JAMA

Review of Head and Neck Cancer

5/10/2026 Source: JAMA

Summary

print. Predictors of Long-Term Outcomes in Hypertrophic Cardiomyopathy: The NHLBI HCM Registry. HCMR Investigators; Kramer CM(1), Kolm P(2), DiMarco JP(1), Desai MY(3), Ho CY(4), Kwong RY(4), Dolman SF(2), Desvigne-Nickens P(5), Geller N(5), Kim DY(5), Schulz-Menger J(6), Friedrich MG(7), Maron MS(8), Appelbaum E(9), Link MS(10), Francis GS(11), Greenberg B(12), Jerosch-Herold M(4), Piechnik S(13), Mahmod M(13), Raman B(13), Jacoby DL(14), Baldassare LA(15), White JA(16), Chiribiri A(17), Helms

Content

# Review of Head and Neck Cancer *Published: 2026 May 11* print. Predictors of Long-Term Outcomes in Hypertrophic Cardiomyopathy: The NHLBI HCM Registry. HCMR Investigators; Kramer CM(1), Kolm P(2), DiMarco JP(1), Desai MY(3), Ho CY(4), Kwong RY(4), Dolman SF(2), Desvigne-Nickens P(5), Geller N(5), Kim DY(5), Schulz-Menger J(6), Friedrich MG(7), Maron MS(8), Appelbaum E(9), Link MS(10), Francis GS(11), Greenberg B(12), Jerosch-Herold M(4), Piechnik S(13), Mahmod M(13), Raman B(13), Jacoby DL(14), Baldassare LA(15), White JA(16), Chiribiri A(17), Helms AS(18), Choudhury L(19), Michels M(20), Bradlow WM(21), Salerno M(22), Heitner SB(14), Masri A(23), Prasad SK(24), Mohiddin SA(25), Plein S(26), Madias C(27), Mahrholdt H(28), Bucciarelli-Ducci C(24), Nightingale AK(29), Weinsaft JW(30), Kim HW(31), McCann GP(32), van Rossum A(33), Germans T(33), Williamson EE(34), Geske JB(34), Flett AS(35), Dawson D(36), Mongeon FP(37), Olivotto I(38), Crean AM(39), Woo A(40), Owens AT(41), Anderson L(42), Sharma S(42), Biagini E(43), Newby DE(44), Andre F(45), Berry C(46), Kim B(47), Larose E(48), Abraham TP(22), Hays AG(49), Sherrid MV(47), Gelfand EV(50), Nagueh SF(51), Rimoldi O(52), Camici P(52), Elstein E(7), Autore C(53), Watkins H(13), Weintraub WS(2), Neubauer S(13). Author information: (1)University of Virginia Health, Charlottesville. (2)MedStar Research Institute, Georgetown University, Washington, DC. (3)Cleveland Clinic, Cleveland, Ohio. (4)Brigham and Women's Hospital, Boston, Massachusetts. (5)National Heart, Lung, and Blood Institute, Bethesda, Maryland. (6)Charite´ Experimental Clinical Research Center and Helios Clinics Berlin-Buch, Berlin, Germany. (7)McGill University, Montreal, Quebec, Canada. (8)Lahey Clinic, Burlington, Massachusetts. (9)Men's Health Center, Boston, Massachusetts. (10)University of Texas, Southwestern, Dallas. (11)University of Minnesota, Minneapolis. (12)University of California, San Diego. (13)University of Oxford, Oxford, United Kingdom. (14)Cytokinetics, Inc, South San Francisco, California. (15)Yale University, New Haven, Connecticut. (16)University of Calgary, Alberta, Canada. (17)King's College, London, London, United Kingdom. (18)University of Michigan, Ann Arbor. (19)Northwestern University, Chicago, Illinois. (20)Erasmus University, Rotterdam, the Netherlands. (21)Queen Elizabeth's Hospital, Birmingham, United Kingdom. (22)University of California, San Francisco. (23)Oregon Health Sciences University, Portland. (24)Royal Brompton Hospital, London, United Kingdom. (25)London Chest Hospital, London, United Kingdom. (26)University of Leeds, Leeds, United Kingdom. (27)Tufts New England Medical Center, Boston, Massachusetts. (28)Robert Bosch Hospital, Stuttgart, Germany. (29)University of Bristol, Bristol, United Kingdom. (30)NewYork-Presbyterian/Weill Cornell Medical Center, New York, New York. (31)Duke University Medical Center, Durham, North Carolina. (32)University of Leicester, Leicester, United Kingdom. (33)Amsterdam University Medical Center, Amsterdam, the Netherlands. (34)Mayo Clinic, Rochester, Minnesota. (35)University Hospital, Southampton, United Kingdom. (36)University of Aberdeen, Aberdeen, Scotland. (37)Montreal Heart Institute, Montreal, Quebec, Canada. (38)Careggi University Hospital, Florence, Italy. (39)University of Manchester, Manchester, United Kingdom. (40)University of Toronto, Toronto, Ontario, Canada. (41)Hospital of the University of Pennsylvania, Philadelphia. (42)St George's, University of London, London, United Kingdom. (43)University of Bologna, Bologna, Italy. (44)University of Edinburgh, Edinburgh, Scotland, United Kingdom. (45)University of Heidelberg, Heidelberg, Germany. (46)University of Glasgow, Glasgow, Scotland, United Kingdom. (47)NYU Langone Medical Center, New York, New York. (48)University Laval, Quebec City, Quebec, Canada. (49)Johns Hopkins University, Baltimore, Maryland. (50)Beth Israel Deaconess Medical Center, Boston, Massachusetts. (51)Houston Methodist, Houston, Texas. (52)San Raffaele Hospital, Milan, Italy. (53)IRCCS San Raffaele Roma, Rome, Italy. Comment in doi: 10.1001/jamacardio.2024.5677. doi: 10.1001/jamacardio.2025.4869. doi: 10.1001/jamanetworkopen.2026.9673. ## 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 percentage of LV mass by late gadolinium enhancement (LGE%; hazard ratio [HR], 1.86; 95% CI, 1.58-2.20; P < .001), LV mass index (HR, 1.09; 95% CI, 1.01-1.17; P = .03), LV end-systolic volume index (HR, 1.28; 95% CI, 1.12-1.46; P < .001 ), all per 10-unit increase, history of heart failure at study entry (HR, 2.89; 95% CI, 1.75-4.77; P < .001), and log N-terminal pro-B-type natriuretic peptide (NT-proBNP; HR, 1.41; 95% CI, 1.17-1.70; P < .001) level per log unit, (C index for all, 0.77). An LGE percentage of the LV mass of 9% or higher substantially increased the primary composite event rate (P = .001). The secondary sudden cardiac death and VA risk factor model (in 69 patients) included LGE%, LV mass index, LV ejection fraction, and log(NT-proBNP) (C index, 0.76). CONCLUSIONS AND RELEVANCE These results provide prospective evidence for incorporating cardiac magnetic resonance and NT-proBNP in the evaluation of patients with hypertrophic cardiomyopathy. ## TRIAL REGISTRATION ClinicalTrials.gov Identifier: NCT01915615. DOI: 10.1001/jama.2026.5633 PMCID: PMC13162147 DOI: 10.1001/jama.2026.3738