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Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
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Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

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Differences in the extent of fibrosis in obstructive and nonobstructive hypertrophic cardiomyopathy

Gustavo Avegliano1,2, María T Politi1, Juan P Costabel2

  • 1Cardiac Imaging Department.

Journal of Cardiovascular Medicine (Hagerstown, Md.)
|April 18, 2019
PubMed

Insights

Left ventricular outflow tract (LVOT) obstruction in hypertrophic cardiomyopathy (HCM) is linked to increased myocardial fibrosis and diastolic dysfunction. This study confirms that obstructive HCM significantly correlates with higher fibrosis extent and impaired LV diastolic function.

Area of Science:

  • Cardiology
  • Cardiovascular Imaging
  • Cardiac MRI

Background:

  • Left ventricular outflow tract (LVOT) obstruction is a hallmark of hypertrophic cardiomyopathy (HCM), associated with adverse patient outcomes.
  • Previous research suggests LVOT obstruction contributes to myocardial fibrosis and elevated left ventricular (LV) filling pressures, leading to clinical decline.
  • Clinical evidence directly linking LVOT obstruction to these pathological changes in HCM patients remains limited.

Purpose of the Study:

  • To investigate the association between LVOT obstruction and myocardial fibrosis in HCM patients.
  • To determine if LVOT obstruction correlates with impaired LV diastolic function in HCM.
  • To provide clinical data supporting the hypothesis that LVOT obstruction exacerbates HCM pathology.

Main Methods:

  • Retrospective analysis of 67 HCM patients undergoing both Doppler echocardiography and cardiac MRI.
  • Assessment of LV diastolic function using the E/e' ratio.
  • Quantification of myocardial fibrosis using late gadolinium enhancement on cardiac MRI.
  • Stratification of patients based on the presence or absence of resting LVOT obstruction.

Main Results:

  • Obstructive HCM showed a significant association with extensive myocardial fibrosis (OR 3.33, P=0.034), even after adjusting for age and sex.
  • Obstructive HCM was strongly linked to a high E/e' ratio (>14), indicating diastolic dysfunction (OR 7.8, P=0.001).
  • A significant correlation was found between elevated E/e' ratio and the extent of fibrosis (OR 1.29, P<0.001), persisting after multivariate adjustment.

Conclusions:

  • LVOT obstruction in HCM appears to play a role in the development and extent of myocardial fibrosis.
  • The findings suggest that LVOT obstruction may contribute to or worsen diastolic dysfunction in HCM patients.
  • This study provides clinical evidence supporting the detrimental impact of LVOT obstruction on HCM pathophysiology.
Abstract

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