Galetta Fabio, Franzoni Ferdinando, Sposito Roberto, Plantinga Yvonne, Femia Francesca Romana, Galluzzi Fabio, Rocchi Anna, Santoro Gino, Siciliano Gabriele
Department of Internal Medicine, School of Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy.
Neuromuscul Disord. 2005 Jun;15(6):403-8. doi: 10.1016/j.nmd.2005.02.006. Epub 2005 Apr 21.
Myocardial involvement is a common finding in certain myopathies, while it has not been extensively investigated in facioscapulohumeral muscular dystrophy (FSHD1A). Aim of this study was to assess in FSHD1A patients the electrical and functional properties of the myocardium. Twenty-four patients with FSHD1A (mean age 41.2+/-14.5 years) and 24 matched healthy subjects were studied. Standard- and signal-averaged electrocardiography were recorded to determine QT dispersion and the presence of ventricular late potentials (VLPs). Standard echocardiography with systo-diastolic variations of integrated backscatter signal (CV-IBS) were performed to assess functional properties of the myocardium. Compared with control subjects, patients with FSHD1A had significantly lower CV-IBS and higher QT dispersion. Nine patients had positive VLPs. QT and QTc dispersion were inversely related to CV-IBS at both septum and posterior wall levels. Moreover, septal CV-IBS was inversely related to the Kpnl-BinI4q fragment size. These results suggest a subclinical cardiac involvement in FSHD1A patients, which can represent a substrate for ventricular arrhythmias and heart failure.
心肌受累在某些肌病中是常见表现，而在面肩肱型肌营养不良症（FSHD1A）中尚未得到广泛研究。本研究的目的是评估FSHD1A患者心肌的电特性和功能特性。对24例FSHD1A患者（平均年龄41.2±14.5岁）和24例匹配的健康受试者进行了研究。记录标准心电图和信号平均心电图以确定QT离散度和心室晚电位（VLP）的存在。进行标准超声心动图检查，观察背向散射信号的收缩期-舒张期变化（CV-IBS），以评估心肌的功能特性。与对照组相比，FSHD1A患者的CV-IBS显著降低，QT离散度更高。9例患者VLP阳性。在室间隔和后壁水平，QT和QTc离散度与CV-IBS呈负相关。此外，室间隔CV-IBS与Kpnl-BinI4q片段大小呈负相关。这些结果表明FSHD1A患者存在亚临床心脏受累，这可能是室性心律失常和心力衰竭的基础。
