Abstract
We report a rare case of Scimitar syndrome (adult variant) diagnosed in a 57-year-old woman. The patient presented with symptoms of right heart failure and a history of old cerebral infarction. Multimodality imaging (transthoracic and transesophageal echocardiography, MSCT, and cardiac catheterization) confirmed a complex congenital heart defect including: (1) Partial anomalous pulmonary venous return (Scimitar syndrome) with the right lower pulmonary vein draining into the inferior vena cava (IVC); (2) A 10mm secundum atrial septal defect (ASD); (3) Severe pulmonary hypertension (PH) with elevated pulmonary vascular resistance (Rp = 7 WU); (4) A persistent left superior vena cava (PLSVC) draining into the coronary sinus; and (5) Multiple old cerebral infarcts, with the mechanism identified as a right-to-left shunt consistent with paradoxical embolism. The patient underwent successful complete surgical repair using an intra-atrial baffle technique. The 4-year follow-up showed remarkable hemodynamic improvement (PAPs decreased from 52 mmHg to 39 mmHg), affirming the value of surgical intervention even in older patients with severe pulmonary hypertension.