Objective The coexistence of wild-type transthyretin cardiac amyloidosis (ATTR) is common in patients with severe aortic stenosis (AS) undergoing transcatheter aortic valve implantation (TAVI). However, the impact of ATTR and AS on the resultant AS-ATTR is unclear and poses diagnostic and management challenges. We therefore used a multicohort approach to evaluate myocardial structure, function, stress and damage by assessing age-related, afterload-related and amyloid-related remodelling on the resultant AS-ATTR phenotype. Methods We compared four samples (n=583): 359 patients with AS, 107 with ATTR (97% Perugini grade 2), 36 with AS-ATTR (92% Perugini grade 2) and 81 age-matched and ethnicity-matched controls. 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) scintigraphy was used to diagnose amyloidosis (Perugini grade 1 was excluded). The primary end-point was NT-pro Brain Natriuretic Peptide (BNP) and secondary end-points related to myocardial structure, function and damage. Results Compared with older age controls, the three disease cohorts had greater cardiac remodelling, worse function and elevated NT-proBNP/high-sensitivity Troponin-T (hsTnT). NT-proBNP was higher in AS-ATTR (2844 (1745, 4635) ng/dL) compared with AS (1294 (1077, 1554)ng/dL; p=0.002) and not significantly different to ATTR (3272 (2552, 4197) ng/dL; p=0.63). Diastology, hsTnT and prevalence of carpal tunnel syndrome were statistically similar between AS-ATTR and ATTR and higher than AS. The left ventricular mass indexed in AS-ATTR was lower than ATTR (139 (112, 167) vs 180 (167, 194) g; p=0.013) and non-significantly different to AS (120 (109, 130) g; p=0.179). Conclusions The AS-ATTR phenotype likely reflects an early stage of amyloid infiltration, but the combined insult resembles ATTR. Even after treatment of AS, ATTR-specific therapy is therefore likely to be beneficial. No data are available.

中文翻译：

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后负荷和浸润对共存主动脉瓣狭窄和运甲状腺素蛋白淀粉样变性的影响

目的 野生型转甲状腺素蛋白心脏淀粉样变性（ATTR）在接受经导管主动脉瓣植入术（TAVI）的严重主动脉瓣狭窄（AS）患者中很常见。然而，ATTR 和 AS 对由此产生的 AS-ATTR 的影响尚不清楚，并且带来了诊断和管理挑战。因此，我们采用多队列方法，通过评估所得 AS-ATTR 表型的年龄相关、后负荷相关和淀粉样蛋白相关重塑来评估心肌结构、功能、应激和损伤。方法 我们比较了四个样本 (n=583)：359 名 AS 患者、107 名 ATTR 患者（97% Perugini 2 级）、36 名 AS-ATTR 患者（92% Perugini 2 级）和 81 名年龄匹配、种族匹配的对照。99mTc-3,3-二膦酰基-1,2-丙二甲酸 (DPD) 闪烁扫描用于诊断淀粉样变性（排除 Perugini 1 级）。主要终点是NT-pro脑钠肽（BNP），次要终点与心肌结构、功能和损伤相关。结果与年龄较大的对照组相比，三个疾病组的心脏重塑程度更高，功能更差，NT-proBNP/高敏肌钙蛋白-T (hsTnT) 升高。与 AS (1294 (1077, 1554)ng/dL；p=0.002) 相比，AS-ATTR (2844 (1745, 4635) ng/dL) 中的 NT-proBNP 较高，与 ATTR (3272 (2552, 4197) 没有显着差异) 纳克/分升；p=0.63)。AS-ATTR 和 ATTR 之间的诊断学、hsTnT 和腕管综合征患病率在统计学上相似，并且高于 AS。AS-ATTR 中的左心室质量指数低于 ATTR（139 (112, 167) vs 180 (167, 194) g；p=0.013），与 AS 没有显着差异（120 (109, 130) g；p =0.179）。结论 AS-ATTR 表型可能反映了淀粉样蛋白浸润的早期阶段，但组合损伤类似于 ATTR。因此，即使在治疗 AS 后，ATTR 特异性治疗也可能是有益的。无可用数据。