Total Anomalous Pulmonary Venous Connection: The Current Management Strategies in A Pediatric Cohort of 768 Patients
Background—Total anomalous pulmonary venous connection (TAPVC) is a rare form of congenital heart disease. This study describes current surgical treatment strategies and experiences for a cohort of patients from two congenital cardiac centers in Shanghai and Guangdong, China.
Methods—This retrospective study included 768 patients operated on between 2005 and 2014. While most patients (n=690) underwent conventional repair, a sutureless technique was employed 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. Nelson-Aalen cumulative risk curve was used to compare distributions of time to 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 intra-operative deaths and 13 late deaths. A younger age at the time of repair (p=0.001), mixed (p=0.004) and infracardiac TAPVC (p=0.035), pre-operative PVO (p= 0.027), prolonged cardiopulmonary bypass (CPB) time (p<0.001) and longer duration of ventilation (p=0.028) were associated with mortality. The median follow-up was 23.2 (range; 1-112) months. Among the 717 survivors, recurrent PVO was observed in 111 (15%) patients. Associated risk factors for recurrent PVO included preoperative PVO (p<0.001), infracardiac TAPVC (p<0.001), mixed TAPVC (p=0.013), and prolonged CPB time (p<0.001). Sutureless technique was associated with a lower restenosis rate compared with conventional repair in patients with pre-operative PVO (p=0.038), except in newborn patients (p=0.443). Re-intervention for restenosis was performed in 24 patients. The function of most survivors (91%) were classified according to the New York Heart Association (NYHA) as function- 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.
- Received June 7, 2016.
- Revision received October 12, 2016.
- Accepted October 24, 2016.