@article{HCMR:2026:10.1001/jama.2026.5633,
author = {HCMR, Investigators and Kramer, CM and Kolm, P and DiMarco, JP and Desai, MY and Ho, CY and Kwong, RY and Dolman, SF and Desvigne-Nickens, P and Geller, N and Kim, D-Y and Schulz-Menger, J and Friedrich, MG and Maron, MS and Appelbaum, E and Link, MS and Francis, GS and Greenberg, B and Jerosch-Herold, M and Piechnik, S and Mahmod, M and Raman, B and Jacoby, DL and Baldassare, LA and White, JA and Chiribiri, A and Helms, AS and Choudhury, L and Michels, M and Bradlow, WM and Salerno, M and Heitner, SB and Masri, A and Prasad, SK and Mohiddin, SA and Plein, S and Madias, C and Mahrholdt, H and Bucciarelli-Ducci, C and Nightingale, AK and Weinsaft, JW and Kim, HW and McCann, GP and van, Rossum A and Germans, T and Williamson, EE and Geske, JB and Flett, AS and Dawson, D and Mongeon, F-P and Olivotto, I and Crean, AM and Woo, A and Owens, AT and Anderson, L and Sharma, S and Biagini, E and Newby, DE and Andre, F and Berry, C and Kim, B and Larose, E and Abraham, TP and Hays, AG and Sherri},
doi = {10.1001/jama.2026.5633},
journal = {JAMA},
title = {Predictors of Long-Term Outcomes in Hypertrophic Cardiomyopathy: The NHLBI HCM Registry.},
url = {http://dx.doi.org/10.1001/jama.2026.5633},
year = {2026}
}

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TY  - JOUR
AB  - IMPORTANCE: Current risk prediction guidelines for hypertrophic cardiomyopathy predict only sudden cardiac death and are imperfect, leading to avoidable deaths and unnecessary implantable cardioverter defibrillators. OBJECTIVE: To combine prospectively collected clinical history, imaging, genetic, and biomarker data to improve risk prediction of adverse events in hypertrophic cardiomyopathy. DESIGN, SETTING, AND PARTICIPANTS: A total of 2750 patients with hypertrophic cardiomyopathy were prospectively enrolled in the registry-based study from 44 sites in North America and Europe with expertise in hypertrophic cardiomyopathy and cardiac magnetic resonance (CMR) imaging. Participants were enrolled from April 1, 2014, to April 7, 2017. EXPOSURES: Patients underwent a health history questionnaire, blood sampling for biomarkers and genotyping, and contrast-enhanced CMR. Patients were followed up yearly by telephone and through records review regarding event documentation. MAIN OUTCOMES AND MEASURES: The predefined composite adjudicated primary end point was time to first event for hypertrophic cardiomyopathy-related deaths; nonfatal sustained ventricular arrhythmias (VAs) requiring cardioversion or defibrillation; and left ventricular (LV) assist device implant or heart transplant. A secondary end point was a composite of sudden cardiac death and nonfatal VA events. The elastic-net method identified the most important predictors. Cox proportional hazards regression assessed associations with time to the first end point. RESULTS: Of the 2750 prospectively enrolled patients, 2698 (98%) had analyzable data after 9 were excluded because they had hypertrophic cardiomyopathy phenocopies and 43 withdrew. Of these remaining patients, 1919 (71%) were male, mean age was 50 years (SD, 11 years), and 423 (16%) were from underrepresented racial and minority groups. The mean follow-up was 6.9 years (SD, 2.1 years). The primary event model in 104 patients included LV scar as a percenta
AU  - HCMR,Investigators
AU  - Kramer,CM
AU  - Kolm,P
AU  - DiMarco,JP
AU  - Desai,MY
AU  - Ho,CY
AU  - Kwong,RY
AU  - Dolman,SF
AU  - Desvigne-Nickens,P
AU  - Geller,N
AU  - Kim,D-Y
AU  - Schulz-Menger,J
AU  - Friedrich,MG
AU  - Maron,MS
AU  - Appelbaum,E
AU  - Link,MS
AU  - Francis,GS
AU  - Greenberg,B
AU  - Jerosch-Herold,M
AU  - Piechnik,S
AU  - Mahmod,M
AU  - Raman,B
AU  - Jacoby,DL
AU  - Baldassare,LA
AU  - White,JA
AU  - Chiribiri,A
AU  - Helms,AS
AU  - Choudhury,L
AU  - Michels,M
AU  - Bradlow,WM
AU  - Salerno,M
AU  - Heitner,SB
AU  - Masri,A
AU  - Prasad,SK
AU  - Mohiddin,SA
AU  - Plein,S
AU  - Madias,C
AU  - Mahrholdt,H
AU  - Bucciarelli-Ducci,C
AU  - Nightingale,AK
AU  - Weinsaft,JW
AU  - Kim,HW
AU  - McCann,GP
AU  - van,Rossum A
AU  - Germans,T
AU  - Williamson,EE
AU  - Geske,JB
AU  - Flett,AS
AU  - Dawson,D
AU  - Mongeon,F-P
AU  - Olivotto,I
AU  - Crean,AM
AU  - Woo,A
AU  - Owens,AT
AU  - Anderson,L
AU  - Sharma,S
AU  - Biagini,E
AU  - Newby,DE
AU  - Andre,F
AU  - Berry,C
AU  - Kim,B
AU  - Larose,E
AU  - Abraham,TP
AU  - Hays,AG
AU  - Sherrid,MV
AU  - Gelfand,EV
AU  - Nagueh,SF
AU  - Rimoldi,O
AU  - Camici,P
AU  - Elstein,E
AU  - Autore,C
AU  - Watkins,H
AU  - Weintraub,WS
AU  - Neubauer,S
DO  - 10.1001/jama.2026.5633
PY  - 2026///
TI  - Predictors of Long-Term Outcomes in Hypertrophic Cardiomyopathy: The NHLBI HCM Registry.
T2  - JAMA
UR  - http://dx.doi.org/10.1001/jama.2026.5633
UR  - https://www.ncbi.nlm.nih.gov/pubmed/42113540
ER  - 

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