Journal of medical case reports · 2026
Ivemark syndrome in an adult with complete atrioventricular septal defect and a single ventricle with L-transposition of the great arteries: a case report.
СтатьяКлинический случайНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
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Ошибки и ограничения
История · было → стало
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Hibatouallah Hind, El-Mhadi Samah, Siagh Selma, Touati Zakia, Cherti Mohamed
Аннотация
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.
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.