title: Treatment of pulmonary atresia with intact ventricular septum in early infancy.
abstract: BACKGROUND
  Early treatment for the neonate with pulmonary atresia and intact ventricular septum (PA-IVS) is important if a high mortality rate is to be avoided. The treatment includes prostaglandin administration, balloon atrial septostomy, pulmonary valvotomy, a shunting procedure and patch repair for the right ventricular outflow tract (RVOT). This study discusses the early treatment and risk factors of this disease that are essential for assessing the risk of surgery and predicting outcome.
  
  
  METHODS
  The medical records, echocardiograms, catheterization data and cineangiograms of 29 patients with PA-IVS diagnosed at our institution from 1987 to 1997 were reviewed retrospectively. Clinical manifestations including age, body weight, sex ratio, type of surgery and outcome were analyzed.
  
  
  RESULTS
  Three of four patients with a right ventricular volume of less than 1 ml died, and all patients with a right ventricular volume of greater than 2 ml survived. Four of six patients with a tricuspid valvular area of between 0.25 and 0.5 cm2 died, but patients with an area of greater than 0.5 cm2 survived. One case with a monopartite right ventricle died. Patients with a bipartite or tripartite right ventricle had higher survival rates than those with a monopartite right ventricle. Patients with a the right ventricle dependent coronary sinusoid had a higher risk for mortality, even after surgery, than those with normal coronary circulation.
  
  
  CONCLUSIONS
  A small right ventricular volume, the presence of a coronary sinusoid involving the right ventricle, a short linear length of the tricuspid valve and a small area of the tricuspid valve were the risk factors associated with a negative outcome in neonates with PA-IVS.
authors.affiliation: Department of Pediatrics, Veterans General Hospital-Taipei, Taiwan, ROC.
authors.forename: W H
authors.initials: WH
authors.lastname: Hsieh
authors.forename: B
authors.initials: B
authors.lastname: Hwang
authors.forename: J H
authors.initials: JH
authors.lastname: Lu
authors.forename: P C
authors.initials: PC
authors.lastname: Lee
authors.forename: Y C
authors.initials: YC
authors.lastname: Fu
authors.forename: H P
authors.initials: HP
authors.lastname: Shing
authors.forename: C C
authors.initials: CC
authors.lastname: Meng
mesh_terms: D005260:Female; D006346:Heart Septum; D006352:Heart Ventricles; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D018633:Pulmonary Atresia / Q000601:surgery*; D012189:Retrospective Studies