Mixed type of total anomalous pulmonary venous connection: diagnosis, surgical approach and outcomes

Journal of Cardiothoracic Surgery

Chun Wu¹  Zhengxia Pan¹  Ming Xiang¹  Quan Wang¹  Linyun Xi¹ 

[1] Department of Cardiothoracic Surgery, Children’s Hospital of Chongqing Medical University, Chongqing, PR China;Ministry of Education Key Laboratory of Child Development and Disorders, Children’s Hospital of Chongqing Medical University, Chongqing, PR China;National Clinical Research Center for Child Health and Disorders(Chongqing), Children’s Hospital of Chongqing Medical University, Chongqing, PR China;China International Science and Technology Cooperation base of Child development and Critical Disorders, Children’s Hospital of Chongqing Medical University, Chongqing, PR China;Chongqing Key Laboratory of Pediatrics, No.136, Zhongshan 2nd Road, Yuzhong Dis, 400014, Chongqing, China;

关键词: Pulmonary vein;    Mixed type;    Mortality;   

DOI  :  10.1186/s13019-020-01332-7

来源: Springer

【 摘 要 】

PurposeTo summarize the diagnosis and treatment of 13 patients with mixed-type total anomalous pulmonary venous connection (TAPVC) and propose another classification for mixed TAPVC.MethodsA retrospective review of 13 patients with mixed TAPVC undergoing repair at a single institution was conducted between January 2010 and November 2019. The diagnosis of mixed-type TAPVC was made in all patients using echocardiography combined with computed tomography angiography. According to the mixed TAPVC anatomy, there were 3 patients with type I TAPVC (2 + 2 veins), 10 patients with type II TAPVC (3 + 1 veins) and no patients with type III TAPVC. Correspondingly, there was 1 patient with the “SVC + VV” subtype, 2 patients with the “CS + C” subtype, 8 patients with the “CS + VV” subtype, 1 patient with the “CS + SVC” subtype and 1 patient with the “RA + SVC” subtype according to our classification system. All patients underwent cardiopulmonary bypass surgery.ResultsThe median weight at surgery was 4.6 ± 1.0 kg (3.4–7.3 kg), and the median age at surgery was 96.2 ± 81.2 days (10–242 days). The median cardiopulmonary bypass time was 132.7 ± 25.1 min (range, 100 to 190 min). The cross-clamping time was 69.2 ± 14.4 min (range, 45 to 88 min). The hospital mortality rate was 7.7% (1 of 13), with late mortality occurring in 1 patient because of pulmonary venous obstruction (PVO) 7 months after surgery. The average follow-up after surgery was 3.4 ± 2.2 years (range, 5 months to 8 years). The survival rates at 3 and 5 years were both 90.9% ± 8.7% (95% CI: 73.8–108%). All remaining surviving patients were asymptomatic.ConclusionMixed TAPVC can be repaired with good results in children and can be correctly diagnosed with echocardiography combined with computed tomography angiography. The classification system we propose is pragmatic and can guide the surgical approach.

【 授权许可】

CC BY