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Prevalence, risk factors, and outcomes of QT prolongation in primary and RASopathy-associated hypertrophic cardiomyopathy
Linköping University, Department of Biomedical and Clinical Sciences, Division of Children's and Women's Health. Linköping University, Faculty of Medicine and Health Sciences. Region Östergötland, Center of Paediatrics and Gynaecology and Obstetrics, H.K.H. Kronprinsessan Victorias barn- och ungdomssjukhus. Univ Toronto, Canada.
Hosp Sick Children, Canada.
Univ Helsinki, Finland; Helsinki Univ Hosp, Finland; Univ Helsinki, Finland; Finnish Inst Hlth & Welf, Finland.
Univ Toronto, Canada.
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2026 (English)In: Heart Rhythm, ISSN 1547-5271, E-ISSN 1556-3871, Vol. 23, no 5, p. 1179-1186Article in journal (Refereed) Published
Abstract [en]

BACKGROUND The clinical significance of QT prolongation in pediatric hypertrophic cardiomyopathy is unclear. OBJECTIVE This study aimed to determine the prevalence, risk factors, and outcomes of QT prolongation in pediatric patients with hypertrophic cardiomyopathy. METHODS Phenotype-positive, pediatric patients with primary hypertrophic cardiomyopathy (P-HCM) (n = 212) and RASopathy hypertrophic cardiomyopathy (n = 55) were included. Corrected JT (JTc) interval as a measure of QT prolongation was calculated at baseline and last follow-up. Factors associated with JTc duration were analyzed using a generalized estimating equation model. Association of JTc prolongation (JTc of > 370 ms) with risk of sudden cardiac death (SCD) events was analyzed. SCD events were defined as a composite of SCD, resuscitated SCD event, or appropriate shock from a primary prevention implantable cardioverter-defibrillator. RESULTS Notably, 24% of patients with P-HCM and 44% of patients with RASopathy hypertrophic cardiomyopathy had prolonged JTc (P = .004). JTc had only a modest correlation with the severity of left ventricular hypertrophy. In P-HCM, JTc prolongation was associated with SCD events on multivariable analysis (hazard ratio 2.9 [1.2-6.8], P = .016); 5-year SCD event-free survival from baseline evaluation was 86% in P-HCM. Including JTc as a risk factor improved the c-statistic for 5-year SCD risk prediction to 0.85 compared with 0.73 when using Precision Medicine in Cardiomyopathy risk scores alone and to 0.73 compared from 0.70 when using hypertrophic cardiomyopathy Risk-Kids scores alone. CONCLUSION JTc prolongation was independently associated with the risk of SCD events. Including JTc prolongation with SCD risk scores improved 5-year SCD risk prediction for P-HCM. This has implications for closer SCD risk monitoring in patients with P-HCM with JTc prolongation.

Place, publisher, year, edition, pages
ELSEVIER SCIENCE INC , 2026. Vol. 23, no 5, p. 1179-1186
Keywords [en]
Hypertrophic cardiomyopathy; RASopathy; QTc prolongation; JTc prolongation; Sudden cardiac death
National Category
Cardiology and Cardiovascular Disease
Identifiers
URN: urn:nbn:se:liu:diva-224087DOI: 10.1016/j.hrthm.2025.05.059ISI: 001758814600001PubMedID: 40472950Scopus ID: 2-s2.0-105009289025OAI: oai:DiVA.org:liu-224087DiVA, id: diva2:2060831
Note

Funding Agencies|Ted Rogers Centre for Heart Research; Heart and Stroke Foundation of Canada & Robert M. Freedom Chair of Cardiovascular Science; Canadian Institutes of Health Research [PJT 183820]; Swedish Heart Lung Foundation; Schelins Stiftelse, and Region Ostergotland; Svenska Sallskapet for Medicinsk Forskning, Svenska Lakaresallskapet

Available from: 2026-05-19 Created: 2026-05-19 Last updated: 2026-05-19

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Wålinder Österberg, Anna
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Division of Children's and Women's HealthFaculty of Medicine and Health SciencesH.K.H. Kronprinsessan Victorias barn- och ungdomssjukhus
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