Complete transcatheter correction of variant scimitar syndrome—a case report

Scimitar syndrome (SS) comprises of an anomalously draining right pulmonary vein (APV), to the inferior vena cava (IVC), maldevelopment of the right pulmonary artery (RPA), and the right lung, with variable number of aorto-pulmonary collaterals (APC) to the right lung. It can cause pulmonary hypertension if left untreated. Surgical correction is the method of choice. We report a case of variant SS with dual drainage of the APV to the IVC and left atrium (LA) that was addressed with a transcatheter approach.

A 13-year-old child was evaluated for dyspnoea. Chest x-ray and transthoracic echo (TTE) were suggestive of SS with an additional central atrial septal defect (ASD). Cardiac computed tomography (CT) revealed dual drainage of the APV to the IVC and via a meandering vein to the LA and three APCs. The ASD was closed, and the APCs were coiled. The connection of the APV to the IVC was closed with a device, rerouting the pulmonary vein blood to the LA. The child is doing well on follow-up after 2 years.

Variant forms of SS are rare. Our case had ASD, multiple APCs, well-developed RPA and right lung and a dual drainage of the APV. This allowed for transcatheter management. Otherwise, surgery is the default choice. Multimodality imaging with TTE, CT, magnetic resonance imaging, and cardiac catheterization will help in diagnosis and anatomical delineation.