Akgüllü Cağdaş, Erdoğan Ercan
Department of Cardiology Adnan Menderes University Faculty of Medicine, Aydın, Turkey.
Turk Kardiyol Dern Ars. 2012 Jun;40(4):350-3. doi: 10.5543/tkda.2012.27003.
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.
肖恩综合征是一种非常罕见的心脏畸形,由四种先天性梗阻性病变组成,包括降落伞样二尖瓣(PMV)、瓣上环、主动脉瓣下狭窄和主动脉缩窄。诊断需要进行全面检查,包括仔细进行的超声心动图检查。在成年期仍未被诊断的患者极为罕见。在此,我们报告一名患有肖恩综合征和动脉导管未闭(PDA)的26岁男性患者。患者自述一直存在运动不耐受和进行性加重的呼吸困难。两年前,他在另一家医院接受手术,当时误诊为二尖瓣脱垂和主动脉瓣下膜。经胸超声心动图显示存在PMV,通过先前植入的二尖瓣环导致严重二尖瓣狭窄。还观察到肖恩综合征的其他组成部分,即主动脉瓣下膜和主动脉缩窄,以及PDA。建议再次手术,但患者拒绝了。在两年的随访期间,尽管患者发生了房颤发作且呼吸困难加重,但仍不想接受再次手术。一旦检测到肖恩综合征复合体的四个组成部分中的任何一个,临床医生必须查找是否存在其他病变。