A Novel Imaging Finding in Williams Syndrome: The Coral Sign
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.
KeywordsFluorescence in situ hybridization (FISH) Electrocardiogram (EKG) Preventricular contractions (PVCs) Cardiac magnetic resonance (Cardiac MR) Left ventricle (LV) Aorta (AO) Balanced steady-state free precession MRI sequence (“bright blood”) (bSSFP)
Special thanks to Carol Coyne, CCRP, CCRC for assistance with editing the manuscript and Cynthia Long, ARDMS for helping to obtain the echocardiogram images.
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Conflict of interest
The authors declare that they have no conflict of interest.