Uhl's anomaly is a rare congenital syndrome characterized by the absence of right ventricular myocardium. The widely accepted pathological mechanism is intrauterine myocardial apoptosis. Uhl's syndrome carries a poor prognosis. In rare situations, patients with Uhl's anomaly reach adulthood. We will present a case of a 29-year-old patient with Uhl's syndrome treated at our center, highlighting the diagnostic, surgical, and postoperative challenges in management.
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