Prophylactic pacemaker implantation in familial amyloid polyneuropathy.
作者： Vincent Algalarrondo , Sylvie Dinanian , Christophe Juin , Denis Chemla , Soumiya L. Bennani
DOI: 10.1016/J.HRTHM.2012.02.033
关键词: Sudden death 、 Asymptomatic 、 Hazard ratio 、 Anesthesia 、 Electrical conduction system of the heart 、 Implantable cardioverter-defibrillator 、 Medicine 、 Electrocardiography 、 Confidence interval 、 Internal medicine 、 Cardiology 、 Bradycardia
摘要: Background Familial amyloid polyneuropathy (FAP) is an autosomic dominant disease with a high rate of conduction disorders and increased risk sudden death. Prophylactic cardiac pacing may be considered in asymptomatic patients FAP. However, the potential benefits are unknown. Objective To document large series FAP incidence high-degree atrioventricular (AV) block prophylactic pacemaker (PM). Methods From January 1999 to 2010, 262 were retrospectively evaluated. PM was implanted His-ventricular interval ≥70 ms, >55 ms associated fascicular block, first-degree AV or Wenckebach anterograde point ≤100 beats/min. The spontaneous then analyzed by temporarily inhibiting PM. Results As compared (n = 100) given class I/IIa indication 18), who did not require 144) younger displayed less severe involvement. Follow-up after implantation 95 100 over 45 ± 35 months, documented 24 (25%). higher beats/min (hazard ratio 3.5; 95% confidence 1.2–10) while microvoltage on surface electrocardiogram reduced 0.2; 0.1–0.7). Conclusion In disorders, prevented major events 25% 45-month mean follow-up. It suggested that symptomatic bradycardia these patients.
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