title: Improved results with selective management in pulmonary atresia with intact ventricular septum.
abstract: OBJECTIVE
  Late outcome of neonatal pulmonary atresia with intact ventricular septum remains poor in most reported series. We have followed a selective approach toward either single ventricle repair versus complete or partial biventricular repair based on the presence of right ventricle-dependent coronary circulation and growth of the right ventricle.
  
  
  METHODS
  A retrospective chart review was conducted of 47 patients who underwent surgery between January 1991 and September 1998.
  
  
  RESULTS
  Sixteen (34%) patients had a right ventricle-dependent coronary circulation, with a tricuspid valve Z-score of -3.0 +/- 0.66 versus -2.0 +/- 0.95 (P =.002) for those without a right ventricle-dependent coronary circulation. A systemic-pulmonary artery shunt only was performed in all patients with a right ventricle-dependent coronary circulation, with 1 death. Fourteen of 16 patients with a right ventricle-dependent coronary circulation underwent a bidirectional Glenn shunt at a median of 9 months after their first operation, 9 of whom have had a Fontan procedure (no deaths). In the 31 (66%) patients without a right ventricle-dependent coronary circulation, 6 patients underwent only a systemic-pulmonary artery shunt, 23 had a shunt and right ventricular decompression, and 2 had only a transannular patch. In this group, 10 patients received a 2-ventricle repair, 6 a 1. 5-ventricle repair, and 8 patients had a Fontan procedure. There was 1 early death and the overall survival was 98% at 1 year, 5 years, and 7 years.
  
  
  CONCLUSIONS
  If patients are stratified well, excellent survival can be achieved in the treatment of pulmonary atresia with intact ventricular septum. This result may be at the price of achieving a 1-ventricle as opposed to a 2-ventricle repair.
authors.affiliation: Department of Cardiac Surgery, Children's Hospital, Boston, MA 02115, USA.
authors.forename: M
authors.initials: M
authors.lastname: Jahangiri
authors.forename: D
authors.initials: D
authors.lastname: Zurakowski
authors.forename: D
authors.initials: D
authors.lastname: Bichell
authors.forename: J E
authors.initials: JE
authors.lastname: Mayer
authors.forename: P J
authors.initials: PJ
authors.lastname: del Nido
authors.forename: R A
authors.initials: RA
authors.lastname: Jonas
mesh_terms: D000206:Actuarial Analysis; D000714:Anastomosis, Surgical; D001166:Arteriovenous Shunt, Surgical; D019917:Blood Vessel Prosthesis Implantation; D003326:Coronary Circulation / Q000502:physiology; D005500:Follow-Up Studies; D018729:Fontan Procedure; D006346:Heart Septum / Q000473:pathology; D006352:Heart Ventricles / Q000002:abnormalities / Q000254:growth & development / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D011651:Pulmonary Artery / Q000601:surgery; D018633:Pulmonary Atresia / Q000601:surgery*; D012189:Retrospective Studies; D013348:Subclavian Artery / Q000601:surgery; D015996:Survival Rate; D016896:Treatment Outcome; D014261:Tricuspid Valve / Q000503:physiopathology / Q000601:surgery; D014683:Vena Cava, Superior / Q000601:surgery