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

• 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; Cardiomyopathy; Fabry disease; Left atrial remodelling; Speckle-tracking echocardiography
• ISSN: 0167-5273; 1874-1754
• DOI: 10.1016/j.ijcard.2024.131891

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