Outcomes in Late Presenting Obstructed Infracardiac Total Anomalous Pulmonary Venous Connection-Single Centre Experience.

Abstract

Background: Survival beyond one month of age is rare in children born with obstructed infracardiac total anomalous pulmonary venous connection (TAPVC). There are limited data available on surgical outcomes of the same subset. We conducted this retrospective study to identify risk factors associated with surgical outcomes in this patient population.

Method: We present a retrospective study of patients presenting at our institute with obstructed infracardiac TAPVC surviving beyond one month of age during the period from January 2015 to August 2019 (n = 14). Data reviewed included demographic parameters, preoperative laboratory results, echocardiogram images, and computed tomography if performed. Intraoperative as well as postoperative data including type of surgery, use of deep hypothermic arrest, mortality, and morbidity were noted. Follow-up clinical data were also collected from institutional record or telephonically. We have also collected echocardiographic data on follow up.

Results: The mean age of our study cohort was 2.14 ± 0.68 months. Out of 14 total patients 3 patients eventually died. Risk factors that were significantly associated with mortality were low weight (odds ratio [OR] = 7.8, P = .005), preoperative bilirubin level (OR = 7.4, P = .007), use of deep hypothermic circulatory arrest (OR = 4.6, P = .031), tricuspid regurgitation grade (OR = 3.8, P = .050), and pulmonary artery hypertension (OR = 10.5, P = .001). Mean follow up for the study cohort was 2189 ± 538 days. One patient has mild flow acceleration across the anastomosis with a gradient of 5/3 mm Hg without any symptoms. The remainder of the patients are alive with excellent clinical as well as echocardiographic outcome.

Conclusion: The presentation of infants with obstructed infracardiac TAPVC beyond one month of age is very rare. However, timely intervention can provide acceptable early and excellent late outcomes in this rare patient cohort.