Basal Segmental Longitudinal Strain: A Marker of Subclinical Myocardial Involvement in Anderson-Fabry Disease - 31/03/21
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.
Le texte complet de cet article est disponible en PDF.Highlights |
• | In Fabry disease, impaired basal strain demonstrates early myocardial involvement. |
• | Basal strain is impaired even when LV wall thickness is normal. |
• | Impaired basal strain is associated with worse clinical outcomes. |
• | Consider basal strain analysis when screening patients with Fabry disease. |
Keywords : Anderson-Fabry disease, Strain, Basal longitudinal strain, Two-dimensional speckle-tracking, Left ventricular wall thickness, Adverse outcomes
Abbreviations : 2D, AFD, AUC, COV, ERT, GCS, GLS, LS, LV, LVEF, LVMI, MACE, ROC, SR, TTE
Plan
| Dr. Zada was supported by the Jerry Koutts fellowship for a masters research program. Dr. Lo was supported by the Australian Postgraduate Award PhD grant. Dr. Bradley was supported in part by an investigator-initiated grant from Sanofi Genzyme. |
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| Drs. Zada and Lo are joint first authors. |
Vol 34 - N° 4
P. 405 - avril 2021 Retour au numéro
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