title: Outcomes of surgery for simple total anomalous pulmonary venous drainage in neonates.
abstract: BACKGROUND
  Repair of total anomalous pulmonary venous drainage (TAPVD) in neonates remains a challenge as it is often associated with severe obstruction. We describe a large cohort of neonates who underwent TAPVD repair at a single institution.
  
  
  METHODS
  From 1973 to 2008, 112 patients underwent simple TAPVD surgery during the first month of life. Data collection occurred retrospectively.
  
  
  RESULTS
  Preoperative pulmonary venous obstruction (PVO) occurred in 89 (79.5%) patients. There were 12 (10.7%) early deaths. Significant risk factors were bypass time greater than 65 minutes (p=0.014) and emergent surgery (p=0.005). Hospital mortality was unchanged throughout the 3 eras (1973 to 1988, 1989 to 1998, 1999 to 2008), despite an increase in patients with preoperative acidosis (p=0.004) and severe TAPVD obstruction (p=0.038) during the recent 10 years. There were 6 (6.25%) late deaths within 2 years of repair. Survival at 20 years was 83.4% (95% confidence interval 75 to 89). Risk factors for late death were operative weight 2.5 kg or less (p=0.004) and postoperative pulmonary hypertensive crisis (p=0.02). Reoperation for recurrent PVO was required in 13 patients (11.9%). Risk factors were operative weight 2.5 kg or less (p=0.035) and postoperative pulmonary hypertensive crisis (p=0.002). Follow-up was 96% complete and survivors (n=90) were asymptomatic at a median age of 11.7 years.
  
  
  CONCLUSIONS
  Hospital mortality remained unchanged over the 36-year period. Survival beyond 2 years offers excellent outcome. Risk factors for mortality were the preoperative clinical status, prolonged bypass time, persisting micro-obstruction, and low operative weight. A reduction in mortality will likely require development of effective medical management for patients who have peripheral PVO not amenable to surgical repair.
authors: Cardiac Surgery and Cardiology, Royal Children's Hospital, University of Melbourne, Melbourne, Australia.
authors: Matthew S
authors: MS
authors: Yong
authors: Yves
authors: Y
authors: d'Udekem
authors: Terry
authors: T
authors: Robertson
authors: Stephen
authors: S
authors: Horton
authors: Mithilesh
authors: M
authors: Dronavalli
authors: Christian
authors: C
authors: Brizard
authors: Robert
authors: R
authors: Weintraub
authors: Frank
authors: F
authors: Shann
authors: Michael
authors: M
authors: Cheung
authors: Igor E
authors: IE
authors: Konstantinov
mesh_terms: D004322:Drainage; D005260:Female; D017052:Hospital Mortality; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000601:surgery; D011668:Pulmonary Veno-Occlusive Disease / Q000601:surgery; D016896:Treatment Outcome