Current Surgical Management of Total Anomalous Pulmonary Venous Connection

Study Questions:

What are the current surgical strategies and outcomes for total anomalous pulmonary venous connection (TAPVC) in China?


A retrospective review was performed at two high-volume surgical centers in China. Patient demographics, vein anatomy, postoperative echocardiography, and hospital- and intermediate-term survival were recorded.


A total of 768 patients (mean age, 214.9 ± 39.2 days; weight, 5.4 ± 3.6 kg) underwent repair of TAPVC between 2005 and 2014. Preoperative studies demonstrated obstructed TAPVC in 192 (25%) patients. A large majority (92%) underwent preoperative computed tomography angiography (CTA). There were 38 intraoperative deaths and 13 late deaths. Risk factors for mortality included younger age at repair, infracardiac TAPVC, preoperative pulmonary venous obstruction (PVO), prolonged cardiopulmonary bypass time, and longer duration of ventilation. The median follow-up was 23.2 (range, 1-112) months. Among the 717 survivors, recurrent PVO was observed in 111 patients (15%). No difference was seen in the restenosis rate with the sutureless technique for patients with unobstructed TAPVC and neonates with obstructed TAPVC, although there was a propensity for lower restenosis rate in older patients with obstructed TAPVC.


The authors concluded that surgical correction in patients with biventricular anatomy can achieve an acceptable outcome. Risk factors associated with a poorer prognosis included younger age at the time of repair, infracardiac and mixed TAPVC, and preoperative venous obstruction.


This study suggests good short- and intermediate-term outcomes after repair of TAPVC in two very large volume surgical centers in China. The mean age of the patient population (approximately 7 months) is older than that seen at many centers. The authors attribute this to late presentation as a result of lack of prenatal diagnosis and lack of health insurance in some patients. There has been some controversy regarding the use of the sutureless technique in reducing the restenosis rate. This study showed that the vast majority of patients did not appear to benefit, although the subset of older patients with obstructed veins appeared to have lower restenosis rates. It is also interesting to note the nearly uniform use of CTA in the preoperative evaluation in this study population. Historical studies (Chin AJ, et al., Am Heart J 1987;113:1153) have shown high accuracy of echocardiography in identifying pulmonary venous anatomy, with some limitation in mixed TAPVC. It is important to accurately identify pulmonary venous return preoperatively, whether it be by echocardiography, CTA, or magnetic resonance imaging.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Vascular Medicine, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, CHD and Pediatrics and Quality Improvement, Interventions and Imaging, Interventions and Structural Heart Disease, Interventions and Vascular Medicine, Angiography, Echocardiography/Ultrasound, Magnetic Resonance Imaging, Nuclear Imaging

Keywords: Angiography, Cardiac Surgical Procedures, Cardiopulmonary Bypass, Echocardiography, Heart Defects, Congenital, Infant, Magnetic Resonance Imaging, Pediatrics, Pulmonary Veno-Occlusive Disease, Risk Factors, Tomography

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