Left atrial structural and functional remodelling in Fabry disease and cardiac amyloidosis: A comparative analysis

Fabry disease (FD) and transthyretin cardiac amyloidosis (TTR CA) are cardiomyopathies with hypertrophic phenotype that share several features, including left atrial (LA) enlargement and dysfunction, but direct comparative data are lacking. Aim of the present study was to perform a comparative analy...

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Published in:International journal of cardiology 2024-05, Vol.402, p.131891-131891, Article 131891
Main Authors: Meucci, Maria Chiara, Lillo, Rosa, Mango, Federica, Marsilia, Mario, Iannaccone, Giulia, Tusa, Filippo, Luigetti, Marco, Biagini, Elena, Massetti, Massimo, Lanza, Gaetano Antonio, Lombardo, Antonella, Graziani, Francesca
Format: Article
Language:English
Subjects:
Amyloidosis
Cardiomyopathies - diagnostic imaging
Cardiomyopathy
Fabry disease
Fabry Disease - complications
Fabry Disease - diagnostic imaging
Heart Atria - diagnostic imaging
Humans
Hypertrophy, Left Ventricular - diagnostic imaging
Left atrial remodelling
Prospective Studies
Speckle-tracking echocardiography
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Summary:Fabry disease (FD) and transthyretin cardiac amyloidosis (TTR CA) are cardiomyopathies with hypertrophic phenotype that share several features, including left atrial (LA) enlargement and dysfunction, but direct comparative data are lacking. Aim of the present study was to perform a comparative analysis of LA remodelling between the two diseases. In this prospective study, a total of 114 patients (31 FD and 83 TTR CA) were included; all of them had left ventricular hypertrophy (LVH), defined as left ventricular (LV) wall thickness ≥ 12 mm. Despite similar degree of LVH, patients with TTR CA showed worse LV systolic and diastolic function. LA maximal volume index was not significantly different between the two groups (p = 0.084), while patients with TTR CA showed larger LA minimal volume index (p = 0.001). Moreover, all phases of LA mechanics were more impaired in the TTR CA group vs FD (reservoir: 6.9[4.2–15.5] vs 19.0[15.5–29.5], p 
ISSN:0167-5273
1874-1754
DOI:10.1016/j.ijcard.2024.131891