Reduced Myocardial 123-Iodine Metaiodobenzylguanidine Uptake A Prognostic Marker in Familial Amyloid Polyneuropathy
Reduced Myocardial 123-Iodine Metaiodobenzylguanidine Uptake A Prognostic Marker in Familial Amyloid Polyneuropathy
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DOI：
10.1161/circimaging.112.000367
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发表时间：
2013-09-01
影响因子：
7.5
通讯作者：
Diogo, Antonio Nunes
中科院分区：
文献类型：
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作者：
Azevedo Coutinho, Maria C.;Cortez-Dias, Nuno;Diogo, Antonio Nunes
转甲状腺素家族性淀粉样蛋白多发性神经病是一种遗传性淀粉样变性，以感觉运动和自主神经病变、心脏传导缺陷和浸润性心肌病为特征。先前的研究表明，123-碘-甲基碘苄基胍（MIBG）成像评估的心肌交感神经去神经发生在疾病进展的早期。然而，其预后意义从未被评估过。我们的目的是研究经甲状腺素家族性淀粉样蛋白多发性神经病中MIBG显像检测心肌交感神经失支配的长期预后价值。方法与结果143例V30M甲状腺素突变患者行动态心电图、动态血压监测、超声心动图和MIBG成像。采用Cox比例风险回归将全因死亡时间与晚期心脏-纵隔MIBG摄取比（H/M；与正常估计下限[1.60]相关或作为连续变量）进行比较。进行多变量分析以检验临床、神经学和心血管参数的预后准确性。在中位5.5年的随访期间，32例（22%）患者死亡。晚期H/M的5年死亡率为42%
Background Transthyretin familial amyloid polyneuropathy is a hereditary form of amyloidosis characterized by sensorimotor and autonomic neuropathy, cardiac conduction defects, and infiltrative cardiomyopathy. Previous studies have suggested that myocardial sympathetic denervation assessed by 123-iodine metaiodobenzylguanidine (MIBG) imaging occurs early in disease progression. However, its prognostic significance was never evaluated. We aimed to study the long-term prognostic value of myocardial sympathetic denervation detected by MIBG imaging in transthyretin familial amyloid polyneuropathy.Methods and Results A total of 143 individuals with V30M transthyretin mutation underwent Holter, ambulatory blood pressure monitoring, echocardiography, and MIBG imaging. Time to all-cause death was compared with late heart-to-mediastinum MIBG uptake ratio (H/M; either in relation to the estimated lower limit of normal [1.60] or as a continuous variable) using Cox proportional hazards regression. Multivariable analyses were performed to test the prognostic accuracy of clinical, neurological, and cardiovascular parameters. During a median follow-up of 5.5 years, 32 (22%) patients died. Five-year mortality rate was 42% for late H/M