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<p>Uhl’s anomaly is an extremely rare congenital heart defect, with approximately 100 cases reported since its first description in 1952 by Dr Henry Uhl. Characterized by partial or complete absence of the right ventricular myocardium, it leads to progressive right ventricle dilation and right heart failure. Due to its rarity, there is no consensus regarding its surgical or medical management. We present a case of an 18-year-old male patient with Uhl’s anomaly diagnosed in early infancy, managed by palliative Glenn shunt. This report provides insight into management of this rare condition.</p>

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Uhl’s anomaly after Glenn shunt - clinical image of a rare congenital heart defect

  • 期刊论文

  • 发表日期：

    2026年2月26日

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  • Kacper Jaros,
  • Maja Butrym,
  • Jarosław Rycaj,
  • Mateusz Knop,
  • Roland Fiszer

摘要

Uhl’s anomaly is an extremely rare congenital heart defect, with approximately 100 cases reported since its first description in 1952 by Dr Henry Uhl. Characterized by partial or complete absence of the right ventricular myocardium, it leads to progressive right ventricle dilation and right heart failure. Due to its rarity, there is no consensus regarding its surgical or medical management. We present a case of an 18-year-old male patient with Uhl’s anomaly diagnosed in early infancy, managed by palliative Glenn shunt. This report provides insight into management of this rare condition.