title: Total Anomalous Pulmonary Venous Connection: The Current Management Strategies in a Pediatric Cohort of 768 Patients.
abstract: BACKGROUND
  Total anomalous pulmonary venous connection (TAPVC) is a rare form of congenital heart disease. This study describes current surgical treatment strategies and experiences in a cohort of patients from 2 congenital cardiac centers in Shanghai and Guangdong in China.
  
  
  METHODS
  This retrospective study included 768 patients operated on between 2005 and 2014. Although most patients (n=690) underwent conventional repair, a sutureless technique was used in 10% (n=78) of cases. A multilevel mixed-effects parametric survival model and a competing-risk analysis were used to analyze associated risk factors for death and recurrent pulmonary venous obstruction (PVO), respectively. Kaplan-Meier analysis was used to analyze the overall survival. The Nelson-Aalen cumulative risk curve was used to compare distributions of time with recurrent PVO.
  
  
  RESULTS
  The mean surgical age and weight were 214.9±39.2 days and 5.4±3.6 kg, respectively. Obstructed TAPVC (PVO) was documented in 192 (25%) of the 768 patients. There were 38 intraoperative deaths and 13 late deaths. A younger age at the time of repair (P=0.001), mixed (P=0.004) and infracardiac (P=0.035) TAPVC, preoperative PVO (P=0.027), prolonged cardiopulmonary bypass time (P<0.001), and longer duration of ventilation (P=0.028) were associated with mortality. The median follow-up was 23.2 months (range; 1-112 months). Among the 717 survivors, recurrent PVO was observed in 111 patients (15%). Associated risk factors for recurrent PVO included preoperative PVO (P<0.001), infracardiac TAPVC (P<0.001), mixed TAPVC (P=0.013), and prolonged cardiopulmonary bypass time (P<0.001). Sutureless technique was associated with a lower restenosis rate compared with conventional repair in patients with preoperative PVO (P=0.038), except in newborn patients (P=0.443). Reintervention for restenosis was performed in 24 patients. The function of most survivors (91%) was classified according to the New York Heart Association as functional class I or II.
  
