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Background <p>Ivemark syndrome is a rare heterotaxy disorder characterized by right atrial isomerism, complex congenital heart defects, and thoraco-abdominal anomalies. Because of such defects, survival beyond infancy is rare.</p> Case presentation <p>We report a case of a 31-year-old North African male with lifelong cyanosis who presented with worsening exertional dyspnea and fatigue. A segmental approach revealed heterotaxy with right atrial isomerism, a complete atrioventricular septal defect, a functional single ventricle with L-transposition of the great arteries, and severe valvular and supravalvular pulmonic&#xa0; stenosis. Despite these complex anomalies, the patient maintained a balanced circulation that enabled survival into adulthood. A multidisciplinary team recommended a palliative care approach, including intravenous hydration, iron supplementation, allopurinol, and preventive measures for infective endocarditis. The patient remains under regular noninvasive follow-up, with a stable condition.</p> Conclusion <p>This case highlights the rare adult presentation of Ivemark syndrome. Long-term survival is exceptionally rare and depends on balanced hemodynamics, comprehensive evaluation, and individualized multidisciplinary care.</p>

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Ivemark syndrome in an adult with complete atrioventricular septal defect and a single ventricle with L-transposition of the great arteries: a case report

  • 期刊论文

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  • 发表日期：

    2026年1月19日

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  • Hind Hibatouallah,
  • Samah El-Mhadi,
  • Selma Siagh,
  • Zakia Touati,
  • Mohamed Cherti

摘要

Background

Ivemark syndrome is a rare heterotaxy disorder characterized by right atrial isomerism, complex congenital heart defects, and thoraco-abdominal anomalies. Because of such defects, survival beyond infancy is rare.

Case presentation

We report a case of a 31-year-old North African male with lifelong cyanosis who presented with worsening exertional dyspnea and fatigue. A segmental approach revealed heterotaxy with right atrial isomerism, a complete atrioventricular septal defect, a functional single ventricle with L-transposition of the great arteries, and severe valvular and supravalvular pulmonic  stenosis. Despite these complex anomalies, the patient maintained a balanced circulation that enabled survival into adulthood. A multidisciplinary team recommended a palliative care approach, including intravenous hydration, iron supplementation, allopurinol, and preventive measures for infective endocarditis. The patient remains under regular noninvasive follow-up, with a stable condition.

Conclusion

This case highlights the rare adult presentation of Ivemark syndrome. Long-term survival is exceptionally rare and depends on balanced hemodynamics, comprehensive evaluation, and individualized multidisciplinary care.