The surgical pathology of hypertrophic cardiomyopathy

Insights

Pathologists can suggest hypertrophic cardiomyopathy (HCM) using endomyocardial biopsies, but septal myectomy specimens offer more definitive diagnostic findings. Myectomy samples reveal characteristic features like myofiber disarray, crucial for confirming HCM diagnosis.

Area of Science:

  • Cardiovascular Pathology
  • Histopathology
  • Cardiac Surgery

Background:

  • The pathological diagnosis of hypertrophic cardiomyopathy (HCM) traditionally relies on gross cardiac morphology and interventricular septal myofiber disarray.
  • Features of HCM in endomyocardial biopsy and septal myomectomy specimens have received limited attention.

Purpose of the Study:

  • To investigate and characterize the histopathological features of hypertrophic cardiomyopathy (HCM) in endomyocardial biopsy and septal myomectomy specimens.
  • To assess the diagnostic utility of these specimen types for HCM.

Main Methods:

  • Examination of nine right ventricular endomyocardial biopsy specimens and 89 septal myomectomy specimens from 98 patients with clinically diagnosed HCM.
  • Histopathological analysis focusing on myofiber disarray, myocyte hypertrophy, interstitial fibrosis, and other associated findings.

Main Results:

  • Myofiber disarray was observed in 33% of biopsy specimens and 58% of myectomy specimens.
  • Myectomy specimens frequently showed patchy fibrosis, thickened coronary arteries, and endocardial plaques.
  • In myectomy specimens with disarray, it was often confined to the middle transverse third of the septum (79%).

Conclusions:

  • Endomyocardial biopsy can suggest HCM but has limited diagnostic value due to small sample size and nonspecific changes.
  • Septal myectomy specimens typically exhibit characteristic pathological findings consistent with HCM.
  • The surgical pathologist's role is to confirm these characteristic changes in myectomy specimens for definitive HCM diagnosis.

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