Abstract / Summary
Total anomalous pulmonary venous connection (TAPVC) is a rare but recognized congenital cardiac defect involving abnormal connection between the pulmonary veins and right atrium instead of left atrium [1]. Its association with severe pulmonary valve stenosis is extremely rare, with reports in the pediatric population being extremely scarce. We present a case of supracardiac TAPVC with severe pulmonary valve stenosis. A 4-year-old boy from Pakistan presented to the cardiology clinic with the complaints of recurrent respiratory tract infections, failure to thrive, shortness of breath on exertion, New York Heart Association (NYHA) class II, with an ejection systolic murmur and mild cyanosis (oxygen saturation 89–94%). Transthoracic echocardiography demonstrated supracardiac total anomalous pulmonary venous connection, with an atrial septal defect (ASD) and severe pulmonary stenosis (PS) (gradient of 75 mmHg), severe tricuspid valve regurgitation (TR), severely dilated right ventricle (RV) with moderate RV hypertrophy, and moderate-to-severe right ventricular hypertension based on interventricular septal positioning. A multidisciplinary team (MDT) meeting decision was to perform complete repair. Open heart surgery included correction of TAPVC along with pulmonary valvotomy and its reconstruction. Postoperative course was uneventful, and the patient was discharged home on the 3rd postoperative day with an echo showing laminar flow in the pulmonary vein left atrial confluence and mild pulmonary valve regurgitation (PR). Though rare, supracardiac TAPVC with severe pulmonary stenosis can be managed with good outcomes with proper planning and surgical execution.