A rare coexistence of common atrium, univentricular atrioventricular connection, total anomalous pulmonary venous connection, and aortic atresia in an asymptomatic teenage girl

Abstract

A functionally univentricular heart is used to describe a heart with inflow from two atrioventricular valves or a common atrioventricular valve draining into one ventricular chamber in which oxygenated and non-oxygenated blood are mixed together. It accounts for 1% of all congenital cardiac anomalies with a reported incidence of 0.05–0.1 per 10,000 live born babies. On the other hand, common atrium is a rare form of interatrial communication which occurs mainly with atrioventricular septal defect. Combination of common atrium and functionally univentricular heart is very rarely seen and is manifested with severe cyanosis and hypoxia. Total anomalous pulmonary venous connection (TAPVC) is another rare congenital heart anomaly in which all pulmonary veins are draining into the systemic veins, right atrium, or coronary sinus. Its incidence is estimated to be 2.2% of all congenital cardiac anomalies (0.008 of all live births). This anomaly can be seen in association with various types of cardiac malformations. Without cardiac surgery, most of involved infants cannot survive more than a few months of age. Patients with functionally univentricular heart and TAPVC are a considerably high risk group. The majority of cases of aortic valve atresia are reported in hypoplastic left heart syndrome. Here, we report a very rare case of an unoperated 16-year-old girl who had these four congenital cardiac anomalies simultaneously: common atrium, functionally univentricular heart, supracardiac TAPVC with the pulmonary venous confluence draining by a vertical vein into the left brachiocephalic vein and then into the superior vena cava (SVC), and atretic aortic valve. To the best of our knowledge, such case has not been reported previously. 2 | CASE REPORT