Why the study?
Shone's complex is a rare congenital heart disease with variants that present with considerable anatomical and clinical heterogeneity.
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.
May warrant consideration for complex reconstruction in late Shone's with severe LV dysfunction; extends evidence for Konno and multi-valve approaches but leaves open optimal selection.
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
No takes yet. Share an insight, caveat, or question.
Mahrouqi et al. (2026) studied this question.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: