title: Surgical outcomes of total anomalous pulmonary venous connection repair: a 22-year experience.
abstract: BACKGROUND
  Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease (CHD), whose surgical repair is associated with high mortality and reoperation rates. We sought to identify predictors of early and late outcomes.
  
  
  METHODS
  Data from medical records of patients who underwent surgical repair for TAPVC from 1989 to 2012 were included. The patients were divided in two groups, according to absence or presence of associated major CHDs.
  
  
  RESULTS
  Forty-six patients were included (M/F: 26/20, median age 26 days, interquartile range 15 to 59, median weight 3.350 kg, interquartile range 1800 to 4470). Anatomic types of TAPVC were: supracardiac in 48%, intracardiac in 20%, infracardiac in 20%, and mixed in 12%; TAPVC was obstructive in 33%; TAPVC was isolated in 63%, complex in 37%. Single ventricle physiology was present in 11 patients, heterotaxy in eight patients. Overall operative mortality was 19.6% (9/46): 6.9% in isolated TAPVC, 41.2% in complex type (p-value: 0.002). It was associated with low weight at intervention (<3 kg, p = 0.027), single ventricle physiology (p = 0.047), and aortic cross-clamp time >60 minutes (p = 0.097). At a median follow-up of 2.97 years (range 43 days to 22 years, 91% complete), there were nine late deaths (24.3%); 15 patients (40.5%) had major events (including late death). Multivariate analysis of event-free survival showed worse outcome in the complex group (p = 0.001).
  
  
  CONCLUSIONS
  Surgical mortality and morbidity remain consistent in patients with TAPVC and associated major CHD, while the isolated type shows excellent outcomes. Pulmonary vein obstruction is a rare but highly lethal complication. doi: 10.1111/jocs.12399 (J Card Surg 2014;29:678-685).
authors.affiliation: Pediatric and Congenital Cardiac Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences, University of Padova Medical School, Padova, Italy.
authors.forename: Massimo A
authors.initials: MA
authors.lastname: Padalino
authors.forename: Giacomo
authors.initials: G
authors.lastname: Cavalli
authors.forename: Marco
authors.initials: M
authors.lastname: De Franceschi
authors.forename: Daniela
authors.initials: D
authors.lastname: Mancuso
authors.forename: Nicola
authors.initials: N
authors.lastname: Maschietto
authors.forename: Vladimiro
authors.initials: V
authors.lastname: Vida
authors.forename: Ornella
authors.initials: O
authors.lastname: Milanesi
authors.forename: Giovanni
authors.initials: G
authors.lastname: Stellin
mesh_terms: D000015:Abnormalities, Multiple; D001835:Body Weight; D006330:Heart Defects, Congenital / Q000150:complications; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications / Q000517:prevention & control; D011379:Prognosis; D011668:Pulmonary Veno-Occlusive Disease / Q000517:prevention & control; D012189:Retrospective Studies; D012587:Scimitar Syndrome / Q000150:complications / Q000401:mortality / Q000601:surgery*; D013997:Time Factors; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000379:methods / Q000401:mortality