Overview and surgical aspects of Uhls anomaly

Introduction: Uhls anomaly is an extremely rare congenital heart defect characterized by partial or complete absence of right ventricular myocardium. Less than 100 cases have been reported in the literature. Survival depends on the severity of the right ventricular dysfunction. The age at presentation is varied but usually presents in infancy or found during autopsy. Diagnosis is made by echocardiogram (ECHO), magnetic resonance imaging (MRI), and histopathological examination. Medical management of this condition understanding the pathology is not possible. Surgical treatment includes single ventricle strategy, one and a half ventricle repair with partial right ventriculectomy, Fontan procedure, and cardiac transplantation. Early recognition of the anomaly, institution of drugs that improves cardiac function and right surgical planning may improve the quality of life.Areas covered: An extensive literature search has been done from many Pubmed and Scopus-indexed journals pertaining to various aspects of Uhls anomaly including history of the disease, etiology, clinical presentation, differential diagnosis, diagnostic aids, and medical and surgical management.Expert opinion: Depending on the hemodynamics, a bidirectional Glenn which is septectomy or a single ventricle pathway would be a better approach. Of course heart transplantation would be the last choice when other surgical options are defied.