Surgical treatment of Shone’s syndrome and patent ductus arteriosus in an adult

Abstract

BackgroundShone’s syndrome is a rare complex congenital anomaly. The classical definition consists of four anomalies: supravalvular mitral membrane, parachute mitral valve (PMV), subaortic stenosis, and coarctation of the aorta (CoA). Few studies have been reported on Shone’s syndrome in adults, particularly the primary surgical correction of the anomalies.Case presentationA 22-year-old female patient presented with chest distress and tachypnea. Echocardiography and CT revealed supravalvular mitral membrane, PMV, Bicuspid aortic valve stenosis, CoA and patent ductus arteriosus. She underwent primary definitive surgical correction successfully and was discharged from hospital with symptoms free.ConclusionsOur case report highlights the importance of echocardiographic evaluation in the diagnosis of Shone’s syndrome. The surgical strategy should be tailored according to both the patient’s profile and the surgeon’s personal surgical experience. Extra-anatomical bypass procedure is an appropriate technique for adult patients with long-segment coarctation and concomitant cardiac lesions. The outcomes of the case study indicate that the primary definitive surgery is encouraging.