Authors

• Refal Mahrouqi, Faculty of Medicine, Umm Al-Qura University, Makkah, Kingdom of Saudi Arabia
• Asayil Almarjan, Faculty of Medicine, Umm Al-Qura University, Makkah, Kingdom of Saudi ArabiaFollow
• Ahmed Sultan, Department of Cardiac Surgery, King Abdullah Medical City, Makkah (KAMC), Kingdom of Saudi Arabia
• Sheeren Khaled, Department of Cardiology, King Abdullah Medical City, Makkah (KAMC), Kingdom of Saudi Arabia
• ELNAZEER O AHMED, Department of Cardiac Surgery, King Abdullah Medical City, Makkah (KAMC), Kingdom of Saudi Arabia

Late Presentation of Shone’s Complex with Severe Left Ventricular Dysfunction

Abstract

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

Recommended Citation

Mahrouqi, Refal; Almarjan, Asayil; Sultan, Ahmed; Khaled, Sheeren; and AHMED, ELNAZEER O (2026) "Late Presentation of Shone’s Complex with Severe Left Ventricular Dysfunction," Journal of the Saudi Heart Association: Vol. 38 : Iss. 1 , Article 13.
Available at: https://doi.org/10.37616/2212-5043.1489

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