Clinical characteristics of wild-type transthyretin cardiac amyloidosis: disprovingmyths
Clinical characteristics of wild-type transthyretin cardiac amyloidosis: disprovingmyths
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DOI：
10.1093/eurheartj/ehx043
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发表时间：
2017-06-24
影响因子：
39.3
通讯作者：
Garcia-Pavia, Pablo
中科院分区：
文献类型：
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作者：
Gonzalez-Lopez, Esther;Gagliardi, Christian;Garcia-Pavia, Pablo
目的野生型甲状腺转维蛋白淀粉样变性（ATTRwt）是一种以老年男性为主的疾病，其特征为左室同心性肥大、LVEF保存、QRS电压低。我们试图描述一大批attrt患者的特征，以更好地定义这种疾病。方法与结果回顾性分析2个中心连续诊断的attrt患者的临床表现。病理或非侵袭性诊断为ATTRwt（左室肥大>= 12 mm， Tc-99m-DPD显像心肌摄取强烈，AL排除）。所有病例均排除TTR突变。研究队列包括108例患者（78.6±8岁）；67例（62%）诊断为侵入性，41例（38%）诊断为非侵入性。20例（19%）为女性。在25例（23%）患者中观察到不对称肥大模式。平均LVEF为52 +/- 14%，39例患者（37%）出现LVEF
Aims Wild-type transthyretin amyloidosis (ATTRwt) is mostly considered a disease predominantly of elderly male, characterized by concentric LV hypertrophy, preserved LVEF, and low QRS voltages. We sought to describe the characteristics of a large cohort of ATTRwt patients to better define the disease.Methods and results Clinical findings of consecutive ATTRwt patients diagnosed at 2 centres were reviewed. ATTRwt was diagnosed histologically or non-invasively (LV hypertrophy >= 12 mm, intense cardiac uptake at Tc-99m-DPD scintigraphy and AL exclusion). Mutations in TTR were excluded in all cases. The study cohort comprised 108 patients (78.6 +/- 8 years); 67 (62%) diagnosed invasively and 41 (38%) non-invasively. Twenty patients (19%) were females. An asymmetric hypertrophy pattern was observed in 25 (23%) patients. Mean LVEF was 52 +/- 14%, with 39 patients (37%) showing a LVEF