title: Total anomalous pulmonary venous connection: 15 years' experience of a tertiary care center in Taiwan.
abstract: BACKGROUND
  Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease in which the connection between the pulmonary vein (PV) and left atrium needs to be surgically created. This study investigated the spectrum and outcome of a Taiwanese cohort.
  
  
  METHODS
  Isolated TAPVC cases were identified from our institutional database between 1995 and 2009. We reviewed the medical chart and conducted telephone interviews with those lost to follow-up.
  
  
  RESULTS
  There were 78 patients (52% male). The anomalous drainage sites were mainly supracardiac type (42.3%) and cardiac type (39.8%). Before operation, PV stenosis was found in 100% of infracardiac type, and in 69.7% of supracardiac type. Among the 75 patients undergoing operation, the surgical mortality was 9% (7/75). Perioperative arrhythmias (mainly of atrial origin) occurred in 35% of the patients. Of the 68 patients who survived the first operation, 28 (41%) developed pulmonary vein restenosis. Half of them progressed to severe PV stenosis, which required reintervention or resulted in mortality. Preoperative PV stenosis was the most significant predictor for postoperative PV restenosis and PV re-intervention. For the cohort, the 1-year and 5-year survivals were 78.9% and 74.2%, respectively, and the predictor for survival was again preoperative PV stenosis.
  
  
  CONCLUSIONS
  The surgical mortality of isolated TAPVC is now low. Preoperative PV stenosis not only increased the risk of late PV restenosis and its reintervention, but also the overall mortality. The spectrum of PV drainage, per se, was not associated with worse outcome. PV restenosis remained the most important issue after correction of TAPVC.
authors: Department of Pediatrics, Hsichu General Hospital, No. 25, Lane 442, Section 1, Jingguo Road, Hsinchu, Taiwan.
authors: Chun-Min
authors: CM
authors: Fu
authors: Jou-Kou
authors: JK
authors: Wang
authors: Chun-Wei
authors: CW
authors: Lu
authors: Shuenn-Nan
authors: SN
authors: Chiu
authors: Ming-Tai
authors: MT
authors: Lin
authors: Chun-An
authors: CA
authors: Chen
authors: Chung-I
authors: CI
authors: Chang
authors: Yih-Sharng
authors: YS
authors: Chen
authors: Ing-Sh
authors: IS
authors: Chiu
authors: Mei-Hwan
authors: MH
authors: Wu
mesh_terms: D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000601:surgery; D013624:Taiwan