Cardiac magnetic resonance LGE% (HR 1.86; 95% CI 1.58-2.20) and NT-proBNP (HR 1.41; 95% CI 1.17-1.70) significantly predicted adverse events in patients with hypertrophic cardiomyopathy.
Cohort (n=2,750)
Yes
Does incorporating cardiac magnetic resonance imaging and NT-proBNP improve risk prediction of adverse events in patients with hypertrophic cardiomyopathy?
Incorporating cardiac magnetic resonance imaging (specifically late gadolinium enhancement) and NT-proBNP significantly improves the prediction of long-term adverse outcomes in patients with hypertrophic cardiomyopathy.
Effect estimate: HR 1.86 (95% CI 1.58-2.20)
p-value: p=<.001
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.
“This is an important next step to do a better job of identifying HCM patients at high risk," he said. "This adds to presently used risk markers derived from the patient's and their family's prior history.”
Published in JAMA, this large registry study identified novel predictors of adverse outcomes in hypertrophic cardiomyopathy, including CMR findings and biomarkers, which could lead to improved risk stratification and management of this complex genetic heart disease.
Investigators et al. (Mon,) conducted a cohort in Hypertrophic cardiomyopathy (n=2,750). Cardiac magnetic resonance imaging and biomarker evaluation was evaluated on Time to first event for hypertrophic cardiomyopathy-related deaths, nonfatal sustained ventricular arrhythmias requiring cardioversion/defibrillation, and LV assist device implant or heart transplant (HR 1.86, 95% CI 1.58-2.20, p=<.001). Cardiac magnetic resonance LGE% (HR 1.86; 95% CI 1.58-2.20) and NT-proBNP (HR 1.41; 95% CI 1.17-1.70) significantly predicted adverse events in patients with hypertrophic cardiomyopathy.