Charfo M, Bouceta A, Errami A, Mokri A, Kamal K, Drighil A
Centre hospitalier universitaire Ibn Rochd, Département de cardiologie, Casablanca, Maroc.
CHU Ibn Rochd of Casablanca, Département de Cardiologie, Maroc.
Ann Cardiol Angeiol (Paris). 2023 Oct;72(4):101632. doi: 10.1016/j.ancard.2023.101632. Epub 2023 Aug 26.
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.
肖恩综合征是一种罕见且复杂的先天性心脏病。其完整形式的特征是左心侧有一组四个潜在的阻塞性病变。不完全性肖恩综合征包括2或3个特征性病变,其中包括二尖瓣上膜、降落伞样二尖瓣导致的瓣膜性二尖瓣狭窄、主动脉瓣下狭窄和主动脉缩窄。我们在此描述一名35岁男性患者的病例,该患者因慢性呼吸困难住院,经超声心动图分析确诊为不完全性肖恩综合征。观察到的病变包括导致轻度反流和中度二尖瓣狭窄的降落伞样二尖瓣、二叶式主动脉瓣、主动脉假性中断伴升主动脉扩张以及动脉导管未闭。该病例是德里吉尔先天性心脏病登记处3942例患者中的第2例。