  
  CONCLUSIONS
  Surgical correction in patients with TAPVC with a biventricular anatomy can achieve an acceptable outcome. Risk factors such as a younger age at the time of repair, infracardiac and mixed TAPVC, and preoperative PVO were associated with a poorer prognosis.
authors.affiliation: From Department of Cardiothoracic Surgery, Heart Center, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China (G.S., Z.Z., H.H., H.Z., J. Zheng, Q.S., J.L., H.C.); and Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences (J.C., Y.O., Z.N., X.L., J. Zhuang), Guangdong, China.
authors.forename: Guocheng
authors.initials: G
authors.lastname: Shi
authors.affiliation: From Department of Cardiothoracic Surgery, Heart Center, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China (G.S., Z.Z., H.H., H.Z., J. Zheng, Q.S., J.L., H.C.); and Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences (J.C., Y.O., Z.N., X.L., J. Zhuang), Guangdong, China.
authors.forename: Zhongqun
authors.initials: Z
authors.lastname: Zhu
authors.affiliation: From Department of Cardiothoracic Surgery, Heart Center, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China (G.S., Z.Z., H.H., H.Z., J. Zheng, Q.S., J.L., H.C.); and Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences (J.C., Y.O., Z.N., X.L., J. Zhuang), Guangdong, China.
authors.forename: Jimei
authors.initials: J
authors.lastname: Chen
authors.affiliation: From Department of Cardiothoracic Surgery, Heart Center, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China (G.S., Z.Z., H.H., H.Z., J. Zheng, Q.S., J.L., H.C.); and Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences (J.C., Y.O., Z.N., X.L., J. Zhuang), Guangdong, China.
authors.forename: Yanqiu
authors.initials: Y
authors.lastname: Ou
authors.affiliation: From Department of Cardiothoracic Surgery, Heart Center, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China (G.S., Z.Z., H.H., H.Z., J. Zheng, Q.S., J.L., H.C.); and Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences (J.C., Y.O., Z.N., X.L., J. Zhuang), Guangdong, China.
authors.forename: Haifa
authors.initials: H
authors.lastname: Hong
authors.affiliation: From Department of Cardiothoracic Surgery, Heart Center, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China (G.S., Z.Z., H.H., H.Z., J. Zheng, Q.S., J.L., H.C.); and Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences (J.C., Y.O., Z.N., X.L., J. Zhuang), Guangdong, China.
authors.forename: Zhiqiang
authors.initials: Z
authors.lastname: Nie
authors.affiliation: From Department of Cardiothoracic Surgery, Heart Center, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China (G.S., Z.Z., H.H., H.Z., J. Zheng, Q.S., J.L., H.C.); and Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences (J.C., Y.O., Z.N., X.L., J. Zhuang), Guangdong, China.
authors.forename: Haibo
authors.initials: H
authors.lastname: Zhang
authors.affiliation: From Department of Cardiothoracic Surgery, Heart Center, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China (G.S., Z.Z., H.H., H.Z., J. Zheng, Q.S., J.L., H.C.); and Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences (J.C., Y.O., Z.N., X.L., J. Zhuang), Guangdong, China.
authors.forename: Xiaoqing
authors.initials: X
authors.lastname: Liu
authors.affiliation: From Department of Cardiothoracic Surgery, Heart Center, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China (G.S., Z.Z., H.H., H.Z., J. Zheng, Q.S., J.L., H.C.); and Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences (J.C., Y.O., Z.N., X.L., J. Zhuang), Guangdong, China.
authors.forename: Jinghao
authors.initials: J
authors.lastname: Zheng
authors.affiliation: From Department of Cardiothoracic Surgery, Heart Center, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China (G.S., Z.Z., H.H., H.Z., J. Zheng, Q.S., J.L., H.C.); and Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences (J.C., Y.O., Z.N., X.L., J. Zhuang), Guangdong, China.
authors.forename: Qi
authors.initials: Q
authors.lastname: Sun
authors.affiliation: From Department of Cardiothoracic Surgery, Heart Center, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China (G.S., Z.Z., H.H., H.Z., J. Zheng, Q.S., J.L., H.C.); and Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences (J.C., Y.O., Z.N., X.L., J. Zhuang), Guangdong, China.
authors.forename: Jinfen
authors.initials: J
authors.lastname: Liu
authors.affiliation: From Department of Cardiothoracic Surgery, Heart Center, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China (G.S., Z.Z., H.H., H.Z., J. Zheng, Q.S., J.L., H.C.); and Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences (J.C., Y.O., Z.N., X.L., J. Zhuang), Guangdong, China. chenhuiwen@scmc.com.cn drzhuangjian5413@163.com.
authors.forename: Huiwen
authors.initials: H
authors.lastname: Chen
authors.affiliation: From Department of Cardiothoracic Surgery, Heart Center, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China (G.S., Z.Z., H.H., H.Z., J. Zheng, Q.S., J.L., H.C.); and Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences (J.C., Y.O., Z.N., X.L., J. Zhuang), Guangdong, China. chenhuiwen@scmc.com.cn drzhuangjian5413@163.com.
authors.forename: Jian
authors.initials: J
authors.lastname: Zhuang
mesh_terms: D002315:Cardiopulmonary Bypass; D002675:Child, Preschool; D015331:Cohort Studies; D000072226:Computed Tomography Angiography; D023903:Coronary Restenosis / Q000209:etiology; D004452:Echocardiography; D005260:Female; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D011183:Postoperative Complications; D011379:Prognosis; D016016:Proportional Hazards Models; D011668:Pulmonary Veno-Occlusive Disease / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors; D014691:Ventilation
keywords: heart disease; pulmonary veins; surgery