Document Detail


Sinus node function and ventricular repolarization during exercise stress test in long QT syndrome patients with KvLQT1 and HERG potassium channel defects.
MedLine Citation:
PMID:  10483966     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVES: This study was performed to evaluate the QT interval and heart rate responses to exercise and recovery in gene and mutation type-specific subgroups of long QT syndrome (LQTS) patients. BACKGROUND: Reduced heart rate and repolarization abnormalities are encountered among long QT syndrome (LQTS) patients. The most common types of LQTS are LQT1 and LQT2. METHODS: An exercise stress test was performed in 23 patients with a pore region mutation and in 22 patients with a C-terminal end mutation of the cardiac potassium channel gene causing LQT1 type of long QT syndrome (KVLQT1 gene), as well as in 20 patients with mutations of the cardiac potassium channel gene causing LQT2 type of long QT syndrome (HERG gene) and in 33 healthy relatives. The QT intervals were measured on electrocardiograms at rest and during and after exercise. QT intervals were compared at similar heart rates, and rate adaptation of QT was studied as QT/heart rate slopes. RESULTS: In contrast to the LQT2 patients, achieved maximum heart rate was decreased in both LQT1 patient groups, being only 76 +/- 5% of predicted in patients with pore region mutation of KvLQT1. The QT/heart rate slopes were significantly steeper in LQT2 patients than in controls during exercise. During recovery, the QT/heart rate slopes were steeper in all LQTS groups than in controls, signifying that QT intervals lengthened excessively when heart rate decreased. At heart rates of 110 or 100 beats/min during recovery, all LQT1 patients and 89% of LQT2 patients had QT intervals longer than any of the controls. CONCLUSIONS: LQT1 is associated with diminished chronotropic response and exaggerated prolongation of QT interval after exercise. LQT2 patients differ from LQT1 patients by having marked QT interval shortening and normal heart rate response to exercise. Observing QT duration during recovery enhances the clinical diagnosis of these LQTS types.
Authors:
H Swan; M Viitasalo; K Piippo; P Laitinen; K Kontula; L Toivonen
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of the American College of Cardiology     Volume:  34     ISSN:  0735-1097     ISO Abbreviation:  J. Am. Coll. Cardiol.     Publication Date:  1999 Sep 
Date Detail:
Created Date:  1999-09-24     Completed Date:  1999-09-24     Revised Date:  2008-10-28    
Medline Journal Info:
Nlm Unique ID:  8301365     Medline TA:  J Am Coll Cardiol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  823-9     Citation Subset:  AIM; IM    
Affiliation:
Department of Medicine, Helsinki University Central Hospital, Finland. heikki.swan@helsinki.fi
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Cation Transport Proteins*
Child
DNA-Binding Proteins*
Electrocardiography / methods,  statistics & numerical data
Ether-A-Go-Go Potassium Channels
Exercise / physiology*
Exercise Test / methods,  statistics & numerical data
Female
Heart Ventricles / physiopathology
Humans
KCNQ Potassium Channels
KCNQ1 Potassium Channel
Long QT Syndrome / diagnosis,  genetics,  physiopathology*
Male
Middle Aged
Mutation / genetics,  physiology
Potassium Channels / genetics,  physiology*
Potassium Channels, Voltage-Gated*
Sinoatrial Node / physiopathology*
Statistics, Nonparametric
Trans-Activators*
Chemical
Reg. No./Substance:
0/Cation Transport Proteins; 0/DNA-Binding Proteins; 0/ERG protein, human; 0/ERG1 potassium channel; 0/Ether-A-Go-Go Potassium Channels; 0/KCNH6 protein, human; 0/KCNQ Potassium Channels; 0/KCNQ1 Potassium Channel; 0/KCNQ1 protein, human; 0/Potassium Channels; 0/Potassium Channels, Voltage-Gated; 0/Trans-Activators

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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