Comparison of echocardiographic parameters in Fabry cardiomyopathy and light-chain cardiac amyloidosis

Josef Marek , Tomas Palecek , Julien Magne (1, 2) , David Lavergne (3) , Cyrille Boulogne (1) , Bahaa Fadel , Arnaud Jaccard (3) , Ales Linhart (4) , Dania Mohty (2, 5)

1 Service de Chirurgie Thoracique et Vasculaire - Médecine vasculaire [CHU Limoges]
2 NET - Neuroépidémiologie Tropicale
3 Service d'Hématologie clinique et thérapie cellulaire [CHU Limoges]
4 CU - Charles University [Prague]
5 Service de cardiologie [CHU Limoges]

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.

Mots clés: Fabry disease; diastolic function; echocardiography; restrictive cardiomyopathy; systolic function

Domaines: Sciences du Vivant [q-bio]; Santé publique et épidémiologie

Josef Marek, Tomas Palecek, Julien Magne, David Lavergne, Cyrille Boulogne, et al.. Comparison of echocardiographic parameters in Fabry cardiomyopathy and light-chain cardiac amyloidosis. Echocardiography, 2018, 35 (11), pp.1755-1763. ⟨10.1111/echo.14144⟩. ⟨hal-02023527⟩