Long-Term Follow-Up of Patients With Short QT Syndrome

Objectives The aim of this study was to investigate the clinical characteristics and the long-term course of a large cohort of patients with short QT syndrome (SQTS). Background SQTS is a rare channelopathy characterized by an increased risk of sudden death. Data on the long-term outcome of SQTS pat...

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Bibliographic Details
Published in:Journal of the American College of Cardiology 2011-08, Vol.58 (6), p.587-595
Main Authors: Giustetto, Carla, MD, Schimpf, Rainer, MD, Mazzanti, Andrea, MD, Scrocco, Chiara, MD, Maury, Philippe, MD, Anttonen, Olli, MD, Probst, Vincent, MD, PhD, Blanc, Jean-Jacques, MD, Sbragia, Pascal, MD, Dalmasso, Paola, MS, Borggrefe, Martin, MD, Gaita, Fiorenzo, MD
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age
Anti-Arrhythmia Agents - pharmacology
arrhythmias
Arrhythmias, Cardiac - therapy
Biological and medical sciences
Cardiac arrhythmia
Cardiology
Cardiology. Vascular system
Cardiovascular
channelopathies
Cohort Studies
Death, Sudden
Defibrillators, Implantable
Electrocardiography
Electrocardiography - methods
ERG1 Potassium Channel
Ether-A-Go-Go Potassium Channels - genetics
Families & family life
Female
Follow-Up Studies
Humans
hydroquinidine
implantable cardioverter defibrillator
Internal Medicine
Male
Medical sciences
Methods
Middle Aged
Mutation
Quinidine - analogs & derivatives
Quinidine - pharmacology
short-QT syndrome
Studies
sudden death
Syncope - pathology
Treatment Outcome
Variance analysis
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Summary:Objectives The aim of this study was to investigate the clinical characteristics and the long-term course of a large cohort of patients with short QT syndrome (SQTS). Background SQTS is a rare channelopathy characterized by an increased risk of sudden death. Data on the long-term outcome of SQTS patients are not available. Methods Fifty-three patients from the European Short QT Registry (75% males; median age: 26 years) were followed up for 64 ± 27 months. Results A familial or personal history of cardiac arrest was present in 89%. Sudden death was the clinical presentation in 32%. The average QTc was 314 ± 23 ms. A mutation in genes related to SQTS was found in 23% of the probands; most of them had a gain of function mutation in HERG (SQTS1). Twenty-four patients received an implantable cardioverter defibrillator, and 12 patients received long-term prophylaxis with hydroquinidine (HQ), which was effective in preventing the induction of ventricular arrhythmias. Patients with a HERG mutation had shorter QTc at baseline and a greater QTc prolongation after treatment with HQ. During follow-up, 2 already symptomatic patients received appropriate implantable cardioverter defibrillator shocks and 1 had syncope. Nonsustained polymorphic ventricular tachycardia was recorded in 3 patients. The event rate was 4.9% per year in the patients without antiarrhythmic therapy. No arrhythmic events occurred in patients receiving HQ. Conclusions SQTS carries a high risk of sudden death in all age groups. Symptomatic patients have a high risk of recurrent arrhythmic events. HQ is effective in preventing ventricular tachyarrhythmia induction and arrhythmic events during long-term follow-up.
ISSN:0735-1097
1558-3597
DOI:10.1016/j.jacc.2011.03.038