Abstract
Congenital heart disease may coexist with airway abnormalities, increasing perioperative risk. We report a 2-year-old girl with a complete atrioventricular canal defect (CAVCD), left superior vena cava (LSVC), and severe pulmonary hypertension, in whom multimodality imaging was pivotal for diagnosis and management. Transthoracic echocardiography confirmed CAVCD, LSVC, and severe pulmonary hypertension. Preoperative computed tomography demonstrated left pulmonary artery compression of the left main bronchus and a suspected cor triatriatum. Intraoperative bronchoscopy revealed left bronchomalacia, while transesophageal echocardiography identified a dilated coronary sinus as the cause of left atrial inflow turbulence, excluding cor triatriatum. Surgical management included CAVCD repair and LSVC anastomosis to the right atrial appendage. Post-cardiopulmonary bypass echocardiography showed no residual atrioventricular valve regurgitation or inflow gradient. Lung ultrasound prior to extubation excluded left lung collapse. This case highlights the role of multimodality imaging in guiding diagnosis, surgical planning, and safe perioperative management.