title: Pulmonary atresia with intact ventricular septum: strategy based on right ventricular morphology.
abstract: OBJECTIVE
  Management strategy for pulmonary atresia with intact ventricular septum is controversial. We treated patients with this anomaly according to a management protocol based on our quantitative assessment of right ventricular morphology (index of right ventricular development and right ventricle-tricuspid valve index). In the present study, we reviewed a 20-year experience of the surgical treatment at our institution to define guidelines for selecting the appropriate type of surgical procedure.
  
  
  METHODS
  Between April 1981 and June 2002, 45 consecutive patients with pulmonary atresia with intact ventricular septum underwent surgical treatment. Open transpulmonary valvotomy was performed in 27 patients and Blalock-Taussig shunt in 18 patients as the initial palliative procedure. Three patients who underwent a successful transpulmonary valvotomy alone in the neonatal period required no further operation. Definitive repair was performed in 32 patients. Biventricular repair was performed on 19 patients, one and a half ventricular repair in 3, and Fontan-type operation in 10.
  
  
  RESULTS
  There were 1 early and 2 late deaths before the definitive operation in patients who underwent transpulmonary valvotomy. Two patients who had coronary artery interruption died 3 months and 13 years after the initial Blalock-Taussig shunt. There were 1 early and 2 late deaths after the definitive operation. Actuarial survival, including noncardiac death, was 91.1% at 5 years after the initial procedure and 81.5% at 10 years.
  
  
  CONCLUSIONS
  We treated patients with pulmonary atresia with intact ventricular septum according to a management protocol based on our quantitative assessment of right ventricular morphology with good results.
authors.affiliation: Department of Cardiothoracic Surgery, Kobe Children's Hospital, Japan. y-naoki@za2.so-net.ne.jp
authors.forename: Naoki
authors.initials: N
authors.lastname: Yoshimura
authors.forename: Masahiro
authors.initials: M
authors.lastname: Yamaguchi
authors.forename: Hidetaka
authors.initials: H
authors.lastname: Ohashi
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Oshima
authors.forename: Shigeteru
authors.initials: S
authors.lastname: Oka
authors.forename: Masahiro
authors.initials: M
authors.lastname: Yoshida
authors.forename: Hirohisa
authors.initials: H
authors.lastname: Murakami
authors.forename: Teruo
authors.initials: T
authors.lastname: Tei
mesh_terms: D006328:Cardiac Catheterization / Q000379:methods*; D006348:Cardiac Surgical Procedures / Q000379:methods* / Q000401:mortality; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000379:methods / Q000401:mortality; D006346:Heart Septum / Q000033:anatomy & histology*; D006352:Heart Ventricles / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care / Q000379:methods; D018633:Pulmonary Atresia / Q000175:diagnosis* / Q000601:surgery*; D012016:Reference Values; D012086:Reoperation; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome