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

Refal Mahrouqi1, Asayil Almarjan1, Ahmed Sultan2,3

  • 1Faculty of Medicine, Umm Al-Qura University, Makkah, Kingdom of Saudi Arabia.

Journal of the Saudi Heart Association
|April 16, 2026
PubMed

Insights

This case highlights a complex congenital heart disease (CHD) presentation in a young patient. Surgical intervention successfully addressed multiple cardiac anomalies, improving the patient's condition.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Congenital Heart Disease

Background:

  • Shone's complex is a rare congenital heart disease (CHD) with variable anatomical and clinical features.
  • It commonly involves supravalvular mitral ring, parachute Mitral Valve (MV), subaortic stenosis, and coarctation of the aorta.

Purpose of the Study:

  • To present a complex case of Shone's complex in a 14-year-old girl with severe cardiac failure.
  • To detail the diagnostic findings and surgical management of this rare CHD variant.

Main Methods:

  • Diagnostic evaluation included echocardiography and CT.
  • Surgical intervention involved Mitral Valve (MV) replacement, Konno procedure with Left Ventricular Outflow Tract (LVOT) and annular enlargement, Aortic Valve (AV) replacement, and Tricuspid Valve (TV) repair.

Main Results:

  • The patient presented with severe cachexia, cardiac failure, bicuspid Aortic Valve (AV), severe valvular and subvalvular aortic stenosis, subaortic membrane, LVOT obstruction, dilated Left Ventricle (LV) with reduced ejection fraction (EF), and a ventricular septal defect.
  • The patient experienced an uneventful intensive care unit (ICU) course post-operatively.

Conclusions:

  • Complex congenital heart disease, such as Shone's complex, requires comprehensive diagnostic assessment.
  • Multistage surgical repair can effectively manage severe forms of Shone's complex, leading to favorable outcomes.
Abstract

Keywords:
Congenital heart diseaseLeft ventricular outflow tract obstructionParachute mitral valveShone’s complex

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