Department of Cardiology, Inselspital Bern University Hospital, University of Bern Bern Switzerland.
CTU Bern University of Bern Bern Switzerland.
J Am Heart Assoc. 2023 Aug 15;12(16):e030271. doi: 10.1161/JAHA.123.030271. Epub 2023 Aug 10.
Background The prevalence of calcific aortic stenosis and amyloid transthyretin cardiomyopathy (ATTR-CM) increase with age, and they often coexist. The objective was to determine the prevalence of ATTR-CM in patients with severe aortic stenosis and evaluate differences in presentations and outcomes of patients with concomitant ATTR-CM undergoing transcatheter aortic valve implantation. Methods and Results Prospective screening for ATTR-CM with Technetium-3,3-diphosphono-1,2-propanodicarboxylic acid bone scintigraphy was performed in 315 patients referred with severe aortic stenosis between August 2019 and August 2021. Myocardial Technetium-3,3-diphosphono-1,2-propanodicarboxylic acid tracer uptake was detected in 34 patients (10.8%), leading to a diagnosis of ATTR-CM in 30 patients (Perugini ≥2: 9.5%). Age (85.7±4.9 versus 82.8±4.5; =0.001), male sex (82.4% versus 57.7%; =0.005), and prior carpal tunnel surgery (17.6% versus 4.3%; =0.007) were associated with coexisting ATTR-CM, as were ECG (discordant QRS voltage to left ventricular wall thickness [42% versus 12%; <0.001]), echocardiographic (left ventricular ejection fraction 48.8±12.8 versus 58.4±10.8; <0.001; left ventricular mass index, 144.4±45.8 versus 117.2±34.4g/m; <0.001), and hemodynamic parameters (mean aortic valve gradient, 23.4±12.6 versus 35.5±16.6; <0.001; mean pulmonary artery pressure, 29.5±9.7 versus 25.8±9.5; =0.037). Periprocedural (cardiovascular death: hazard ratio [HR], 0.71 [95% CI, 0.04-12.53]; stroke: HR, 0.46 [95% CI, 0.03-7.77]; pacemaker implantation: HR, 1.54 [95% CI, 0.69-3.43]) and 1-year clinical outcomes (cardiovascular death: HR, 1.04 [95% CI, 0.37-2.96]; stroke: HR, 0.34 [95% CI, 0.02-5.63]; pacemaker implantation: HR, 1.50 [95% CI, 0.67-3.34]) were similar between groups. Conclusions Coexisting ATTR-CM was observed in every 10th elderly patient with severe aortic stenosis referred for therapy. While patients with coexisting pathologies differ in clinical presentation and echocardiographic and hemodynamic parameters, peri-interventional risk and early clinical outcomes were comparable up to 1 year after transcatheter aortic valve implantation. REGISTRATION URL: https://www.clinicaltrials.gov. Unique identifier: NCT04061213.
随着年龄的增长，钙化性主动脉瓣狭窄和转甲状腺素蛋白淀粉样变心肌病（ATTR-CM）的患病率增加，且它们常同时存在。本研究旨在确定严重主动脉瓣狭窄患者中 ATTR-CM 的患病率，并评估同时患有 ATTR-CM 行经导管主动脉瓣置换术（TAVR）患者的临床表现和结局差异。
前瞻性筛选 2019 年 8 月至 2021 年 8 月期间因严重主动脉瓣狭窄而转诊的 315 例患者，使用锝-3,3-二膦酸-1,2-丙二醇骨骼闪烁显像术进行 ATTR-CM 的筛查。在 34 例（10.8%）患者中检测到心肌锝-3,3-二膦酸-1,2-丙二醇示踪剂摄取，其中 30 例（佩鲁吉尼评分≥2：9.5%）诊断为 ATTR-CM。共存 ATTR-CM 与年龄（85.7±4.9 岁比 82.8±4.5 岁；=0.001）、男性（82.4%比 57.7%；=0.005）和既往腕管手术（17.6%比 4.3%；=0.007）相关，并存 ATTR-CM 患者还与心电图（不同步的 QRS 电压与左心室壁厚度[42%比 12%；<0.001]）、超声心动图（左心室射血分数 48.8±12.8 比 58.4±10.8；<0.001；左心室质量指数 144.4±45.8 比 117.2±34.4g/m；<0.001]）和血流动力学参数（平均主动脉瓣梯度 23.4±12.6 比 35.5±16.6；<0.001；平均肺动脉压 29.5±9.7 比 25.8±9.5；=0.037）相关。围手术期（心血管死亡：风险比[HR]，0.71[95%CI，0.04-12.53]；卒：HR，0.46[95%CI，0.03-7.77]；起搏器植入：HR，1.54[95%CI，0.69-3.43]）和 1 年临床结局（心血管死亡：HR，1.04[95%CI，0.37-2.96]；卒：HR，0.34[95%CI，0.02-5.63]；起搏器植入：HR，1.50[95%CI，0.67-3.34]）在两组之间相似。
在接受 TAVR 治疗的每 10 例老年严重主动脉瓣狭窄患者中，均可发现共存的 ATTR-CM。虽然共存疾病患者在临床表现、超声心动图和血流动力学参数方面存在差异，但在 TAVR 后 1 年内，围手术期风险和早期临床结局相似。
