Uhl’s anomaly: A one and a half ventricular repair in a patient presenting with cardiac arrest

• Main Authors: Reginald Chounoune, Adam Lowry, Karthik Ramakrishnan, Gail D. Pearson, Jeffrey P. Moak, Dilip S. Nath
• Format: Article
• Language: English
• Published: Saudi Heart Association 2018-01-01
• Series: Journal of the Saudi Heart Association
• Subjects: Cardiac arrest; Glenn anastomosis; Uhl’s anomaly
• Online Access: http://www.sciencedirect.com/science/article/pii/S1016731517300374
• ISSN: 1016-7315

Uhl’s anomaly, first reported in 1952, is an extremely rare congenital cardiac defect characterized by partial or complete loss of the right ventricular myocardium and unknown etiology. Fewer than 100 cases have been described. The response to medical management is poor and there is no known ideal surgical approach or timing for treatment. We report the case of a previously active adolescent male presenting with cardiac arrest, who underwent successful bidirectional cavopulmonary anastomosis (“Glenn” anastomosis) with right atrial reduction and right ventricular free wall plication.