Heart failure in Fabry disease revisited: application of current heart failure guidelines and recommendations

Autor: Daniel Rob, Josef Marek, Gabriela Dostalova, Ales Linhart
Jazyk: angličtina
Rok vydání: 2022
Předmět:
Zdroj: ESC Heart Failure, Vol 9, Iss 6, Pp 4043-4052 (2022)
Druh dokumentu: article
ISSN: 2055-5822
DOI: 10.1002/ehf2.14091
Popis: Abstract Aims Fabry disease (FD) is often associated with heart failure (HF). However, data on HF prevalence, prognosis, and applicability of echocardiographic criteria for HF diagnosis in FD remain uncertain. Methods and results We evaluated patients with genetically proven FD for symptoms and natriuretic peptides indicating HF. We then analysed the diagnostic utility of the currently recommended European Society of Cardiology (ESC) echocardiographic criteria for HF diagnosis and their relationship to natriuretic peptides. Finally, we examined the association between HF and echocardiographic criteria with mortality and cardiovascular events during follow‐up. Of 116 patients with FD, 48 (41%) had symptomatic HF (mean age 58 ± 11 years, 62% male). HF with preserved ejection fraction (HF‐pEF) was diagnosed in 43 (91%) patients, representing the dominant phenotype. Left ventricular mass index (LVMi) had the highest diagnostic utility (sensitivity 71% and specificity 83%) for HF diagnosis in FD, followed by E/e′ > 9 (sensitivity 76% and specificity 78%) and global longitudinal strain (GLS) 9, and GLS
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