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Myocardial Histopathology in Patients With Obstructive Hypertrophic Cardiomyopathy

Hao Cui1, Hartzell V Schaff1, Juliano Lentz Carvalho1

  • 1Department of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota, USA.

Journal of the American College of Cardiology
|April 30, 2021
PubMed

Insights

This study reveals that specific hypertrophic cardiomyopathy (HCM) histopathology features correlate with clinical outcomes and survival post-myectomy. Understanding these pathological markers can help predict patient prognosis after surgery.

Area of Science:

  • Cardiology
  • Pathology
  • Genetics

Background:

  • Hypertrophic cardiomyopathy (HCM) is a complex cardiac condition characterized by myocyte hypertrophy, disarray, and fibrosis.
  • Understanding the relationship between myocardial histopathology and clinical presentation is crucial for managing obstructive HCM.
  • Septal myectomy is a surgical intervention for obstructive HCM, and its outcomes can be influenced by underlying pathology.

Purpose of the Study:

  • To correlate myocardial histopathological findings in patients with obstructive hypertrophic cardiomyopathy (HCM) with their clinical characteristics.
  • To investigate the association between histopathological features and post-operative outcomes following septal myectomy.
  • To explore the relationship between genetic variants and myocardial histopathology in HCM patients.

Main Methods:

  • Retrospective analysis of myocardial specimens from 1,836 patients who underwent septal myectomy for obstructive HCM (2000-2016).
  • Grading and analysis of myocyte hypertrophy, myocyte disarray, interstitial fibrosis, and endocardial thickening.
  • Correlation of histopathological findings with clinical data, implantable cardioverter-defibrillator (ICD) implantation, atrial fibrillation (AF), genotype, and post-myectomy survival using Cox regression.

Main Results:

  • Myocyte hypertrophy, disarray, and interstitial fibrosis were associated with ICD implantation, while fibrosis and endocardial thickening correlated with pre-operative atrial fibrillation.
  • Older age, lower degrees of myocyte hypertrophy, and lower degrees of endocardial thickening were independently associated with worse post-myectomy survival.
  • Patients with pathogenic/likely pathogenic genetic variants showed greater myocyte disarray, though some with variants had no disarray.

Conclusions:

  • Myocardial histopathology in hypertrophic cardiomyopathy (HCM) is linked to clinical manifestations like disease onset and arrhythmias.
  • Myocyte hypertrophy and endocardial thickening negatively correlate with mortality after septal myectomy, suggesting a protective role.
  • Histopathological findings provide valuable prognostic information for patients with obstructive HCM undergoing surgical intervention.
Abstract

Keywords:
histologyhypertrophic cardiomyopathyinterstitial fibrosismyocyte disarraymyocyte hypertrophy

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