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Cardiac transplantation as resolution for Uhl's anomaly: A case report
Juan J Bacigalupe1, Natalia Vensentini1, Santiago Torroba1
1Cardiology Department, Hospital El Cruce, Buenos Aires, Argentina.
JHLT Open
|August 8, 2025
Summary
Uhl's anomaly, a rare congenital heart defect, typically leads to early death. This case highlights a rare adult survivor who successfully underwent heart transplantation for this condition.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Uhl's anomaly is an extremely rare congenital cardiac defect.
- Characterized by absent right ventricular myocardium, it typically presents with a poor prognosis and is often fatal in the perinatal period.
- Survival into adulthood is exceptionally rare.
Purpose of the Study:
- To report a unique case of adult Uhl's anomaly.
- To discuss the management and outcomes of cardiac transplantation in an adult patient with Uhl's anomaly.
- To highlight Uhl's anomaly as an exceedingly rare indication for adult cardiac transplantation.
Main Methods:
- Case report of a 28-year-old female patient.
- Diagnosis of Uhl's anomaly.
- Management with cardiac transplantation.
Main Results:
- The patient presented with heart failure and ventricular arrhythmia.
- Successful cardiac transplantation was performed.
- Favorable postoperative outcomes were achieved.
Conclusions:
- Uhl's anomaly can present in adulthood with severe cardiac dysfunction.
- Cardiac transplantation is a viable, albeit rare, treatment option for adult patients with Uhl's anomaly.
- This case expands the understanding of the clinical spectrum and management of Uhl's anomaly.
