Myocardial Scarring and Sudden Cardiac Death in Young Patients with Hypertrophic Cardiomyopathy: A Multicenter Cohort Study
Issued Date
2024-11-13
Resource Type
ISSN
23806583
eISSN
23806591
Scopus ID
2-s2.0-85209155807
Pubmed ID
39320884
Journal Title
JAMA Cardiology
Volume
9
Issue
11
Rights Holder(s)
SCOPUS
Bibliographic Citation
JAMA Cardiology Vol.9 No.11 (2024)
Suggested Citation
Chan R.H., Van Der Wal L., Liberato G., Rowin E., Soslow J., Maskatia S., Chan S., Shah A., Fogel M., Hernandez L., Anwar S., Voges I., Carlsson M., Buddhe S., Laser K.T., Greil G., Valsangiacomo-Buechel E., Olivotto I., Wong D., Wolf C., Grotenhuis H., Rickers C., Hor K., Rutz T., Kutty S., Samyn M., Johnson T., Hasbani K., Moore J.P., Sieverding L., Detterich J., Parra R., Chungsomprasong P., Toro-Salazar O., Roest A.A.W., Dittrich S., Brun H., Spinner J., Lai W., Dyer A., Jablonowsk R., Meierhofer C., Gabbert D., Prsa M., Patel J.K., Hornung A., Diab S.G., House A.V., Rakowski H., Benson L., Maron M.S., Grosse-Wortmann L. Myocardial Scarring and Sudden Cardiac Death in Young Patients with Hypertrophic Cardiomyopathy: A Multicenter Cohort Study. JAMA Cardiology Vol.9 No.11 (2024). doi:10.1001/jamacardio.2024.2824 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/102134
Title
Myocardial Scarring and Sudden Cardiac Death in Young Patients with Hypertrophic Cardiomyopathy: A Multicenter Cohort Study
Author(s)
Chan R.H.
Van Der Wal L.
Liberato G.
Rowin E.
Soslow J.
Maskatia S.
Chan S.
Shah A.
Fogel M.
Hernandez L.
Anwar S.
Voges I.
Carlsson M.
Buddhe S.
Laser K.T.
Greil G.
Valsangiacomo-Buechel E.
Olivotto I.
Wong D.
Wolf C.
Grotenhuis H.
Rickers C.
Hor K.
Rutz T.
Kutty S.
Samyn M.
Johnson T.
Hasbani K.
Moore J.P.
Sieverding L.
Detterich J.
Parra R.
Chungsomprasong P.
Toro-Salazar O.
Roest A.A.W.
Dittrich S.
Brun H.
Spinner J.
Lai W.
Dyer A.
Jablonowsk R.
Meierhofer C.
Gabbert D.
Prsa M.
Patel J.K.
Hornung A.
Diab S.G.
House A.V.
Rakowski H.
Benson L.
Maron M.S.
Grosse-Wortmann L.
Van Der Wal L.
Liberato G.
Rowin E.
Soslow J.
Maskatia S.
Chan S.
Shah A.
Fogel M.
Hernandez L.
Anwar S.
Voges I.
Carlsson M.
Buddhe S.
Laser K.T.
Greil G.
Valsangiacomo-Buechel E.
Olivotto I.
Wong D.
Wolf C.
Grotenhuis H.
Rickers C.
Hor K.
Rutz T.
Kutty S.
Samyn M.
Johnson T.
Hasbani K.
Moore J.P.
Sieverding L.
Detterich J.
Parra R.
Chungsomprasong P.
Toro-Salazar O.
Roest A.A.W.
Dittrich S.
Brun H.
Spinner J.
Lai W.
Dyer A.
Jablonowsk R.
Meierhofer C.
Gabbert D.
Prsa M.
Patel J.K.
Hornung A.
Diab S.G.
House A.V.
Rakowski H.
Benson L.
Maron M.S.
Grosse-Wortmann L.
