Onset of apical hypertrophic cardiomyopathy in adulthood

Barry J Maron1, Tammy S Haas, Carrie Kitner

  • 1Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation at Abbott Northwestern Hospital, Minnesota, USA. hcm.maron@mhif.org

Insights

Late-onset hypertrophic cardiomyopathy (HC) can develop in adulthood, even in older individuals. Electrocardiogram abnormalities may predict future left ventricular hypertrophy in adults with HC.

Area of Science:

  • Cardiology
  • Genetics

Background:

  • Hypertrophic cardiomyopathy (HC) with left ventricular (LV) hypertrophy typically manifests in adolescence.
  • Late-onset HC presenting in adulthood is infrequently documented.

Purpose of the Study:

  • To describe cases of adult-onset hypertrophic cardiomyopathy (HC).
  • To investigate the clinical, imaging, and electrocardiographic features of late-onset HC.
  • To explore the implications for clinical screening and genetic counseling.

Main Methods:

  • Case series of 4 adult patients with asymptomatic, nonobstructive HC.
  • Serial cardiovascular magnetic resonance imaging or echocardiography.
  • Electrocardiographic analysis.

Main Results:

  • LV hypertrophy appeared in midlife or later (patients >40, >50, or >70 years old).
  • Hypertrophy was confined to the apex with a "spade" deformity, normal ejection fraction, and no systolic anterior motion.
  • Diffuse, marked T-wave inversion on ECG preceded the development of LV hypertrophy.

Conclusions:

  • The onset of LV hypertrophy in HC can be delayed into adulthood and advanced age.
  • Clinical screening practices for families may need to be extended beyond adolescence.
  • Electrocardiographic repolarization abnormalities can predict future apical LV hypertrophy in adults with HC.

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