[Uhl's anomaly. A case report with anatomoechocardiographic correlation]

Area of Science:

• Cardiology
• Developmental Biology

Background:

• Uhl's anomaly is a rare congenital heart defect characterized by the absence of right ventricular myocardium.
• It typically presents with normal tricuspid and pulmonary valves.

Observation:

• A 33-year-old male presented with symptoms suggestive of atrial septal defect and signs of right ventricular failure.
• Echocardiography revealed right atrial and ventricular dilation with a thin, smooth right ventricular free wall.

Findings:

• The echocardiographic findings were consistent with the diagnosis of Uhl's anomaly.
• Anatomoechocardiographic correlation with a cardiac specimen provided precise insights into the anomaly's morphology.

Implications:

• Echocardiography is crucial for diagnosing Uhl's anomaly.
• Anatomoechocardiographic correlation aids in a deeper comprehension of this rare cardiac malformation.