The Heart Hospital, University College London Hospitals NHS Foundation Trust, London, UK.
Europace. 2011 Dec;13(12):1781-8. doi: 10.1093/europace/eur267. Epub 2011 Aug 18.
Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder associated with bradyarrhythmias. We sought to examine the nature of conduction system abnormalities and the indications and determinants of anti-bradycardia pacing in patients with AFD.
We studied 204 patients with AFD (49% male, mean age 42 years) in an observational, longitudinal, retrospective cohort study. At baseline, 5 (2.5%) patients had pacemakers for the treatment of bradycardias [4/5 (80%) for atrioventricular disease; 1/5 (20%) for sinus node disease]. PR interval <120 ms was observed in 15 (7%); PR interval >200 ms in 6 (3%); QRS interval >120 ms 18 (9%); left QRS axis deviation in 16 (8%); and right-axis deviation in 2 (1%). Age was an independent determinant of prolonged PR interval, QRS duration and left QRS axis deviation. During follow-up (189 patients; 899 patient-years), 12 (6%) had a device implanted to treat spontaneously occurring bradyarrhythmias [5/12 (42%) for atrioventricular disease; 7/12 (58%) sinus node disease] with 8% 5-year cumulative incidence. Two independent predictors of future anti-bradycardia pacing were identified in a multivariable Cox model: QRS duration [hazard ratio (HR) 1.05, 95% confidence intervals (CI) 1.02-1.09, P= 0.001; receiver operating characteristic (ROC) curve c-statistic 0.726] and PR interval duration (HR 1.03, 95% CI 1.004-1.060, P = 0.023; ROC curve c-statistic 0.548). QRS duration ≥110 ms at baseline had a sensitivity of 64%, specificity of 84%, 49% positive predictive value, and 91% negative predictive value for identifying patients likely to require anti-bradycardia pacing.
In patients with AFD increasing age is associated with PR and QRS interval prolongation and left QRS axis deviation. Pacing for atrioventricular and sinus node disease is common and patients with QRS≥110 ms should be closely monitored for bradyarrhythmias.
安德森-法布里病（AFD）是一种与缓慢性心律失常相关的 X 连锁溶酶体贮积症。我们旨在研究传导系统异常的性质以及 AFD 患者抗心动过缓起搏的适应证和决定因素。
我们在一项观察性、纵向、回顾性队列研究中研究了 204 名 AFD 患者（49%为男性，平均年龄 42 岁）。在基线时，5 名（2.5%）患者因心动过缓而接受起搏器治疗[4/5（80%）为房室病变；1/5（20%）为窦房结病变]。15 名（7%）患者的 PR 间期<120ms；6 名（3%）患者的 PR 间期>200ms；18 名（9%）患者的 QRS 间期>120ms；16 名（8%）患者的左 QRS 轴偏移；2 名（1%）患者的右轴偏移。年龄是 PR 间期延长、QRS 持续时间和左 QRS 轴偏移的独立决定因素。在随访期间（189 名患者；899 患者年），12 名（6%）患者因自发性出现的心动过缓而植入了设备[5/12（42%）为房室病变；7/12（58%）为窦房结病变]，5 年累计发生率为 8%。在多变量 Cox 模型中确定了两个未来抗心动过缓起搏的独立预测因素：QRS 持续时间[风险比（HR）1.05，95%置信区间（CI）1.02-1.09，P=0.001；接收器操作特性（ROC）曲线 c 统计量 0.726]和 PR 间期持续时间（HR 1.03，95%CI 1.004-1.060，P=0.023；ROC 曲线 c 统计量 0.548）。基线时 QRS 持续时间≥110ms 的患者的敏感性为 64%，特异性为 84%，阳性预测值为 49%，阴性预测值为 91%，用于识别可能需要抗心动过缓起搏的患者。
在 AFD 患者中，随着年龄的增长，PR 和 QRS 间隔延长以及左 QRS 轴偏移。房室和窦房结疾病的起搏很常见，QRS≥110ms 的患者应密切监测是否出现缓慢性心律失常。
