic Surgery, University of California, Los Angeles Medical Center 90024.
authors.forename: H
authors.initials: H
authors.lastname: Laks
authors.forename: J M
authors.initials: JM
authors.lastname: Pearl
authors.forename: D C
authors.initials: DC
authors.lastname: Drinkwater
authors.forename: J
authors.initials: J
authors.lastname: Jarmakani
authors.forename: J
authors.initials: J
authors.lastname: Isabel-Jones
authors.forename: B L
authors.initials: BL
authors.lastname: George
authors.forename: R G
authors.initials: RG
authors.lastname: Williams
mesh_terms: D001021:Aortic Valve / Q000637:transplantation; D001705:Bioprosthesis; D002303:Cardiac Output, Low / Q000517:prevention & control; D002675:Child, Preschool; D006344:Heart Septal Defects, Atrial / Q000601:surgery*; D006350:Heart Valve Prosthesis; D006801:Humans; D006973:Hypertension / Q000517:prevention & control; D010496:Pericardium / Q000637:transplantation; D011138:Polytetrafluoroethylene; D011183:Postoperative Complications / Q000517:prevention & control; D019736:Prostheses and Implants; D011664:Pulmonary Valve / Q000002:abnormalities*; D013536:Suture Techniques; D016896:Treatment Outcome
chemical_list: D011138:Polytetrafluoroethylene