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MYBPC3 hypertrophic cardiomyopathy can be detected by using advanced ECG in children and young adults
Region Östergötland, Center of Paediatrics and Gynaecology and Obstetrics, Department of Paediatrics in Linköping. Skåne University Hospital, Sweden; Lund University, Sweden.
Skåne University Hospital, Sweden; Lund University, Sweden.
Lund University, Sweden; Skåne University Hospital, Sweden.
Lund University, Sweden; Skåne University Hospital, Sweden.
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2016 (English)In: Journal of Electrocardiology, ISSN 0022-0736, E-ISSN 1532-8430, Vol. 49, no 3, 392-400 p.Article in journal (Refereed) PublishedText
Abstract [en]

Introduction: The conventional ECG is commonly used to screen for hypertrophic cardiomyopathy (HCM), but up to 25% of adults and possibly larger percentages of children with HCM have no distinctive abnormalities on the conventional ECG, whereas 5 to 15% of healthy young athletes do. Recently, a 5-min resting advanced 12-lead ECG test ("A-ECG score") showed superiority to pooled criteria from the strictly conventional ECG in correctly identifying adult HCM. The purpose of this study was to evaluate whether in children and young adults, A-ECG scoring could detect echocardiographic HCM associated with the MYBPC3 genetic mutation with greater sensitivity than conventional ECG criteria and distinguish healthy young controls and athletes from persons with MYBPC3 HCM with greater specificity. Methods: Five-minute 12-lead ECGs were obtained from 15 young patients (mean age 13.2 years, range 0-30 years) with MYBPC3 mutation and phenotypic HCM. The conventional and A-ECG results of these patients were compared to those of 198 healthy children and young adults (mean age 13.2, range 1 month-30 years) with unremarkable echocardiograms, and to those of 36 young endurance-trained athletes, 20 of whom had athletic (physiologic) left ventricular hypertrophy. Results: Compared with commonly used, age-specific pooled criteria from the conventional ECG, a retrospectively generated A-ECG score incorporating results from just 2 derived vectorcardiographic parameters (spatial QRS-T angle and the change in the vectorcardiographic QRS azimuth angle from the second to the third eighth of the QRS interval) increased the sensitivity of ECG for identifying MYBPC3 HCM from 46% to 87% (p amp;lt; 0.05). Use of the same score also demonstrated superior specificity in a set of 198 healthy controls (94% vs. 87% for conventional ECG criteria; p amp;lt; 0.01) including in a subset of 36 healthy, young endurance-trained athletes (100% vs. 69% for conventional ECG criteria, p amp;lt; 0.001). Conclusions: In children and young adults, a 2-parameter 12-lead A-ECG score is retrospectively significantly more sensitive and specific than pooled, age-specific conventional ECG criteria for detecting MYBPC3-HCM and in distinguishing such patients from healthy controls, including endurance-trained athletes. (C) 2016 Elsevier Inc. All rights reserved.

Place, publisher, year, edition, pages
Keyword [en]
Sudden cardiac death; Spatial QRS-T angle; Vectorcardiogram
National Category
Cardiac and Cardiovascular Systems
URN: urn:nbn:se:liu:diva-129172DOI: 10.1016/j.jelectrocard.2016.03.001ISI: 000376053100019PubMedID: 27061026OAI: diva2:936016

Funding Agencies|Lundgren foundation Malmo; Samariten foundation Stockholm; FORSS; Medical Research Council of Southeast Sweden; SUS foundations and donations

Available from: 2016-06-13 Created: 2016-06-13 Last updated: 2016-06-22

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Fernlund, Eva
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Department of Paediatrics in Linköping
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