Value of Holter Monitoring in Patients with the Long QT Syndrome

Eggeling, Thomas; Osterhues, H.-H.; Hoeher, Martin; Gabrielsen, F. G.; Weismueller, Peter; Hombach, Vinzenz

doi:10.1159/000175784

Cited by 23 publications

(10 citation statements)

References 4 publications

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“…As has been demonstrated in adult patients with congenital LQTS, approximately one third of these patients show abnormalities, such as torsades de pointes, T-wave alternans, or bradycardia during a 24-h period [10]. Approximately 50% of children with congenital LQTS and a history of syncopal attacks showed ventricular extrasystoles during 24-h ECG recordings [15].…”

Section: Discussionmentioning

confidence: 99%

QTc interval prolongation in children with Turner syndrome: the results of exercise testing and 24-h ECG

et al. 2008

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None of the patients experienced adverse cardiac events during the tests. The mean QTc interval decreased from 0.467 to 0.432 s in group 1 and from 0.432 to 0.412 s in group 2. During the 24-h ECG, the maximum QTc interval was significantly prolonged in group 1 (0.51 vs. 0.465 s, p<0.05, respectively). We conclude that exercise testing and 24-h ECG recording provide valuable information about the cardiac risk in the single TS patient with a prolonged QTc interval. This helps in counseling these girls, as clear therapeutic guidelines are currently lacking.

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Section: Discussionmentioning

confidence: 99%

QTc interval prolongation in children with Turner syndrome: the results of exercise testing and 24-h ECG

et al. 2008

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“…A blunted heart rate acceleration was observed in our LQTS patients as compared to the control group. Sinus rate response in LQTS patients is controversial with some studies demonstrating sinus node impairment especially in LQT1 patients (25-27). Due to the possibility of blunted heart rate acceleration in LQTS patients, postural QT rather than QTc change should be assessed in patients evaluated for LQTS.…”

Section: Discussionmentioning

confidence: 99%

Genotype- and Mutation Site–Specific QT Adaptation During Exercise, Recovery, and Postural Changes in Children With Long-QT Syndrome

Aziz

Wieand

Ganley

et al. 2011

Circ: Arrhythmia and Electrophysiology

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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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“…The ECG remains the primary screening tool for LQT. Other techniques used have included T-wave alternans [24], RR interval variability, QT duration [25], T-wave morphology analysis [26], echocardiographic wall motion findings [27], QT dispersion [28], Holter monitoring [29], exercise testing, and provocative drug infusions [30]. Kaufmann et al [24] published their experience studying 101 family members of a proband with congenital LQT from a HERG mutation.…”

Section: Identification Of Carriers Of Abnormal Genesmentioning

confidence: 99%

Genetic basis for the origin of cardiac arrhythmias: Implications for therapy

et al. 2002

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The recognition of the role that genetic abnormalities play in the generation of cardiac arrhythmias and sudden cardiac death has evolved enormously over the past decade. One result is new insight into underlying physiologic and pathophysiologic mechanisms. New therapies based on this evolving insight are being developed. This review summarizes recent discoveries with a focus on the genetic basis of cardiac arrhythmias and their implications for new therapies.

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Value of Holter Monitoring in Patients with the Long QT Syndrome

Cited by 23 publications

References 4 publications

QTc interval prolongation in children with Turner syndrome: the results of exercise testing and 24-h ECG

QTc interval prolongation in children with Turner syndrome: the results of exercise testing and 24-h ECG

Genotype- and Mutation Site–Specific QT Adaptation During Exercise, Recovery, and Postural Changes in Children With Long-QT Syndrome

Genetic basis for the origin of cardiac arrhythmias: Implications for therapy

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