Author's Affiliation
Siriraj Hospital
Matematikcentrum
Institutionen för Kliniska Vetenskaper, Lund
Peter Munk Cardiac Centre
Universitäre Herz- und Gefäßzentrum UKE Hamburg GmbH
Oslo Universitetssykehus
Joe DiMaggio Children's Hospital
Nationwide Children’s Hospital
Wilhelmina Kinderziekenhuis
UCSF Benioff Children‘s Hospital
Lucile Packard Children's Hospital Stanford
Ronald Reagan UCLA Medical Center
UCSF School of Medicine
Vanderbilt University Medical Center
The Hospital for Sick Children
The Children's Hospital of Philadelphia
University of Oklahoma Health Sciences Center
Pontificia Universidad Católica de Chile
College of Medicine
Herz- und Diabeteszentrum Nordrhein-Westfalen Universitätsklinik der Ruhr-Universität Bochum
Columbia University Irving Medical Center
UT Southwestern Medical Center
Centre Hospitalier Universitaire Vaudois
Universitätsklinikum und Medizinische Fakultät Tübingen
Indiana University School of Medicine
Azienda Ospedaliera Careggi
Lahey Hospital & Medical Center
Children's Hospital Los Angeles
Connecticut Children's Medical Center
Technische Universität München
Children's Mercy Hospitals and Clinics
Children's Hospital of Eastern Ontario, Ottawa
Doernbecher Children's Hospital
Leids Universitair Medisch Centrum
University of Washington
Keck School of Medicine of USC
The University of Texas at Austin
Medical College of Wisconsin
Rijksuniversiteit Groningen
Universitätsklinikum Gießen und Marburg, Standort Gießen
Universidade de São Paulo
CHOC Children‘s UC Irvine School of Medicine
Universitätsklinikum Schleswig-Holstein Campus Kiel
Kinderspital Zürich
Baylor College of Medicine
The Johns Hopkins Hospital
Friedrich-Alexander-Universität Erlangen-Nürnberg
Meyer Children's Hospital IRCCS
Cook Children's Medical Center
Matematikcentrum
Institutionen för Kliniska Vetenskaper, Lund
Peter Munk Cardiac Centre
Universitäre Herz- und Gefäßzentrum UKE Hamburg GmbH
Oslo Universitetssykehus
Joe DiMaggio Children's Hospital
Nationwide Children’s Hospital
Wilhelmina Kinderziekenhuis
UCSF Benioff Children‘s Hospital
Lucile Packard Children's Hospital Stanford
Ronald Reagan UCLA Medical Center
UCSF School of Medicine
Vanderbilt University Medical Center
The Hospital for Sick Children
The Children's Hospital of Philadelphia
University of Oklahoma Health Sciences Center
Pontificia Universidad Católica de Chile
College of Medicine
Herz- und Diabeteszentrum Nordrhein-Westfalen Universitätsklinik der Ruhr-Universität Bochum
Columbia University Irving Medical Center
UT Southwestern Medical Center
Centre Hospitalier Universitaire Vaudois
Universitätsklinikum und Medizinische Fakultät Tübingen
Indiana University School of Medicine
Azienda Ospedaliera Careggi
Lahey Hospital & Medical Center
Children's Hospital Los Angeles
Connecticut Children's Medical Center
Technische Universität München
Children's Mercy Hospitals and Clinics
Children's Hospital of Eastern Ontario, Ottawa
Doernbecher Children's Hospital
Leids Universitair Medisch Centrum
University of Washington
Keck School of Medicine of USC
The University of Texas at Austin
Medical College of Wisconsin
Rijksuniversiteit Groningen
Universitätsklinikum Gießen und Marburg, Standort Gießen
Universidade de São Paulo
CHOC Children‘s UC Irvine School of Medicine
Universitätsklinikum Schleswig-Holstein Campus Kiel
Kinderspital Zürich
Baylor College of Medicine
The Johns Hopkins Hospital
Friedrich-Alexander-Universität Erlangen-Nürnberg
Meyer Children's Hospital IRCCS
Cook Children's Medical Center
Corresponding Author(s)
Other Contributor(s)
Abstract
Importance: The ability to predict sudden cardiac death (SCD) in children and adolescents with hypertrophic cardiomyopathy (HCM) is currently inadequate. Late gadolinium enhancement (LGE) by cardiovascular magnetic resonance (CMR) imaging is associated with SCD events in adults with HCM. Objective: To examine the prognostic significance of LGE in patients with HCM who are younger than 21 years. Design, Setting, and Participants: This multicenter, retrospective cohort study was conducted from April 8, 2015, to September 12, 2022, in patients with HCM who were younger than 21 years and had undergone CMR imaging across multiple sites in the US, Europe, and South America. Observers of CMR studies were masked toward outcomes and demographic characteristics. Exposure: Natural history of HCM. Main Outcome and Measures: The primary outcome was SCD and surrogate events, including resuscitated cardiac arrest and appropriate discharges from an implantable defibrillator. Continuous and categorical data are expressed as mean (SD), median (IQR), or number (percentage), respectively. Survivor curves comparing patients with and without LGE were constructed by the Kaplan-Meier method, and likelihood of subsequent clinical events was further evaluated using univariate and multivariable Cox proportional hazards models. Results: Among 700 patients from 37 international centers, median (IQR) age was 14.8 (11.9-17.4) years, and 518 participants (74.0%) were male. During a median (IQR) [range] follow-up period of 1.9 (0.5-4.1) [0.1-14.8] years, 35 patients (5.0%) experienced SCD or equivalent events. LGE was present in 230 patients (32.9%), which constituted an mean (SD) burden of 5.9% (7.3%) of left ventricular myocardium. The LGE amount was higher in older patients and those with greater left ventricular mass and maximal wall thickness; patients with LGE had lower left ventricular ejection fractions and larger left atrial diameters. The presence and burden of LGE was associated with SCD, even after correcting for existing risk stratification tools. Patients with 10% or more LGE, relative to total myocardium, had a higher risk of SCD (unadjusted hazard ratio [HR], 2.19; 95% CI, 1.59-3.02; P <.001). Furthermore, the addition of LGE burden improved the performance of the HCM Risk-Kids score (before LGE addition: 0.66; 95% CI, 0.58-0.75; after LGE addition: 0.73; 95% CI, 0.66-0.81) and Precision Medicine in Cardiomyopathy score (before LGE addition: 0.68; 95% CI, 0.49-0.77; after LGE addition: 0.73; 95% CI, 0.64-0.82) SCD predictive models. Conclusions and Relevance: In this retrospective cohort study, quantitative LGE was a risk factor for SCD in patients younger than 21 years with HCM and improved risk stratification.