Shone’s Syndrome and Patent Ductus Arteriosus: A Rare Case Report

Abstract

Introduction: Shone syndrome is a rare congenital anomaly consisting of multiple levels of obstruction in the left side of the heart and aorta. In its complete form it comprises four types of lesions: (1) parachute mitral valve; (2) supravalvular ring of left atrium; (3) subaortic stenosis of either the muscular or membranous type; or (4) coarctation of the aorta. Shone complex is rarely associated with patent ductus arteriosus. Only two other cases of such association has previously been reported. Case Presentation: We report an 18 year old male referred to our center for aortic and mitral valve replacement who was a near complete case of Shone syndrome associated with a history of repaired coarctation and patent ductus arteriosus during infancy and subsequently repair of aortic valve at 3 years of age. Now he became symptomatic due to the progression of aortic stenosis and congenital mitral valve stenosis. Echocardiography revealed unicuspid aortic valve with severe stenosis and parachute mitral valve deformity with supravalvular ring resulting in severe mitral stenosis. The patient had undergone aortic and mitral valve replacements. Conclusions: This rare case is a near complete form of Shone complex associated with patent ductus arteriosus.It is emphasized that when any form of left heart obstruction is encountered we should meticulously look for other associated lesions.