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Updated: Apr 30, 2026
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High Frequency Ultrasound for the Analysis of Fetal and Placental Development In Vivo
Published on: November 8, 2018
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Uhl's anomaly: a difficult prenatal diagnosis.
Laurence Vaujois1, Nicolaas van Doesburg1, Marie-Josée Raboisson1
1Department of Pediatrics,Sainte-Justine Hospital Center,Division of Pediatric Cardiology,Montréal,Québec,Canada.
Cardiology in the Young
|May 3, 2014
Summary
Uhl's anomaly, a rare condition causing right ventricular failure, was studied in a fetus. Progressive myocardial reduction, possibly due to apoptosis, led to fetal demise.
Area of Science:
- Cardiology
- Fetal Medicine
- Developmental Biology
Background:
- Uhl's anomaly is a rare cardiac condition characterized by the absence of normal right ventricular myocardium.
- It leads to progressive right ventricular failure and is challenging to differentiate from Ebstein anomaly.
- Early diagnosis and understanding of its pathophysiology are crucial for management.
Purpose of the Study:
- To describe the prenatal evolution of Uhl's anomaly.
- To investigate the potential mechanisms, such as apoptosis, involved in myocardial reduction.
- To highlight the diagnostic challenges in differentiating Uhl's anomaly from other congenital heart diseases.
Main Methods:
- Case report of a fetus diagnosed with Uhl's anomaly.
- Serial ultrasound examinations from 21 to 30 weeks of gestation.
- Histopathological examination of the fetus post-demise (implied).
Main Results:
- Progressive reduction in the anterior right ventricular myocardium was observed during gestation.
- The observed changes were suggestive of apoptosis.
- The condition ultimately led to fetal demise.
Conclusions:
- Uhl's anomaly can exhibit progressive myocardial changes during fetal development.
- Apoptosis may play a role in the pathogenesis of Uhl's anomaly.
- This case underscores the importance of considering Uhl's anomaly in the differential diagnosis of fetal right heart failure.
Keywords:
foetal echocardiography
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