Introduction:
Total anomalous pulmonary venous connection (TAPVC) is one of the rarest congenital cardiac diseases with 7 per 100000 neonates’ approximate incidence (1,2). It accounts for about 1% of all congenital heart diseases (3). In this anomaly, all the pulmonary veins drain into right atrium (or one of its tributaries) instead of the normal drainage into left atrium (4). Anatomically, TAPVC can be divided into 4 major types depending on the level of pulmonary veins’ drainage: supracardiac (into the innominate vein or right superior vena cava), cardiac (into the coronary sinus or directly to the right atrium), infracardiac (most commonly into the portal vein or into the ductus venosus, hepatic vein, or directly into the infradiaphragmatic inferior vena cava), and mixed (in which there is a combination between types above) (1-4). The separation of both pulmonary and systemic circulations in TAPVC makes the presence of right-to-left shunt obligatory for survival, and any restriction in this communication represents an ”obstruction”. The obstruction may be at any level of the drainage, and is encountered in about 25% to 50% of TAPVC patients. The degree and level of obstruction are remarkable factors affecting the pathophysiology and presentation (5, 6). In the presence of obstruction, the patient is usually symptomatic in the neonatal period, symptoms progressively worsen, and the neonate may present with profound acidosis and hypoxemia despite the aggressive management and oxygen supplement (4). Herein, we report a rare case of an obstructed mixed-type TAPVC in a neonate in association with large VSD in whom successful surgical repair was performed.