Uhl's Anomaly in Adulthood

Area of Science:

• Cardiology
• Pediatric Cardiology
• Congenital Heart Disease

Background:

• Uhl's anomaly is a rare congenital syndrome defined by the absence of right ventricular myocardium.
• The presumed etiology involves intrauterine myocardial apoptosis, leading to a poor prognosis.

Observation:

• This report details a rare case of a 29-year-old adult diagnosed with Uhl's syndrome.
• The patient was managed at our center, presenting unique clinical circumstances.

Findings:

• The case highlights significant diagnostic challenges associated with Uhl's anomaly in adulthood.
• Management involved complex surgical and postoperative considerations.

Implications:

• This case underscores the possibility of adult survival with Uhl's anomaly, despite its typically poor prognosis.
• Understanding these challenges can improve the care of rare congenital heart conditions.