Shone综合征是一种罕见而复杂的先天性心脏病。它的特点是它的完整形式,由左心侧四个潜在的阻塞性病变组成的星座。不完全光复合体包括2或3个特征性病变,包括二尖瓣膜上,降落伞二尖瓣(PMV)引起的二尖瓣狭窄(MS),主动脉瓣下狭窄和主动脉缩窄(Coa)。我们在这里描述,一个35岁男子的案子,因慢性呼吸困难住院,超声心动图分析保留了不完全Shone's复合体的诊断。观察到的病变包括引起轻度反流和中度二尖瓣狭窄的降落伞二尖瓣。二叶主动脉瓣,和假主动脉中断,升主动脉扩张和动脉导管未闭。该病例是Drighil先天性心脏病登记处3942例患者中的第2例。
Shone syndrome is a rare and complex congenital heart disease. It is characterized in its complete form, by a constellation of the four potentially obstructive lesions of the left heart side. Incomplete Shone\'s complex includes 2 or 3 of the characteristic lesions which, include a supravalvular mitral membrane, valvular mitral stenosis (MS) by a parachute mitral valve (PMV), subaortic stenosis and aortic coarctation (Coa). We describe here, the case of a 35-year-old man, hospitalized for chronic dyspnea and in whom, the diagnosis of incomplete Shone\'s complex is retained by echocardiographic analysis. Observed lesions include a parachute mitral valve responsible for mild regurgitation and moderate mitral stenosis, bicuspid aortic valve, and pseudo interruption of the aorta with dilatation of the ascending aorta and a patent ductus arteriosus. This case is the 2nd on 3942 patients in Drighil congenital heart disease registry.