Basal Segmental Longitudinal Strain: A Marker of Subclinical Myocardial Involvement in Anderson-Fabry Disease

Matthew Zada; Queenie Lo; Anita C Boyd; Sue Bradley; Kerry Devine; Charles P Denaro; Norman Sadick; David A B Richards; Michel C Tchan; Liza Thomas

doi:10.1016/j.echo.2020.11.009

Basal Segmental Longitudinal Strain: A Marker of Subclinical Myocardial Involvement in Anderson-Fabry Disease

J Am Soc Echocardiogr. 2021 Apr;34(4):405-413.e2. doi: 10.1016/j.echo.2020.11.009. Epub 2020 Nov 24.

Authors

Affiliations

¹ Westmead Clinical School, University of Sydney, Westmead Hospital, Sydney, Australia; Department of Cardiology, Westmead Hospital, Sydney, Australia.
² South Western Sydney Clinical School, University of New South Wales, Liverpool Hospital, Sydney, Australia.
³ Westmead Private Cardiology, Sydney, Australia.
⁴ Westmead Clinical School, University of Sydney, Westmead Hospital, Sydney, Australia.
⁵ Genetic Medicine, Westmead Hospital, Sydney, Australia.
⁶ Department of Internal Medicine and Aged Care, Royal Brisbane and Women's Hospital, Brisbane, Australia; University of Queensland Faculty of Medicine, Brisbane, Australia.
⁷ Westmead Clinical School, University of Sydney, Westmead Hospital, Sydney, Australia; South Western Sydney Clinical School, University of New South Wales, Liverpool Hospital, Sydney, Australia.
⁸ Westmead Clinical School, University of Sydney, Westmead Hospital, Sydney, Australia; Department of Cardiology, Westmead Hospital, Sydney, Australia; South Western Sydney Clinical School, University of New South Wales, Liverpool Hospital, Sydney, Australia. Electronic address: liza.thomas@sydney.edu.au.

PMID: 33242609
DOI: 10.1016/j.echo.2020.11.009

Abstract

Background: Cardiac involvement in Anderson-Fabry disease (AFD) is associated with increased left ventricular (LV) wall thickness. The aim of this study was to evaluate if two-dimensional global and regional strain in patients with AFD can identify early myocardial involvement (when LV wall thickness and function are normal). Additionally, the association of altered strain with adverse cardiovascular events was evaluated.

Methods: In a retrospective cross-sectional study, 43 patients with AFD, before enzyme replacement therapy (mean age, 44 ± 12 years; 58.1% men), were compared with age- and gender-matched healthy control subjects. The mean follow-up duration among patients with AFD for major adverse cardiovascular events (MACE) was 82 months.

Results: LV ejection fraction was similar between groups (patients with AFD vs control subjects, 61 ± 8% vs 61 ± 6%; P = .89). However, global longitudinal strain (LS) was impaired in patients with AFD compared with control subjects (-16.5 ± 3.8% vs -20.2 ± 1.7%, P < .001), with greater impairment in patients with AFD with increased LV wall thickness (-15.4 ± 3.9% vs -18.7 ± 2.3%, P < .006). Additionally, LS was most impaired in the basal segments in patients with AFD (-14.8 ± 3.7% vs -20.3 ± 1.1%, P < .001). MACE occurred in 19 of 43 patients (four women, 15 men), and Kaplan-Meier analysis demonstrated that MACE were associated with impaired basal LS.

Conclusions: In patients with AFD, altered basal LS is present even in those with normal LV wall thickness and is associated with MACE. Therefore, basal LS should be considered when screening for cardiac involvement in AFD, particularly in female patients with AFD with normal LV wall thickness.

Keywords: Adverse outcomes; Anderson-Fabry disease; Basal longitudinal strain; Left ventricular wall thickness; Strain; Two-dimensional speckle-tracking.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adult
Cross-Sectional Studies
Fabry Disease* / complications
Fabry Disease* / diagnosis
Female
Humans
Male
Myocardium
Retrospective Studies
Ventricular Dysfunction, Left*
Ventricular Function, Left