Shone’s complex: An extremely rare congenital cardiac anomaly in a preterm newborn

Abstract

Shone’s Anomaly (SA) is a rare congenital cardiac malformation that consists of four obstructive lesions of the left heart and the aortic arch. We report on a preterm infant with Respiratory Distress syndrome diagnosed with SA after cardiac auscultation revealed a loud second heart sound. A 2D echocardiogram study revealed a large ventricular septal defect and a variant of SA consisting of sub-valvular aortic stenosis, hypoplastic aortic arch, coarctation of the aorta, and dysplastic mitral valve along. The infant underwent a successful Hybrid procedure at two months of age consisting of pulmonary artery banding, stenting of the patent ductus arteriosus, and placement of an atrial stent. Although SA can present in infancy, we have not found a separate report of a preterm infant with SA in the literature. This case presentation raises awareness about this anomaly, sheds light on the importance of careful cardiac examination in newborns, and emphasizes the importance of an echocardiographic exam in evaluating preterm infants with an unusual course of RDS.