Treatment considerations in total anomalous pulmonary venous connection

Abstract

Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic abnormality that accounts for about 1%–3% of congenital heart disease malformations. TAPVC is a condition in which there is no direct connection between all four pulmonary veins and the left atrium but makes abnormal connections to the right atrium or systemic venous system. TAPVC caused a high mortality rate of 80% in the first year of life, and 50% of them die within 3 months after birth without intervention. The aim of this review is to elucidate the various treatment considerations of TAPVC. A literature search was conducted on PubMed, ScienceDirect and Google Scholar using various combinations of keywords related to treatment of TAPVC. The citations from all selected articles were reviewed for additional studies. TAPVC intervention, including medical and surgical, is tailored to each type of TAPVC. Catheter-based interventions are frequently used to temporize neonates and provide time to optimize patients medically prior to definite repair. Corrective surgery is required for all patients with this condition. Several latest catheter-based or surgical intervention technique modifications have also been reported.