Transcatheter intervention in a child with scimitar Syndrome

Abstract

Scimitar syndrome is a rare congenital heart disease characterised by anomalous pulmonary venous drainage to the inferior vena cava, aortopulmonary collaterals, hypoplasia of the right lung and intracardiac defects. Surgical correction remains the gold-standard therapy. However, non-surgical intervention has been reported effective in selected cases with scimitar syndrome. We report on a one-year-old boy with scimitar syndrome who underwent stepwise transcatheter intervention as an alternative treatment. Embolisation of the aortopulmonary collaterals and occlusion of the atrial septal defect were performed using detachable coils and an Amplatzer septal occluder, respectively. The patient's postcathetherisation course was uneventful. The right cardiac chamber and pulmonary arterial pressure returned to normal during follow up.