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The Natural History of Nonobstructive Hypertrophic Cardiomyopathy

Virginia B Hebl1, William R Miranda1, Kevin C Ong1

  • 1Department of Medicine/Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN.

Mayo Clinic Proceedings
|March 6, 2016
PubMed

Insights

Survival in nonobstructive hypertrophic cardiomyopathy (NO-HCM) patients mirrors the general population. "Burned out" HCM indicates higher mortality risk, suggesting a need for tailored therapies.

Area of Science:

  • Cardiology
  • Genetics
  • Internal Medicine

Background:

  • Hypertrophic cardiomyopathy (HCM) is a significant cause of heart disease.
  • Nonobstructive HCM (NO-HCM) represents a distinct phenotype requiring specific survival analysis.
  • Understanding mortality risk factors in NO-HCM is crucial for patient management.

Purpose of the Study:

  • To determine the survival rates of a large cohort of patients with NO-HCM.
  • To identify independent risk factors associated with increased mortality in NO-HCM.
  • To compare NO-HCM survival with general population mortality rates.

Main Methods:

  • Retrospective observational study of 706 patients from the Mayo Clinic HCM database (1975-2006).
  • Exclusion of patients with resting or provocable left ventricular outflow tract gradients.
  • Comparison of echocardiographic, clinical, genetic data, and survival rates with age- and sex-matched US population data.

Main Results:

  • Overall survival for NO-HCM patients was comparable to the general US population.
  • Independent predictors of death included age at diagnosis, "burned out" HCM, and history of transient ischemic attack or stroke.
  • Implantable cardioverter defibrillator (ICD) use was inversely related to mortality, but survival was similar after ICD exclusion.

Conclusions:

  • Patients with NO-HCM exhibit similar survival rates to the general population.
  • "Burned out" HCM is an independent risk factor for increased mortality, identifying a high-risk subgroup.
  • Aggressive therapeutic strategies may benefit patients with "burned out" NO-HCM.
Abstract

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