title: [Individualized surgical management of pulmonary atresia with intact ventricular septum].
abstract: OBJECTIVE
  To delineate individualized surgical management strategy for the optimal management of pulmonary atresia with intact ventricular septum (PA/IVS).
  
  
  METHODS
  Between 1997 to 2005, 52 consecutive patients with PA/IVS, 31 male and 21 female, aged 2-9, divided into 2 groups according if right ventricle-dependent coronary circulation (RVDCC) existed, underwent individualized surgical treatment. The patients in Group I (with: RVDCC, n = 6) underwent single ventricle repair. Forty-six patients were in Group II (without RVDCC), 13 of which with severe right ventricular hypoplasia (tricuspid valve Z value < -4)underwent single ventricle repair and 33 of which were with mild to moderate right ventricular hypoplasia (Z value > -4). One critical neonate underwent systemic- pulmonary artery shunt. The other 32 of the 33 patients received right ventricular decompression procedures firstly, and 6 of them presented optimal hemodynamics and oxygenation and achieved biventricular repair. If the patients presented unstable hemodynamics or hypoxemia after right ventricular decompression procedure, an additional shunt was added. 13 young infants (0-2 months) underwent additional systemic-pulmonary artery shunt, and 13 patients (> 3 months-old) underwent additional bidirectional cavopulmonary shunt (1.5 ventricular repair)The patients were followed up for 1-10 years.
  
  
  RESULTS
  There were 1 early death in Group I and 7 early deaths in Group II. The total early mortality was 15%. A successful definitive repair was achieved in 26 cases (50%). Follow-up reported 3 late deaths.
  
  
  CONCLUSIONS
  Individualized surgical management based on the presence of RVDCC, right ventricular hypoplasia grading, right ventricular growth potential, hemodynamic situation, and oxygen saturation after the decompression procedure is helpful to improve the surgical results of PA/IVS
authors.affiliation: Department of Pediatric Thoracic & Cardiovascular Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China. zzqheart@yahoo.com.cn
authors.forename: Zhong-Qun
authors.initials: ZQ
authors.lastname: Zhu
authors.forename: Jin-Fen
authors.initials: JF
authors.lastname: Liu
authors.forename: Zhao-Kang
authors.initials: ZK
authors.lastname: Su
authors.forename: Zhi-Wei
authors.initials: ZW
authors.lastname: Xu
authors.forename: Ding-Fang
authors.initials: DF
authors.lastname: Cao
authors.forename: Wen-Xiang
authors.initials: WX
authors.lastname: Ding
authors.forename: Yan-jun
authors.initials: YJ
authors.lastname: Sun
mesh_terms: D013504:Cardiovascular Surgical Procedures / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D003326:Coronary Circulation; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D018633:Pulmonary Atresia / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D016896:Treatment Outcome; D020257:Ventricular Remodeling; D054088:Ventricular Septum