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Congenital Long‐QT Syndrome Concealed by Hypercalcemia in Williams Syndrome
Author(s) -
CZOSEK RICHARD J.,
BERUL CHARLES I.
Publication year - 2008
Publication title -
journal of cardiovascular electrophysiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.193
H-Index - 138
eISSN - 1540-8167
pISSN - 1045-3873
DOI - 10.1111/j.1540-8167.2008.01263.x
Subject(s) - medicine , qt interval , long qt syndrome , concomitant , cardiology , short qt syndrome , electrocardiography , williams syndrome , pediatrics , psychiatry , cognition
We report a case of gene‐positive long‐QT syndrome (KCNH2) in a patient with concomitant Williams Syndrome. The hypercalcemia that developed in association with Williams Syndrome pseudo‐normalized the QTc interval on surface ECG, concealing the clinical and electrocardiographic manifestations of the disease. Initiation of medical therapy for hypercalcemia unmasked the prolonged QT interval, allowing for the diagnosis of long‐QT syndrome to be made.