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Comparison of echocardiographic parameters in Fabry cardiomyopathy and light-chain cardiac amyloidosis.
- Source :
-
Echocardiography (Mount Kisco, N.Y.) [Echocardiography] 2018 Nov; Vol. 35 (11), pp. 1755-1763. Date of Electronic Publication: 2018 Sep 24. - Publication Year :
- 2018
-
Abstract
- Background: Fabry cardiomyopathy (FC) and light-chain amyloid cardiomyopathy (AL) present with concentric left ventricular (LV) hypertrophy/remodeling and diastolic rather than systolic dysfunction. Direct comparisons are difficult due to rarity and confounded by variability of LV thickness.<br />Aims: To compare LV diastolic and systolic properties between patients with FC and AL in a cohort matched for interventricular septal thickness (IVS).<br />Methods: A two-center echocardiographic analysis was performed, comprising 118 patients with IVS ≥12 mm (FC and AL 59 patients each) matched by IVS.<br />Results: Fabry cardiomyopathy patients had larger LV end-diastolic diameter (47.7 [44.0-50.9] vs 45.0 [41.5-49.0] mm, P = 0.002), better LV ejection fraction (EF 68.7 [63.4-74.0] vs 63.0 [54.0-70.0]%, P = 0.001) and midwall fractional shortening (midFS 14.8 [13.0-16.1] vs 12.1 [8.9-15.0]%, P = 0.006). LV EF <40% was rare in both (2% vs 7%, P = 0.17). AL patients expressed higher LV diastolic dysfunction grade (III in 26% vs 4%, II in 21% vs 12% and I in 54% vs 84%, P = 0.004), with higher E/e' ratio (13.6 [10.2-18.8] vs 9.8 [7.5-12.3], P < 0.0001). Average E/e' ratio and midFS were significantly associated with NYHA severity in both groups (P < 0.05 for all).<br />Conclusions: Matched AL patients had worse LV diastolic function than FC, driven by E/e'. Significant LV systolic dysfunction was rare overall. MidFS and E/e' were associated with heart failure severity in both groups.<br /> (© 2018 Wiley Periodicals, Inc.)
- Subjects :
- Aged
Amyloidosis physiopathology
Cardiomyopathies physiopathology
Fabry Disease physiopathology
Female
Heart Diseases physiopathology
Heart Ventricles diagnostic imaging
Heart Ventricles physiopathology
Humans
Male
Middle Aged
Reproducibility of Results
Retrospective Studies
Sensitivity and Specificity
Ventricular Dysfunction, Left physiopathology
Ventricular Remodeling physiology
Amyloidosis diagnostic imaging
Cardiomyopathies diagnostic imaging
Echocardiography methods
Fabry Disease diagnostic imaging
Heart Diseases diagnostic imaging
Ventricular Dysfunction, Left diagnostic imaging
Subjects
Details
- Language :
- English
- ISSN :
- 1540-8175
- Volume :
- 35
- Issue :
- 11
- Database :
- MEDLINE
- Journal :
- Echocardiography (Mount Kisco, N.Y.)
- Publication Type :
- Academic Journal
- Accession number :
- 30247786
- Full Text :
- https://doi.org/10.1111/echo.14144