| Genotype and Mutation Site Specific QT Adaptation during Exercise, Recovery and Postural Changes in Children with LQTS. | |
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MedLine Citation:
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PMID: 21956039 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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BACKGROUND: -Exercise stress testing has shown diagnostic utility in adult patients with long QT syndrome (LQTS). However, the QT interval adaptation in response to exercise in pediatric patients with LQTS has received little attention. METHODS AND RESULTS: -One-hundred and fifty eight patients were divided into three groups: LQT1, LQT2 and normal controls without cardiovascular disease. Each patient underwent a uniform exercise protocol employing a cycle ergometer followed by a 9 minute recovery phase with continuous 12-lead electrocardiogram (ECG) monitoring. Each patient underwent a baseline electrocardiogram while resting in the supine position and in a stand still position during continuous ECG recording for determining changes in the QT and RR intervals. Fifty patients were gene-positive for LQTS (n=29, LQT1 and n=21, LQT2) and the control group consisted of 108 patients. QT interval adaptation was abnormal in the LQT1 patients compared to LQT2 and control patients (P<0.001). A QTc >460 ms in the late recovery phase at 7 minutes predicted LQT1 or LQT2 vs. controls with 96% specificity, 86% sensitivity and 91% positive predictive value. A recovery ΔQTc ((7 minute - 1 minute)) >30 ms predicted LQT2 vs. LQT1 with 75% sensitivity, 82% specificity and 75% positive predictive value. The postural ΔQT was significantly different between LQTS and control groups (P=0.005). CONCLUSIONS: -Genotype specific changes in repolarization response to exercise and recovery exist in the pediatric population and are of diagnostic utility in LQTS. An extended recovery phase is preferable to assess the repolarization response after exercise in the pediatric population. |
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Authors:
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Peter F Aziz; Tammy S Wieand; Jamie Ganley; Jacqueline Henderson; Akash R Patel; V Ramesh Iyer; R Lee Vogel; Michael McBride; Victoria L Vetter; Maully J Shah |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-9-28 |
Journal Detail:
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Title: Circulation. Arrhythmia and electrophysiology Volume: - ISSN: 1941-3084 ISO Abbreviation: - Publication Date: 2011 Sep |
Date Detail:
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Created Date: 2011-9-29 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101474365 Medline TA: Circ Arrhythm Electrophysiol Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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The Children's Hospital of Philadelphia & Univ of Pennsylvania School of Med, Philadelphia, PA. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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