- 作者：Vincent Algalarrondo ; Sylvie Dinanian ; Soumiya Bennani ; David Adams ; Michel Slama
- 刊名：Archives of Cardiovascular Diseases Supplements
- 出版年：2011
- 出版时间：January 2011
- 年：2011
- 卷：3
- 期：1
- 页码：80
- 全文大小：57 K
Introduction
Familial amyloid polyneuropathy (FAP) is an autosomic dominant inherited disease. FAP cardiopathy is characterized by conduction disorders, denervation, and cardiac infiltration. Because of the risk of high degree atrioventricular (AV) block and sudden death, prophylactic pacing can be suggested when there is evidence of amyloid infiltration of the AV conduction pathways. Our purpose was to estimate prevalence of AV block and pacing dependence for high risk FAP patients.Methods
In a cohort of 51 FAP patients, we performed standard ECG and electrophysiological study. Patients with conduction disorders underwent prophylactic pacing. During follow up, AV conduction was studied by using data from the Holter memories of pacemakers.
Results
Baseline characteristics of the patients are summarized on table 1. During the follow up, permanent high degree AV block occurred in 23.5 % of the patients (12/51)(cf. fig. 1). Average percentage was 23 ¡À 35 % for ventricular pacing and 18 ¡À 24 % for atrial pacing. Paroxystic AV block was recorded for all patients but 3 (94 % ). Prevalence of AV conduction disorders was 283 ¡À 465 events per month (epm) and was distributed in 14 ¡À 46 epm for third degree AV block, 195 ¡À 386 epm for second degree AV block, 73 ¡À 225 epm for first degree AV block and 0.2 ¡À 0.7 epm for pauses. During the study, sustained atrial arrhythmias occurred in three patients. No sustained ventricular arrhythmia was recorded.