Supracardiac Total Anomalous Pulmonary Venous Connection in Adolescence

Background Supracardiac total anomalous pulmonary communication (TAPVC) constitutes a rare congenital cardiac anomaly. Most babies with supracardiac TAPVC are diagnosed in infancy and undergo complete surgical repair during infancy. Delayed presentation of supracardiac TAPVC is rare, and the surgical outcomes are not well known. This retrospective study was conducted to determine the presentation and surgical outcome of supracardiac TAPVC among adolescents, which constitutes an extremely rare subgroup of TAPVC.Methodology This retrospective analysis was conducted among 15 adolescent patients with supracardiac TAPVC who underwent surgical repair in the cardiothoracic surgery department of a tertiary care center in India. This study aimed to assess the intraoperative, postoperative, immediate, early, and late outcomes of adolescent patients with supracardiac TAPVC who had undergone surgical repair between 2010 and 2014 in a tertiary care center in India.Results The study included 15 patients with a diagnosis of isolated supracardiac TAPVC. A mild degree of cyanosis was present in eight patients, recurrent episodes of lower respiratory tract infections were present in five patients, and dyspnea (New York Heart Association I/II) was noted in 12 patients. Mean oxygen saturation was 92% (range =85-93%), and mean pulmonary artery pressure was 24 mmHg (range = 15-50 mmHg). After median stenotomy, a wide anastomosis was made between the common pulmonary venous chamber and the posterior wall of the left atrium. A fenestration was made in the Dacron patch in three patients who had raised pulmonary vascular resistance (PVR) preoperatively. Twelve patients were weaned off cardiopulmonary bypass (CPB) with minimal inotropic support. Three patients who had high preoperative PVR had difficulty in weaning from CPB. The mean CPB and cross-clamp time was 75 +/- 12 minutes and 58 +/- 9 minutes, respectively. Atrial fibrillation was noted in five (33.3%) patients in the early postoperative period, and three (20%) patients had pulmonary artery hypertensive crises postoperatively. There was no superficial or deep sternal wound infection in the postoperative period. Mild and moderate right ventricular dysfunction was present in four (26.67%) and two (13.3%) patients, respectively, in the postoperative period. On twodimensional echocardiography during follow-up at the end of one year, there was no gradient across the anastomosis, and pulmonary artery pressure was normal in all patients.Conclusions Surgical repair of supracardiac TAPVC in adolescence has an excellent outcome. Survival of patients with supracardiac TAPVC until adolescence depends on the presence of a dilated vertical vein and a large atrial septal defect facilitating unobstructed pulmonary venous flow. The aim of the surgical repair should be to create a wide anastomosis between the left atrium and the pulmonary venous chamber which should be bigger than the size of the mitral valve orifice indexed to the body surface area as it would amount to no or negligible anastomotic gradient postoperatively.