作者
Antonia Camporeale, Maurizio Pieroni, Federico Pieruzzi, Paola Lusardi, Silvia Pica, Marco Spada, Renzo Mignani, Alessandro Burlina, Francesco Bandera, Marco Guazzi, Francesca Graziani, Filippo Crea, Adreas Greiser, Sara Boveri, Federico Ambrogi, Massimo Lombardi
发表日期
2019/4
期刊
Circulation: Cardiovascular Imaging
卷号
12
期号
4
页码范围
e008424
出版商
Lippincott Williams & Wilkins
简介
Background
In prehypertrophic Fabry disease, low myocardial T1 values, reflecting sphingolipid storage, are associated with early structural and ECG changes. The correlations between T1 values and functional parameters have not been explored. Furthermore, the potential prognostic role of T1 in predicting disease worsening is still unknown.
Methods
ECG, 2D echocardiography, cardiopulmonary test, and cardiac magnetic resonance were performed in 44 Fabry patients without left ventricular hypertrophy (35.7±14.5 years, 68.2% females). After a 12-month follow-up, clinical stability was evaluated using Fabry Stabilization Index.
Results
At baseline, T1 values showed a negative correlation with left ventricular mass (r=−0.79; P<0.0001), maximum wall thickness (r=−0.79; P<0.0001), Sokolow-Lyon Index (r=−0.54; P<0.0001), left atrial volume (r=−0.49; P<0.0002), and Mainz Severity Score Index (r=−0.61; P<0 …
学术搜索中的文章
A Camporeale, M Pieroni, F Pieruzzi, P Lusardi, S Pica… - Circulation: Cardiovascular Imaging, 2019