The Natural History of Massive Left Ventricular Hypertrophy in Pediatric Hypertrophic Cardiomyopathy: A Multiregistry Analysis

BACKGROUND: Massive left ventricular hypertrophy (LVH) is a risk factor for sudden cardiac death in children with hypertrophic cardiomyopathy (HCM), but little is understood about its natural history. METHODS: score ≥+20 at <18 years of age. Data from SHaRe and IPHCC include encounters from January 1960 through March 2024 and January 1970 through March 2024, respectively. Demographic, clinical, and serial MLVWT data were collected. Composite outcomes included major ventricular arrhythmia event (sudden cardiac death, aborted sudden cardiac death, or appropriate implantable cardioverter defibrillator therapy); heart failure (HF) event (left ventricular ejection fraction <50%, New York Heart Association class III or IV, transplant, or HF-related death); major adverse cardiac event (stroke or any major ventricular arrhythmia or HF outcome aside from left ventricular ejection fraction <50%); and HCM-related mortality (sudden cardiac death or HF-related death). Time-to-event analyses were performed using Cox proportional hazards models. RESULTS: =0.25). The last absolute MLVWT recorded was >5 mm less than the largest recorded MLVWT in 25 patients (22%). CONCLUSIONS: In pediatric HCM, massive LVH disproportionately affects those diagnosed in early childhood with sarcomeric disease, with increased risk for adverse events. Significant MLVWT regression is seen in nearly a quarter of patients.

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Journal
Circulation
Published
2026-04-17
DOI
https://doi.org/10.1161/circulationaha.126.078843
Primary Topic
Cardiomyopathy and Myosin Studies
Type
article
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article

The Natural History of Massive Left Ventricular Hypertrophy in Pediatric Hypertrophic Cardiomyopathy: A Multiregistry Analysis

Carolyn Yung Ho, Peter Kubuš, John C. Stendahl, Gabrielle Norrish et al.
Circulation
Cardiomyopathy and Myosin Studies
article

The Natural History of Massive Left Ventricular Hypertrophy in Pediatric Hypertrophic Cardiomyopathy: A Multiregistry Analysis

Carolyn Yung Ho, Peter Kubuš, John C. Stendahl, Gabrielle Norrish, Michelle Michels, James S. Ware, Francesca Girolami, Robert G. Weintraub, Sara Saberi, ANJALI TIKU OWENS, Rachel J. Lampert, Juan Pablo Kaski, Neal K. Lakdawala, Victoria Nicole Parikh, Kimberly Y. Lin, Peter-Paul Zwetsloot, Silvia Passantino, Vinay Bhole, Dominic James Abrams, Lidia Ziółkowska, Adam S. Helms, Euan Angus Ashley, Robert Przybylski, Georgia Spentzou, Cristina Ramona Rădulescu, Adrián Fernández, Sharlene M. Day, Thomas D. Ryan, Mark W. W. Russell, Grazia Delle Donne, Erin Miller, J Ingles, J Rossano, Brian L. Claggett, for the SHaRe and IPHCC Investigators, Belinda Gray, Iacopo Olivotto
article en

Abstract

BACKGROUND: Massive left ventricular hypertrophy (LVH) is a risk factor for sudden cardiac death in children with hypertrophic cardiomyopathy (HCM), but little is understood about its natural history. METHODS: score ≥+20 at <18 years of age. Data from SHaRe and IPHCC include encounters from January 1960 through March 2024 and January 1970 through March 2024, respectively. Demographic, clinical, and serial MLVWT data were collected. Composite outcomes included major ventricular arrhythmia event (sudden cardiac death, aborted sudden cardiac death, or appropriate implantable cardioverter defibrillator therapy); heart failure (HF) event (left ventricular ejection fraction <50%, New York Heart Association class III or IV, transplant, or HF-related death); major adverse cardiac event (stroke or any major ventricular arrhythmia or HF outcome aside from left ventricular ejection fraction <50%); and HCM-related mortality (sudden cardiac death or HF-related death). Time-to-event analyses were performed using Cox proportional hazards models. RESULTS: =0.25). The last absolute MLVWT recorded was >5 mm less than the largest recorded MLVWT in 25 patients (22%). CONCLUSIONS: In pediatric HCM, massive LVH disproportionately affects those diagnosed in early childhood with sarcomeric disease, with increased risk for adverse events. Significant MLVWT regression is seen in nearly a quarter of patients.

CirculationVol. 153(19)
Brigham and Women's Hospital (US), Cincinnati Children's Hospital Medical Center (US), Boston Children's Hospital (US), Leeds General Infirmary (GB), The University of Sydney (AU), Royal Children's Hospital (AU), Children's Hospital of Philadelphia (US), Harvard University (US), Garvan Institute of Medical Research (AU), Carol Davila University of Medicine and Pharmacy (RO), Cardiovascular Institute of the South (US), University of Michigan (US), Royal Prince Alfred Hospital (AU), Hospital of the University of Pennsylvania (US), Great Ormond Street Hospital (GB), Inova Fairfax Hospital (US), Bristol Royal Hospital for Children (GB), Erasmus MC (NL), Yale University (US), University Hospital in Motol (CZ), Birmingham Children's Hospital (GB), Lung Institute (US), Cancer Research And Biostatistics (US), Stanford Health Care (US), NIHR Royal Brompton Cardiovascular Biomedical Research Unit (GB), Meyer Children's Hospital (IT), Children's Memorial Health Institute (PL), Michigan Medicine (US), Stanford Medicine (US), Murdoch Children's Research Institute (AU), Favaloro Foundation (AR), University College London (GB), Erasmus University Rotterdam (NL)
Good health and well-being
Openalex Percentile: Top 27%
Cardiomyopathy and Myosin Studies
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