ISSN 1016-5169 | E-ISSN 1308-4488
Archives of the Turkish Society of Cardiology
Adult patient with Shone’s syndrome and patent ductus arteriosus: a case report [Turk Kardiyol Dern Ars]
Turk Kardiyol Dern Ars. 2012; 40(4): 350-353 | DOI: 10.5543/tkda.2012.27003

Adult patient with Shone’s syndrome and patent ductus arteriosus: a case report

Çağdaş Akgüllü1, Ercan Erdoğan2
1Department of Cardiology, Adnan Menderes University Faculty of Medicine, Aydin, Turkey
2Department of Cardiology, Bezmi Alem Vakif University Faculty of Medicine, Istanbul, Turkey

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.

Keywords: Abnormalities, multiple, aortic coarctation; heart defects, congenital; mitral valve/abnormalities; mitral valve stenosis/congenital; mitral valve stenosis; parachute mitral valve; patent ductus arteriosus











How to cite this article
Çağdaş Akgüllü, Ercan Erdoğan. Adult patient with Shone’s syndrome and patent ductus arteriosus: a case report. Turk Kardiyol Dern Ars. 2012; 40(4): 350-353

Corresponding Author: Çağdaş Akgüllü, Türkiye
Manuscript Language: English


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