Surgical Correction of Isolated Total Anomalous Pulmonary Venous Connection: A Prospective Longitudinal Study

Abstract

Introduction: Total Anomalous Pulmonary Venous Connection (TAPVC) is an uncommon congenital heart defect. They are classified into cardiac, supracardiac, infracardiac and mixed subtypes. It has a very high mortality without surgical correction. Aim: To assess the results and early outcomes of surgical repair in patients with isolated total anomalous pulmonary venous drainage with biventricular anatomy. Materials and Methods: This was a prospective study on 27 patients with isolated TAPVC, who underwent surgical correction between January 2012 and December 2018 at a tertiary cardiac care centre in Chennai, Tamil Nadu, India. This study involved detailed pre-operative Echocardiography and CT pulmonary angiography. Analysis of surgical repair, post-operative intensive care unit stay and up to 1 year follow-up of patients was done. Parametric variables were expressed as mean±standard deviation. Chi-square test was used to analyse categorical variables and calculate p-value. Results: The mean age of 27 patients was 4.86±10.08 months and mean weight was 4.69±4.13 kg. The subtype of anomalous connection decided the specific technique of surgical correction. The mean cardio-pulmonary bypass time for all TAPVC types was 111.04±39.82 minutes, mean aortic cross clamp time was 61.44±25.95 minutes and mean hospital stay of the patients was 9.11±2.08 days. There was one early post-operative death in supracardiac TAPVC variant (72 hours after surgery). There were three late post-operative deaths in infracardiac TAPVC variant due to post-operative pulmonary venous obstruction. One patient died three months after surgery and the other two died six months after surgery. The cumulative proportion of patients surviving at the end of one year was 0.88 (standard error of 0.07). Conclusion: Good early outcomes after surgical correction for TAPVC can be achieved. The duration of cardio-pulmonary bypass in infracardiac type of TAPVC is significantly higher than supracardiac and cardiac variants. Infracardiac TAPVC and preoperative Pulmonary Venous Obstruction (PVO) are associated with a poorer prognosis. This subset of patients represents the extreme end of TAPVC spectrum where they often require prolonged cardiopulmonary bypass support.

Keywords

• congenital
• cyanotic
• pulmonary vein stenosis
• total anomalous pulmonary venous return