Case Report--Late Presentation of Shone's Complex With Severe Left Ventricular Dysfunction.

BACKGROUND

Shone's complex is a rare Congenital Heart Disease (CHD), often including supravalvular mitral ring, parachute Mitral Valve (MV), subaortic stenosis, and coarctation of the aorta. Variants of this complex have been reported with considerable anatomical and clinical heterogeneity.

CASE PRESENTATION: A 14-year-old girl with severe cachexia and cardiac failure, referred for CHD evaluation. Echocardiography and CT revealed a bicuspid Aortic Valve (AV), severe valvular and subvalvular aortic stenosis, a subaortic membrane, a muscle ridge causing Left Ventricular Outflow Tract (LVOT) obstruction, dilated LV with EF 25-30%, and a ventricular septal defect.

INTERVENTION: MV replacement, Konno procedure with LVOT and annular enlargement, AV replacement, and Tricuspid Valve (TV) repair. She had an uneventful ICU course.