A 14-year-old girl presenting late with Shone's complex and severe left ventricular dysfunction (EF 25-30%) was successfully treated with multi-valve replacement and the Konno procedure.
Complex surgical reconstruction including the Konno procedure and multiple valve replacements was successfully performed in a pediatric patient with late-presenting Shone's complex and severe left ventricular dysfunction.
Absolute Event Rate: 0% vs 0%
Background: Shone’s complex is a rare CHD, often including supravalvular mitral ring, parachute 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 (BMI 12.37) and cardiac failure, referred for CHD evaluation. Echocardiography and CT revealed a bicuspid AV, severe valvular and subvalvular aortic stenosis, subaortic membrane, muscle ridge causing LVOT obstruction, dilated LV with EF 25–30%, and VSD. Intervention: MV replacement, Konno procedure with LVOT and annular enlargement, AV replacement, and TV repair. She had an uneventful ICU course
Mahrouqi et al. (Wed,) reported a other. A 14-year-old girl presenting late with Shone's complex and severe left ventricular dysfunction (EF 25-30%) was successfully treated with multi-valve replacement and the Konno procedure.