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Sinus bradycardia, junctional rhythm, and low-rate atrial fibrillation in Short QT syndrome during 20 years of follow-up: three faces of the same genetic problem

Published online by Cambridge University Press:  17 August 2015

Daniela Righi ,
Massimo S. Silvetti  and
Fabrizio Drago
Daniela Righi*
Affiliation:
Department of Pediatric Cardiology and Cardiac Surgery, Cardiac Arrhythmia Complex Unit and Syncope Unit, Bambino Gesù Children’s Hospital and Research Institute, Fiumicino (Rome), Italy
Massimo S. Silvetti
Affiliation:
Department of Pediatric Cardiology and Cardiac Surgery, Cardiac Arrhythmia Complex Unit and Syncope Unit, Bambino Gesù Children’s Hospital and Research Institute, Fiumicino (Rome), Italy
Fabrizio Drago
Affiliation:
Department of Pediatric Cardiology and Cardiac Surgery, Cardiac Arrhythmia Complex Unit and Syncope Unit, Bambino Gesù Children’s Hospital and Research Institute, Fiumicino (Rome), Italy
*
Correspondence to: D. Righi, MD, Department of Pediatric Cardiology and Cardiac Surgery, Cardiac Arrhythmia Complex Unit and Syncope Unit, Bambino Gesù Children’s Hospital and Research Institute, Via Torre di Palidoro, snc, 00050 Fiumicino (Rome), Italy. Tel: +39 066 859 3559; Fax: +39 066 859 4841; E-mail: righi.daniela@gmail.com
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Abstract

We describe the case of an asymptomatic girl with sinus bradycardia and short QT interval at birth, junctional bradycardia in infancy requiring single-chamber pacemaker, atrial fibrillation in adolescence, and V141M mutation in the KCNQ1 gene. Atrial fibrillation recurred and became unresponsive to electrical or anti-arrhythmic therapy. During 20 years of follow-up, a progressive evolution from sinus node dysfunction to low-rate atrial fibrillation was observed.

Keywords

Atrial fibrillationgene mutationgenetic testingShort QT syndromesudden cardiac death
Type
Brief Reports
Information
Cardiology in the Young , Volume 26 , Issue 3 , March 2016 , pp. 589 - 592
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Copyright
© Cambridge University Press 2015 

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