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01-06-2017 | Review

Short QT syndrome in pediatrics

Authors: Roberta Pereira, Oscar Campuzano, Georgia Sarquella-Brugada, Sergi Cesar, Anna Iglesias, Josep Brugada, Fernando E. S. Cruz Filho, Ramon Brugada

Published in: Clinical Research in Cardiology | Issue 6/2017

Abstract

Short QT syndrome is a malignant cardiac disease characterized by the presence of ventricular tachyarrhythmias leading to syncope and sudden cardiac death. Currently, international guidelines establish diagnostic criteria when QTc is below 340 ms. This entity is one of the main diseases responsible for sudden cardiac death in the pediatric population. In recent years, clinical, genetic and molecular advances in pathophysiological mechanisms related to short QT syndrome have improved diagnosis, risk stratification, and preventive measures. Despite these advances, automatic implantable cardiac defibrillator remains the most effective measure. Currently, six genes have been associated with short QT syndrome, which account for nearly 60% of clinically diagnosed families. Here, we review the main clinical hallmarks of the disease, focusing on the pediatric population.

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Title: Short QT syndrome in pediatrics
Authors: Roberta Pereira
Oscar Campuzano
Georgia Sarquella-Brugada
Sergi Cesar
Anna Iglesias
Josep Brugada
Fernando E. S. Cruz Filho
Ramon Brugada
Publication date: 01-06-2017
Publisher: Springer Berlin Heidelberg
Published in: Clinical Research in Cardiology / Issue 6/2017
Print ISSN: 1861-0684
Electronic ISSN: 1861-0692
DOI: https://doi.org/10.1007/s00392-017-1094-1

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