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

Kacper Jaros1, Maja Butrym2, Jarosław Rycaj3

  • 1Student Scientific Association at the Department of Pediatric Cardiology and Congenital Heart Defects, Silesian Center for Heart Diseases in Zabrze, Faculty of Medical Sciences in Zabrze, Medical University of Silesia, Katowice, Poland.

The International Journal of Cardiovascular Imaging
|February 26, 2026
PubMed

Insights

Uhl's anomaly, a rare congenital heart defect, involves right ventricle absence, leading to heart failure. This case highlights palliative Glenn shunt management in an 18-year-old patient, offering insights into this condition.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Congenital Heart Disease

Background:

  • Uhl's anomaly is an extremely rare congenital heart defect.
  • It is characterized by partial or complete absence of the right ventricular myocardium.
  • This condition leads to progressive right ventricle dilation and right heart failure.

Purpose of the Study:

  • To present a case study of Uhl's anomaly management.
  • To provide insights into the treatment of this rare condition.

Main Methods:

  • Case report of an 18-year-old male patient diagnosed with Uhl's anomaly in infancy.
  • Management involved a palliative Glenn shunt.

Main Results:

  • The patient was diagnosed in early infancy and managed with a palliative Glenn shunt.
  • The report offers insights into the management of this rare condition.

Conclusions:

  • Due to its rarity, there is no established consensus on the surgical or medical management of Uhl's anomaly.
  • Palliative Glenn shunt can be a management option for patients with Uhl's anomaly.

Keywords:
Glenn shuntHeart defects, CongenitalMagnetic resonance imaging, CardiovascularUhl’s anomalyVentricular dysfunction, Right

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