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Case Reports in Medicine
Volume 2019, Article ID 1384139, 4 pages
Case Report

A Rare Case of the Digenic Inheritance of Long QT Syndrome Type 2 and Type 6

Department of Cardiology, Erasmus Medical Center, Rotterdam, Netherlands

Correspondence should be addressed to Natasja M. S. de Groot; moc.oohay@toorgedsmn

Received 25 January 2019; Revised 11 April 2019; Accepted 23 May 2019; Published 20 June 2019

Academic Editor: Alexander Bauer

Copyright © 2019 Annejet Heida et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


We report a 37-year-old woman with an out-of-hospital cardiac arrest caused by ventricular fibrillation due to digenic inheritance of long QT syndrome type 2 (KCNH2 gene) and type 6 (KCNE2 gene). During hospitalization, prolonged QTc intervals and frequent episodes of ventricular tachyarrhythmias manifested. Genetic testing identified a mutation of the KCNH2 gene and an unclassified variant, most likely pathogenic, of the KCNE2 gene. This digenic inheritance is extremely rare.