title: Outcome of patients with double-inlet left ventricle or tricuspid atresia with transposed great arteries.
abstract: OBJECTIVE
  We sought to determine the long-term outcomes and risk factors for mortality in patients with double-inlet left ventricle (DILV) or tricuspid atresia with transposed great arteries (TA-TGA).
  
  
  BACKGROUND
  Patients with DILV or TA-TGA are at risk of systemic outflow obstruction and a poor outcome. The impact of various management strategies on the long-term outcomes of these patients remains unknown.
  
  
  METHODS
  We reviewed the outcomes of 164 consecutive pediatric patients with DILV or TA-TGA who underwent surgical palliation between 1983 and 2002. Patients with a Holmes heart or heterotaxy syndrome or who were lost to follow-up (n = 24) were excluded. Risk factors for mortality or the need for orthotopic heart transplantation (OHT) were assessed by multivariate analysis.
  
  
  RESULTS
  There were 105 patients with DILV and 35 patients with TA-TGA. The overall mortality rate, including OHT, was 29%. Patients with DILV had a lower mortality rate than patients with TA-TGA (23% vs. 49%, p = 0.007). Multivariate analysis showed the presence of arrhythmia and pacemaker requirement as independent risk factors for mortality, whereas pulmonary atresia or stenosis and pulmonary artery banding were associated with decreased mortality. Gender, era of birth, aortic arch anomaly, and systemic outflow obstruction were not risk factors. The perioperative and overall mortality were similar between patients who underwent the Damus-Kaye-Stansel procedure beyond the neonatal period and those had subaortic resection.
  
  
  CONCLUSIONS
  The mortality of patients with DILV or TA-TGA remains high. The outcomes of these patients are influenced by restriction of pulmonary blood flow, arrhythmia, and pacemaker requirement. Surgical palliation to relieve systemic outflow obstruction is not associated with a poor outcome.
authors.affiliation: Division of Cardiology, Department of Pediatrics, Los Angeles, California, USA.
authors.forename: Yueh-Tze
authors.initials: YT
authors.lastname: Lan
authors.forename: Ruey-Kang
authors.initials: RK
authors.lastname: Chang
authors.forename: Hillel
authors.initials: H
authors.lastname: Laks
mesh_terms: D000015:Abnormalities, Multiple / Q000401:mortality / Q000601:surgery*; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006352:Heart Ventricles / Q000002:abnormalities* / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D011183:Postoperative Complications / Q000453:epidemiology / Q000628:therapy; D015996:Survival Rate; D013997:Time Factors; D014188:Transposition of Great Vessels / Q000150:complications / Q000401:mortality / Q000601:surgery*; D016896:Treatment Outcome; D018785:Tricuspid Atresia / Q000150:complications / Q000401:mortality / Q000601:surgery*