A Novel Imaging Finding in Williams Syndrome: The Coral Sign

Abstract

A 16-year-old female, with a history of Williams syndrome, presented to our institution with a 2-week history of intermittent dizziness. Holter monitoring demonstrated occasional premature ventricular contractions with rare couplets and triplets as well as one short run of nonsustained ventricular tachycardia. Echocardiography revealed an abnormal and irregular left ventricular septum with multiple mobile, pedunculated muscular projections extending into the left ventricular cavity. Cardiac MR confirmed abnormally thickened trabeculations consisting of multiple parallel ridges of myocardium crossing the left ventricle. The appearance of these findings closely resembled bands of coral lining the ocean floor. As such, this finding can henceforth be known as the "coral sign." To our knowledge, no other reports of this finding in patients with Williams syndrome have been published.