Rakovec P, Zidar J, Sinkovec M, Zupan I, Brecelj A
University Medical Center, Ljubljana, Slovenia.
Pacing Clin Electrophysiol. 1995 Sep;18(9 Pt 1):1721-4. doi: 10.1111/j.1540-8159.1995.tb06996.x.
Emery-Dreifuss muscular dystrophy is an X-linked recessive myopathy. Its progression is slow, and it rarely leads to cessation of walking; therefore, it has often been called "benign." On the other hand, cardiac involvement is often severe and sudden death is not uncommon. We describe a family with four affected males, two of whom died suddenly. The case of an affected man with first-degree AV block, incomplete right bundle branch block, and left anterior fascicular block is described in detail. The prophylactic insertion of a diagnostic pacemaker enabled us to follow the progress of conduction disturbances without leaving the patient unprotected. While AV and intraventricular conduction defects were more prominent before pacemaker implantation, sinus node dysfunction became more important during the follow-up.
埃默里-德赖富斯肌营养不良症是一种X连锁隐性肌病。其进展缓慢，很少导致无法行走；因此，它常被称为“良性”。另一方面，心脏受累往往很严重，猝死并不罕见。我们描述了一个有四名患病男性的家庭，其中两人突然死亡。详细描述了一名患有一度房室传导阻滞、不完全性右束支传导阻滞和左前分支传导阻滞的患病男性病例。预防性植入诊断性起搏器使我们能够跟踪传导障碍的进展，同时又不会让患者失去保护。虽然房室和心室内传导缺陷在起搏器植入前更为突出，但在随访期间窦房结功能障碍变得更为重要。
