Surgical Options for Uhl's Anomaly.

Background: Uhl's anomaly is an extremely rare congenital heart defect characterized by a near total absence of the myocardium of the parietal wall of the right ventricle. Few reports of surgical management exist in literature. We present three patients with this anomaly who were managed with different surgical strategies.

Patients And Methods: Patient 1: This 43-month-old girl had maternal rubella syndrome with speech and hearing deficits and gross right heart failure. Diagnosis was made on echocardiography and magnetic resonance imaging. She underwent partial excision and plication of the right ventricular parietal wall and total cavopulmonary connection. Patient 2: This 19-month-old boy presented with progressive cyanosis and features of right heart failure. Diagnosis was confirmed on echocardiography. He underwent right ventricular exclusion by tricuspid valve closure and free wall plication followed by a bidirectional Glenn procedure. Patient 3: This 21-year-old male presented with right heart failure and cyanosis. Diagnosis was established with transesophageal echocardiography and magnetic resonance imaging. As the hemodynamics were not suitable for a Fontan conversion, a one and a half ventricle repair was done along with plication of the right ventricular free wall and tricuspid valve annuloplasty.

Results: All three patients were extubated within 24 hours. Patients 2 and 3 were discharged relatively uneventfully, whereas patient 1 had a more prolonged stay due to transient hepatic failure. All were symptomatically and clinically improved on short-term follow-up.

Conclusion: Uhl's anomaly is an extremely rare condition with varied clinical presentation. Surgical exclusion of the right ventricle yields gratifying results; however, surgical technique has to be adapted to the individual patient.