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[Hypertrophic cardiomyopathy with left ventricular dilatation].

G Iwami1, Y Miyazaki, K Matsuyama

  • 1Third Department of Internal Medicine, Kurume University School of Medicine.

Journal of Cardiology
|June 1, 1988
PubMed
Summary

Some patients with hypertrophic cardiomyopathy (HCM) develop dilated cardiomyopathy (DCM) features, showing reduced function and increased left ventricular size. This dilated HCM variant is observed in 12% of HCM patients and has a significant mortality rate.

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Area of Science:

  • Cardiology
  • Genetics
  • Pathology

Background:

  • Hypertrophic cardiomyopathy (HCM) can present with diverse clinical and morphological phenotypes.
  • Increasing evidence suggests some HCM patients may progress to a dilated cardiomyopathy (DCM)-like state.

Observation:

  • A subset of 20 out of 165 HCM patients exhibited left ventricular dilatation (end-diastolic diameter ≥ 50 mm), termed dilated HCM.
  • Compared to hypertrophic obstructive cardiomyopathy (HOCM) and typical apical septal hypertrophy (ASH) groups, dilated HCM patients had more frequent family histories of HCM and less frequent hypertension.

Findings:

  • Dilated HCM patients demonstrated significantly reduced fractional shortening (FS), decreased interventricular septal thickness, and elevated left ventricular end-diastolic pressure (LVEDP).
  • During a 3.5-year follow-up, 35% of dilated HCM patients died, primarily from congestive heart failure (CHF).

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  • Apical ASH patients shared similarities with dilated HCM, including higher familial frequency, less septal hypertrophy, and higher LVEDP, with a tendency towards left ventricular dilatation and reduced FS.

    • Implications:

    • Dilated HCM is a recognized, albeit less common, manifestation within the HCM spectrum, occurring in 12% of cases.
    • The findings highlight the importance of recognizing dilated HCM as a distinct phenotype with a potentially poor prognosis.
    • Further research into the genetic and molecular mechanisms underlying the transition from HCM to dilated HCM is warranted.