EBSTEIN ANOMALY AND MALFORMATION

Course overview

EBSTEIN ANOMALY IS A RARE CONGENITAL HEART DISEASE THAT INVOLVES THE APICAL DISPLACEMENT OF THE TRICUSPID VALVE WITH ADHERENCE OF THE SEPTAL AND POSTERIOR LEAFLETS TO THE MYOCARDIUM AND "ATRIALIZATION OF THE INLET PORTION OF THE RIGHT VENTRICLE". IT IS USUALLY ACCOMPANIED BY TRICUSPID REGURGITATION, RIGHT VENTRICULAR FAILURE, AND ARRHYTHMIAS. CLINICAL MANIFESTATIONS RANGE FROM ASYMPTOMATIC TO SEVERE, DEPENDING ON THE DEGREE OF TRICUSPID VALVE DISPLACEMENT AND SEVERITY OF REGURGITATION, THE EFFECTIVE RIGHT VENTRICULAR VOLUME, AND THE ASSOCIATED MALFORMATIONS (I.E., PULMONARY VALVE STENOSIS, ATRESIA, ATRIAL SEPTAL DEFECT, ETC.). ARRHYTHMIAS ARE COMMON AND PROTRACTED DUE TO THE LIKELIHOOD OF HAVING ACCESSORY PATHWAYS, IN ADDITION TO HAVING RIGHT ATRIAL DILATATION. SYMPTOMATIC PATIENTS CAN PRESENT WITH CYANOSIS, CONGESTIVE HEART FAILURE, AND ARRHYTHMIAS, WITH EXERTIONAL DYSPNEA BEING COMMON IN OLDER PATIENTS. THIS ACTIVITY REVIEWS THE PATHOPHYSIOLOGY AND PRESENTATION OF EBSTEIN'S MALFORMATION AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN ITS MANAGEMENT.