[Rare case of incomplete Shone syndrome in adult man with marfanoid morphotype]

Abstract

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.

Keywords: Bicuspid aortic valve; Bicuspidie aortique; Complexe de Shone; Mitral stenosis; Parachute mitral valve; Pseudo aortic interruption; Shone's complex; canal artériel persistant; coarctation aortique; patent ductus arteriosus; sténose mitrale; valve mitrale en parachute.