New Study Helps Doctors Predict Deaths From Common Heart Condition

Hypertrophic cardiomyopathy affects as many as one in 500 people.

WASHINGTON – A new study is reshaping how doctors treat the most common cause of sudden cardiac death (SCD) in young people. Hypertrophic cardiomyopathy (HCM), a thickening of muscle in the heart’s left ventricle, affects up to one in 500 people worldwide.

HCM patients carry increased risk of fatal and non-fatal heart complications, like atrial fibrillation. For the first time, researchers combined a new set of biomarkers that greatly improve the prediction of early HCM-related deaths from heart failure, stroke, and SCD.

While some HCM patients have no symptoms, many develop chest pain, shortness of breath, heart failure, and life-threatening arrhythmias over time.

“This work will directly lead to clinicians identifying high-risk HCM patients earlier and potentially saving lives,” said study author William S. Weintraub, MD, MAAC, recently retired director of Popular Health Research at MedStar Health Research Institute and professor of Medicine at Georgetown University.

Dr. Weintraub directed the Data Coordinating Center for the HCM Registry study, funded by National Institutes of Health’s National Heart, Lung and Blood Institute and industry partners. The HCM Registry includes nearly 2750 patients followed by more than 40 research sites over seven years.

Researchers from University of Virginia School of Medicine and University of Oxford served as principal investigators on the study, which published in JAMA on May 11.

Identifying New HCM Warning Signs

Researchers found that building cardiac magnetic resonance imaging (CMR) and blood test data into risk assessment, alongside clinical and genetic history, more accurately predicted heart failure, stroke, and SCD deaths over the long term:

• CMR more precisely identified the amount of dense scar tissue in the left ventricle. This tissue is associated with deadly HCM complications. 
• Pairing CMR with a blood test for the N-terminal pro B-type natriuretic peptide (NT-proBNP)—a biomarker previously associated with HCM deaths—revealed the highest-risk patients.

“For many high-risk HCM patients, receiving treatment at the right time may reduce their chances of early death,” Weintraub said. “On the other hand, lower-risk patients can be spared from unnecessary layers of care.”

Some HCM patients need an implantable cardioverter-defibrillator (ICD) to correct potentially deadly abnormal heart rhythms. In light of these findings, Weintraub says some patients may be able to forgo ICDs that are recommended under current guidelines.

The HCM Registry study has already generated 16 additional research papers on the condition with several more in progress.

This research was supported by the National Institutes of Health’s National Heart Lung & Blood Institute, Oxford NIHR Biomedical Research Centre, Cytokenics Inc., and the Frederick Thomas Fund.

MedStar Health also received support from Bristol Myers Squibb.