title: Early and intermediate term results for surgical correction of total anomalous pulmonary venous connection.
abstract: BACKGROUND
  This retrospective study evaluated factors associated with mortality and morbidity in patients having functionally biventricular heart (FBH) and functionally univentricular heart (FUH) undergoing total anomalous pulmonary venous connection (TAPVC) repair.
  
  
  METHODS
  We reviewed medical records retrospectively and identified 72 patients undergoing TAPVC repair between July 2002 and December 2010; and 62 patients had FBH and 10 patients had FUH. The median age and weight at repair was 9.1 months (range, 8 days-16 years) and 6.7 kg (range, 2.5-57 kg). The TAPVC anatomy was supracardiac in 41 (56.9%), cardiac in 18 (25%), infracardiac in eight (11.1%), and mixed in five patients (6.9%).
  
  
  RESULTS
  There were seven early deaths (2 FBH [25%] and 5 FUH [62.5%]) and one late death (FUH). Presence of FUH (p < 0.01), heterotaxy syndrome (p = 0.03), young age (p < 0.01), low weight (p < 0.01) and long CPB time (p = 0.04) at the time of surgery were statistically significant risk factors for mortality. Morbidity was related to long duration of postoperative mechanical ventilation, intensive care unit and hospital stay. Presence of FUH was the only significant risk factor for morbidity (p < 0.01). After a mean follow-up of 52.1 months (range, 3-74 months), 59 (92%) of 64 survivors were asymptomatic without any medication, and five have mild symptoms with regular physical activity.
  
  
  CONCLUSIONS
  Operative results of TAPVC have dramatically improved in recent years. However, patients having FUH (especially with concomitant palliative surgery), heterotaxy syndrome, low weight, early age, and long CPB time still have an increased operative mortality.
authors.affiliation: Department of Pediatric Cardiac Surgery, Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey.
authors.forename: Ali Riza
authors.initials: AR
authors.lastname: Karaci
authors.forename: Bugra
authors.initials: B
authors.lastname: Harmandar
authors.forename: Numan Ali
authors.initials: NA
authors.lastname: Aydemir
authors.forename: Ahmet
authors.initials: A
authors.lastname: Sasmazel
authors.forename: Ahmet Yavuz
authors.initials: AY
authors.lastname: Balci
authors.forename: Turkay
authors.initials: T
authors.lastname: Saritas
authors.forename: Ibrahim
authors.initials: I
authors.lastname: Yekeler
authors.forename: Mehmet Salih
authors.initials: MS
authors.lastname: Bilal
mesh_terms: D000015:Abnormalities, Multiple / Q000401:mortality / Q000601:surgery; D000293:Adolescent; D000367:Age Factors; D001835:Body Weight; D002315:Cardiopulmonary Bypass; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006325:Heart Atria / Q000002:abnormalities / Q000601:surgery*; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications; D016016:Proportional Hazards Models; D011667:Pulmonary Veins / Q000002:abnormalities / Q000601:surgery*; D012189:Retrospective Studies; D012307:Risk Factors; D012587:Scimitar Syndrome / Q000401:mortality / Q000601:surgery*; D015996:Survival Rate; D016896:Treatment Outcome