The complex form of anomalous pulmonary venous return; new generation imaging and surgical approach of scimitar syndrome

Abstract

Scimitar syndrome is a rare congenital heart disease where anamolous pulmonary veins drain into inferior vena cava Its pathogenesis is unknown and it is caused by abnormal development of the pulmonary bud during early embryogenesis In the chest radiography the shadow of anomalous veins resembles a Turkish sword better known as scimitar located between the lower right lung and the heart It is associated with other anomalies such as right pulmonary hypoplasia cardiac dextroposition anomalous systemic arterial supply to the right lung pulmonary sequestration and atrial septal defect In this article we present an 8 year old boy operated for scimitar syndrome with partial anomalous pulmonary venous return draining into the inferior vena cava atrial septal defect dextropositon of the heart and hypoplastic right lung The diagnostic significance of new generation imaging methods and surgical strategy have been scrutinized Turk Arch Ped 2013; 48: 173 5