Total Anomalous Pulmonary Venous Connection: The Current Management Strategies in a Pediatric Cohort of 768 Patients. Issue 1 (3rd January 2017)

• Record Type: Journal Article
• Authors: Shi, Guocheng; Zhu, Zhongqun; Chen, Jimei; Ou, Yanqiu; Hong, Haifa; Nie, Zhiqiang; Zhang, Haibo; Liu, Xiaoqing; Zheng, Jinghao; Sun, Qi; Liu, Jinfen; Chen, Huiwen; Zhuang, Jian
• Is Part Of: Circulation. Volume 135:Issue 1(2017)
• Publication Date: 2017-01-03
• Subjects: heart disease -- pulmonary veins -- surgery; Blood -- Circulation -- Periodicals; Cardiovascular system -- Periodicals; Cardiology -- Periodicals; Heart -- Diseases -- Periodicals; Blood Circulation; Cardiovascular System; Vascular Diseases; 616.1
• DOI: 10.1161/CIRCULATIONAHA.116.023889
• Languages: English
• ISSNs: 0009-7322

Abstract

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. Abstract : Supplemental Digital Content is available in the text.