Value of the “Standing Test” in the Diagnosis and Evaluation of Beta-blocker Therapy Response in Long QT Syndrome

Muñoz‐Esparza, Carmen; Zorio, Esther; Valero, D. Domingo; Peñafiel-Verdú, Pablo; Sánchez-Muñoz, Juan J.; García-Molina, Esperanza; Sabater, María; Navarro, Marina; San-Román, Irene; Pérez, Inmaculada; Santos, Juan José Arranz; Cabañas-Perianes, Valentín; Valdés, Mariano; Pascual, Domingo; García‐Alberola, Arcadio; Blanes, Juan Ramón Gimeno

doi:10.1016/j.rec.2017.01.006

Cited by 6 publications

(20 citation statements)

References 23 publications

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“…In this variant of the bedside stand-up test, only 2 measures of the QTc are performed, before standing (QTc supine) and within 10seconds after standing (QTc standing). In their cohort, Muñoz-Esparza et al 1 also found that QTc standing was significantly longer in the LQTS group than in controls (528 ± 46 vs 420 ± 15ms; P < .001). This simplified version of the bedside stand-up test was thus confirmed to have diagnostic utility and a cutoff of 475ms for QTc standing demonstrated a 90% sensitivity and 100% specificity in differentiating LQTS cases from controls.…”

Section: Strategies To Detect Qt Prolongation: Stand Up!mentioning

confidence: 73%

“…Receiver-operating characteristic curves showed that the test added diagnostic value compared with the baseline supine QTc. In the article by Muñoz-Esparza et al 1 the authors propose a "simplified" version of the Viskin's protocol to evaluate the response of the QT interval to standing in 36 LQTS patients (81% of whom had genetic confirmation) compared with 41 age-and sex-matched controls. In this variant of the bedside stand-up test, only 2 measures of the QTc are performed, before standing (QTc supine) and within 10seconds after standing (QTc standing).…”

Section: Strategies To Detect Qt Prolongation: Stand Up!mentioning

confidence: 99%

“…

In an original article recently published in Revista Española de Cardiología, Muñoz-Esparza et al 1 report their observations on the value of the "stand-up" test in the diagnosis of long QT syndrome (LQTS) and its usefulness in guiding patient management. The topic is of interest, because in the clinical setting, the evaluation of patients with borderline prolongation of the corrected QT interval is one of the most common issues faced by the cardiologists when assessing family members of index cases affected by LQTS.

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confidence: 99%

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Diagnosis of Long QT Syndrome: Time to Stand Up!

Mazzanti

Priori

2017

Revista Española de Cardiología (English Edition)

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Section: Strategies To Detect Qt Prolongation: Stand Up!mentioning

confidence: 73%

Section: Strategies To Detect Qt Prolongation: Stand Up!mentioning

confidence: 99%

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confidence: 99%

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Diagnosis of Long QT Syndrome: Time to Stand Up!

Mazzanti

Priori

2017

Revista Española de Cardiología (English Edition)

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“…Схожие данные были получены группой испанских авторов в 2017 году [12]. Было показано, что значения интервала QTс в ортостазе являются лучшим диагностическим маркером СУИQT, чем продолжительность интервала QTс на стандартной ЭКГ, при 90 %-ной чувствительности, специфичность составила соответственно 100 и 58 %.…”

Section: Discussionunclassified

Changing QT interval in orthostasis — new diagnostic marker of syndrome of extended QT interval

Комолятова¹,

Макаров²,

Киселева³

et al. 2019

Medicinskij alfavit

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Long QT interval syndrome (LQTS) is a disease that causes life-threatening arrhythmias and sudden death syndrome. Recently, it has been shown that about half of patients with LQTS have normal QTc interval values on an ECG. The purpose of this study was to assess changes in the QT interval in patients with LQTS in orthostasis to identify additional criteria for the disease. Surveyed 64 patients from 4 to 47 years old (13.3 ± 10.0) with LQTIS. The control group consisted of 87 healthy people of 4–20 years (11.0 ± 3.5). An ECG was performed in a lying and standing position. HR, QT and QTc interrvals in both positions and the difference between these indicators were evaluated. In both groups, we noted an increase in heart rate and QTc and a shortening of the QT interval in orthostasis. Distinctions between all parameters were revealed reliably, except for the increase in the QTc interval (∆QTc) in the main and control groups. ROC analysis revealed that the optimal point for identifying patients with LQTS is QTc interval in orthostasis –500 ms with Se 73 % and Sp 93 %. Conclusions. In the early period of orthostasis, an increase in the heart rate, a shortening of the QT interval and a prolongation of the QTc interval occur; lengthening the QTc interval in the early period of orthostasis over 500 ms is highly sensitive and highly specific in identifying patients with the syndrome of prolonged QT-interval.

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“…However, genetic testing also has limitations, such as a sensitivity of 75‐80% for known LQTS gene mutations, high cost, and limited availability to all patients depending on their health insurance 4 . In order to diagnose LQTS in those individuals with a “concealed” phenotype, postural change in QTc (QT comparison in rest and standing), 5–7 QTc prolongation during and after exercise, and QT hysteresis (QT interval difference between exercise and 2 minutes into recovery phase) have been used 5 . The utility of exercise stress testing in children with “concealed” LQTS and its correlation with genetic studies is limited.…”

Section: Introductionmentioning

confidence: 99%

Treadmill exercise testing improves diagnostic accuracy in children with concealed congenital long QT syndrome

Patel

Kamande

Jarosz

et al. 2020

Pacing Clinical Electrophis

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Background Resting electrocardiogram (ECG) identification of long QT syndrome (LQTS) has limitations. Uncertainty exists on how to classify patients with borderline prolonged QT intervals. We tested if exercise testing could help serve to guide which children with borderline prolonged QT intervals may be gene positive for LQTS. Methods Pediatric patients (n = 139) were divided into three groups: Controls (n = 76), gene positive LQTS with borderline QTc (n = 21), and gene negative patients with borderline QTc (n = 42). Borderline QTc was defined between 440‐470 (male) and 440‐480 (female) ms. ECGs were recorded supine, sitting, and standing. Patients then underwent treadmill stress testing with Bruce protocol followed by a 9‐minute recovery phase. Results Supine resting QTc, age, and Schwartz score for the three groups were: (a) gene positive: 446 ± 23 ms, 12.4 ± 3.4 years old, 3.2 ± 1.8; (b) gene negative: 445 ± 20 ms, 12.1 ± 2 years old, 2.0 ± 1.2; and (c) control: 400 ± 24 ms, 15.0 ± 3 years old. The three groups could be differentiated by their QTc response at two time points: standing and recovery phase at 6 minutes. Standing QTc ≥460 ms differentiated borderline prolonged QTc patients (gene positive and gene negative) from controls. Late recovery QTc ≥480 ms distinguished gene positive from gene negative patients. Conclusion Exercise stress testing can be useful to identify children who are gene positive borderline LQTS from a normal population and gene negative borderline QTc children, allowing for selective gene testing in a higher risk group of patients with borderline QTc intervals and intermediate Schwartz scores.

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Value of the “Standing Test” in the Diagnosis and Evaluation of Beta-blocker Therapy Response in Long QT Syndrome

Cited by 6 publications

References 23 publications

Diagnosis of Long QT Syndrome: Time to Stand Up!

Diagnosis of Long QT Syndrome: Time to Stand Up!

Changing QT interval in orthostasis — new diagnostic marker of syndrome of extended QT interval

Treadmill exercise testing improves diagnostic accuracy in children with concealed congenital long QT syndrome

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