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Left atrial remodeling in hypertrophic cardiomyopathy and Fabry disease: A CMR-based head-to-head comparison and

Alice Moroni1, Lara Tondi2, Valentina Milani3

  • 1Clinical and Interventional Cardiology Department, IRCCS Policlinico San Donato, San Donato Milanese, Italy; Multimodality Cardiac Imaging Section, IRCCS Policlinico San Donato, San Donato Milanese, Italy.

International Journal of Cardiology
|September 11, 2023
PubMed

Insights

Hypertrophic cardiomyopathy (HCM) shows greater left atrial (LA) remodeling than Fabry disease cardiomyopathy (FD) with similar left ventricular hypertrophy (LVH). LA reservoir strain predicts heart failure (HF) in HCM patients.

Area of Science:

  • Cardiology
  • Cardiovascular Imaging
  • Genetic Medicine

Background:

  • Hypertrophic cardiomyopathy (HCM) and Fabry disease cardiomyopathy (FD) present as phenocopies with left ventricular hypertrophy (LVH).
  • The left atrium (LA) is increasingly recognized as a marker for disease severity in both HCM and FD.
  • This study investigates LA morpho-functional parameters in HCM versus FD with comparable LVH to identify differences and clinical outcome correlates.

Purpose of the Study:

  • To compare left atrial (LA) morpho-functional parameters between hypertrophic cardiomyopathy (HCM) and Fabry disease cardiomyopathy (FD) patients with similar left ventricular hypertrophy (LVH).
  • To explore the association of LA parameters with clinical outcomes in these patient groups.

Main Methods:

  • Cardiovascular magnetic resonance (CMR) feature tracking (FT), T1 mapping, and conventional imaging were used to assess 30 HCM and 30 FD patients matched for age, sex, BSA, LV mass, and risk factors, plus 30 controls.
  • Transthoracic echocardiography evaluated LV diastolic function.
  • Clinical events, including atrial/ventricular events, bradyarrhythmia, heart failure (HF) hospitalization, and death, were tracked during follow-up.

Main Results:

  • HCM patients exhibited significantly greater LA remodeling than FD patients, characterized by higher LA end-systolic volume index (LAVi max), lower LA ejection fraction (LA-EF), and impaired reservoir (εs) and booster (εa) functions.
  • LA parameters, including LAVi max, demonstrated potential in differentiating between FD and HCM.
  • LAVi max independently predicted HCM diagnosis, and LA reservoir strain (εs) was associated with HF occurrence in HCM patients at 2-year follow-up.

Conclusions:

  • Left atrial remodeling is more pronounced in hypertrophic cardiomyopathy (HCM) compared to Fabry disease cardiomyopathy (FD) even with similar left ventricular hypertrophy (LVH).
  • LA reservoir strain is a significant predictor of heart failure (HF) in HCM patients.
  • These findings highlight distinct LA involvement in HCM and FD and the prognostic value of LA function in HCM.
Abstract

Keywords:
Cardiac magnetic resonanceFabry disease cardiomyopathyFeature-tracking analysisHypertrophic cardiomyopathyLeft atrial strain

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