Adult Patient with Shone's Syndrome and Patent Ductus Arteriosus

Abstract

Shone's syndrome is a very rare cardiac malformation composed of four obstructive congenital lesions, which include the parachute mitral valve (PMV), supravalvular ring, subaortic stenosis, and coarctation of aorta. Diagnosis requires a comprehensive examination including a carefully performed echocardiography. It is extremely unusual for a patient to remain undiagnosed during adulthood. Here we report a 26-year-old male patient with Shone's syndrome and patent ductus arteriosus (PDA). The patient reported that he had been suffering from exercise intolerance and aggravating dyspnea. Two years ago, he was operated on in another hospital based on the wrong diagnosis of mitral valve prolapse and subaortic membrane. Transthoracic echocardiography revealed the existence of a PMV, which led to severe mitral stenosis through a previously implanted mitral annular ring. Other components of Shone's syndrome, subaortic membrane and aortic coarctation, together with PDA, were also observed. Another operation was suggested, but the patient refused. During the two year follow-up period, in spite of an atrial fibrillation attack and deteriorating dyspnea, the patient still did not want another operation. Once any of the four components of the Shone's complex is detected, clinicians must look for the presence of other lesions.