Heart failure & cardiomyopathies

Predictors of Long-Term Outcomes in Hypertrophic Cardiomyopathy: The NHLBI HCM Registry

JAMAPublished Prospective registry-based cohort study

Authors' conclusion

These results provide prospective evidence for incorporating cardiac magnetic resonance and NT-proBNP in the evaluation of patients with hypertrophic cardiomyopathy.

Study context

Population
Patients with hypertrophic cardiomyopathy, enrolled April 1, 2014, to April 7, 20172,698 (98%) analysable
Participants
2,750 patients
Outcome
Time to first HCM-related death, nonfatal sustained VA requiring cardioversion or defibrillation, or LV assist device implant or heart transplant (adjudicated composite)
Methodological detailsmulticentre, 44 sites in North America and Europe
  • Multicentre
  • 44 sites in North America and Europe

Main finding

As reported in the abstract

Primary composite (HCM-related death, nonfatal sustained VA, LV assist device or heart transplant): multivariable predictors
Hazard ratio (95% CI)P value
LGE%per 10-unit increase
1.86(1.58–2.20)
P < 0.001
LV mass indexper 10-unit increase
1.09(1.01–1.17)
P = 0.03
LV end-systolic volume indexper 10-unit increase
1.28(1.12–1.46)
P < 0.001
History of heart failure at study entry
2.89(1.75–4.77)
P < 0.001
Log NT-proBNPper log unit
1.41(1.17–1.70)
P < 0.001

Each row per its own increment, as printed

C index for all
0.77
LGE ≥9% of left ventricular (LV) mass substantially increased primary composite event rate
P = 0.001

Observational data: associations, not effects.

Secondary outcomes

SCD and nonfatal VA composite (69 patients)

Model included LGE%, LV mass index, LVEF and log(NT-proBNP); C index
0.76

Original publication

Predictors of Long-Term Outcomes in Hypertrophic Cardiomyopathy: The NHLBI HCM Registry. JAMA. Published 9 June 2026. doi:10.1001/jama.2026.5633

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