Aryana Arash, Fifer Michael A, Ruskin Jeremy N, Mela Theofanie
Cardiac Arrhythmia Service, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts 02114, USA.
Pacing Clin Electrophysiol. 2008 Jun;31(6):782-3. doi: 10.1111/j.1540-8159.2008.01088.x.
Fabry disease (FD) is a rare lysosomal storage disease characterized by intracellular deposition of glycosphingolipids that can involve various organs including the heart. Here, we report the finding of a short PR interval in a patient with FD and recurrent syncope associated with short atrial-His (A-H) and His-ventricular (H-V) intervals in the absence of preexcitation. While electrocardiographic abnormalities are not uncommon among FD patients, there have been few reports on electrophysiological studies performed on these patients. The exact mechanism for the short A-H and H-V intervals remains unclear, but they may result from accelerated conduction in the AV node-His-Purkinje system.
法布里病（FD）是一种罕见的溶酶体贮积病，其特征是糖鞘脂在细胞内沉积，可累及包括心脏在内的多个器官。在此，我们报告一例法布里病患者，其伴有反复晕厥，心电图显示PR间期缩短，且在无预激情况下心房-希氏束（A-H）和希氏束-心室（H-V）间期缩短。虽然心电图异常在法布里病患者中并不少见，但对这些患者进行电生理研究的报道较少。A-H和H-V间期缩短的确切机制尚不清楚，但可能是由于房室结-希氏束-浦肯野系统传导加速所致。
