Total anomalous systemic and pulmonary venous connection

摘要

Abstract Total anomalous systemic and pulmonary venous connection is an extremely rare congenital cardiac anomaly. We present our unique experience of managing this complex partially diagnosed cardiac anomaly in a 16-month-old boy. The systemic venous anomaly was not detected during the initial preoperative evaluation. He was doing well on follow-up, with normal pulmonary artery pressure. Total anomalous pulmonary venous connection and total anomalous systemic venous connection account for approximately 2% of congenital cardiac anomalies.1'2 Total anomalous systemic and pulmonary venous connection is an extremely rare cardiac anomaly. Only one such case of common atrium with pulmonary stenosis and anomalous systemic and pulmonary venous connection (cardiac total anomalous pulmonary venous connection) with visceral heterotaxy has been reported in the English literature. A 16-month-old boy weighing 7 kg was admitted to our institute with a history of recurrent respiratory infections since birth. Physical examination revealed cyanosis with systemic oxygen saturation of 78% on room air. Two-dimensional and Doppler echocardiography revealed situs solitus, atrioventricular and ventriculoar-terial concordance. All 4 pulmonary veins formed a common chamber that drained via the vertical vein into the left innominate vein, which in turn drained into a right-sided superior vena cava (SVC). A nonres-trictive secundum atrial septal defect (ASD) shunting blood from the right atrium to the left atrium, with severe pulmonary arterial hypertension. An elective surgical repair was planned under cardiopulmonary bypass and cardioplegic arrest.