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Uhl's anomaly: rare but does exist
Parveen Kumar1, Hemant Chaturvedi1, Payal Khatri2
11 Department of Cardiology, Eternal Hospital, Jaipur, India.
Asian Cardiovascular & Thoracic Annals
|September 27, 2018
Summary
This case report highlights Uhl's anomaly, a rare condition causing severe right ventricular dilation and dysfunction. Early diagnosis through advanced imaging is crucial for managing this complex cardiac issue.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Cardiac Imaging
Background:
- Uhl's anomaly is a rare congenital heart condition characterized by the absence of myocardial tissue in the right ventricle.
- It often presents in infancy or childhood with symptoms of right heart failure.
Observation:
- A 17-year-old male presented with facial puffiness, lower limb swelling, and syncope.
- Echocardiography revealed a dilated right atrium and right ventricle with systolic dysfunction and a thinned, trabeculation-devoid right ventricular free wall.
- Cardiac MRI confirmed an extremely dilated, thin-walled right ventricle lacking trabeculations and myocardial fat signal.
Findings:
- The imaging findings were consistent with Uhl's anomaly, differentiating it from arrhythmogenic ventricular dysplasia.
- The case demonstrates the utility of advanced cardiac imaging in diagnosing rare congenital heart conditions.
Implications:
- Accurate diagnosis of Uhl's anomaly is crucial for appropriate management and prognosis.
- This case underscores the importance of considering rare cardiac pathologies in adolescent patients with heart failure symptoms.