HOLT ORAM SYNDROME

Course overview

HOLT-ORAM SYNDROME ALSO REFERRED TO AS THE HEART-HAND SYNDROME, IS AN AUTOSOMAL DOMINANT DISORDER THAT IS DISTINGUISHED BY UPPER LIMB ABNORMALITIES IN ASSOCIATION WITH CONGENITAL HEART LESIONS. FIRST DESCRIBED IN 1960 BY HOLT AND ORAM, THE SYNDROME WAS IDENTIFIED WHEN THUMB ANOMALIES AND ATRIAL SEPTAL DEFECTS (ASD) WERE OBSERVED IN FAMILY MEMBERS ACROSS FOUR GENERATIONS. THIS ACTIVITY REVIEWS THE PATHOPHYSIOLOGY AND PRESENTATION OF HOLT ORAM SYNDROME AND THE INTERPROFESSIONAL TEAMS ROLL IN MANAGEMENT OF PATIENTS WITH THIS CONDITION.