title: Clinical presentation and echocardiographic characteristics of Uhl's anomaly.
abstract: Uhl's anomaly is a rare cardiac malformation that results in partial or complete absence of the right ventricular myocardium. It most commonly presents in prenatal or newborn infants; however, it may also be found in some adults as advanced right-sided heart failure. Differential diagnoses include arrhythmogenic right ventricular dysplasia and Ebstein's anomaly. Herein, we describe the clinical presentation of Uhl's anomaly in a previously undiagnosed middle-aged adult, and review the echocardiographic criteria used to diagnose and differentiate this rare, albeit important, myocardial disorder.
authors.affiliation: Cardiac Ultrasound Laboratory, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Christos G
authors.initials: CG
authors.lastname: Mihos
authors.affiliation: Division of Cardiology, Mount Sinai Heart Institute, Columbia University, Miami Beach, FL, USA.
authors.forename: Maiteder
authors.initials: M
authors.lastname: Larrauri-Reyes
authors.affiliation: Cardiac Ultrasound Laboratory, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Evin
authors.initials: E
authors.lastname: Yucel
authors.affiliation: Division of Cardiology, Mount Sinai Heart Institute, Columbia University, Miami Beach, FL, USA.
authors.forename: Orlando
authors.initials: O
authors.lastname: Santana
mesh_terms: D002311:Cardiomyopathy, Dilated / Q000000981:diagnostic imaging*; D003937:Diagnosis, Differential; D004452:Echocardiography / Q000379:methods*; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging*; D006352:Heart Ventricles / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D008875:Middle Aged
keywords: Uhl's anomaly; congenital heart disease; echocardiography; heart failure; right ventricle; right ventricular dysfunction