Kocher Florian, Kaser Alex, Escher Felix, Doerler Jacob, Zaruba Marc-Michael, Messner Moritz, Mussner-Seeber Christine, Mayr Agnes, Ulmer Hanno, Schneiderbauer-Porod Stephanie, Ebner Christian, Poelzl Gerhard
Department of Internal Medicine V (Hematology and Oncology), Medical University of Innsbruck, Anichstrasse 35, Innsbruck, 6020, Austria.
Department of Internal Medicine III (Cardiology and Angiology), Medical University of Innsbruck, Innsbruck, Austria.
ESC Heart Fail. 2020 Dec;7(6):3919-3928. doi: 10.1002/ehf2.12986. Epub 2020 Oct 1.
Amyloid cardiomyopathy is an underappreciated cause of morbidity and mortality. Recent evidence suggests that ATTR wild-type cardiomyopathy (ATTRwt-CM) is probably much more common than widely appreciated. So far, no data are available on comparison of mortality from ATTRwt-CM and other heart failure aetiologies.
This was a retrospective, observational, cohort study of 2251 patients and their data collected prospectively from May 2000 to June 2018. Long-term mortality was the main outcome measure. Underlying cardiomyopathies were classified as amyloid CM (6.1%) [ATTRwt 3.0%; light-chain amyloidosis (AL) 3.1%], dilated CM (dCMP) (46.4%), ischaemic heart disease (IHD) (24.4%), hypertensive heart disease (HHD) (14.6%), hypertrophic CM (HCM) (5.1%), and valvular heart disease (VHD) (3.4%). Median duration of follow-up was 7.1 years (interquartile range 3.4-11.3). Five-year overall survival in the whole cohort was 80.1%. In multivariate analysis, individuals with amyloid CM were 3.74 times [95% confidence interval (CI) 2.72-5.14; P < 0.001] more likely to die of any reason than were individuals with dCMP. Mortality was higher in AL-CM compared with ATTRwt-CM [hazard ratio (HR) 2.88; 95% CI 1.48-5.58; P = 0.002]. Mortality rates in patients with ATTRwt-CM were higher than in patients with dCMP (HR 1.96; 95% CI 1.24-3.22; P = 0.007), HCM (HR 2.94; 95% CI 1.28-6.67; P = 0.011), HHD (HR 2.08; 95% CI 1.27-3.45; P = 0.004), VHD (HR 2.38; 95% CI 1.30-4.35; P = 0.005), or left ventricular ejection fraction ≥ 40% (HR 1.99; 95% CI 1.12-3.52; P = 0.018).
Our study demonstrates that amyloid CM is independently associated with poor survival among patients with various causes of heart failure. ATTRwt-CM had a better long-term prognosis than did AL-CM, but was associated with higher mortality than were dCMP, HCM, HHD, VHD, and heart failure with preserved or mid-range ejection fraction.
淀粉样心肌病是发病率和死亡率的一个未得到充分认识的原因。最近的证据表明，野生型转甲状腺素蛋白淀粉样变心肌病（ATTRwt-CM）可能比普遍认为的更为常见。到目前为止，尚无关于ATTRwt-CM与其他心力衰竭病因死亡率比较的数据。
这是一项对2251例患者的回顾性观察队列研究，其数据于2000年5月至2018年6月前瞻性收集。长期死亡率是主要结局指标。潜在的心肌病分类为淀粉样心肌病（6.1%）[ATTRwt 3.0%；轻链淀粉样变（AL）3.1%]、扩张型心肌病（dCMP）（46.4%）、缺血性心脏病（IHD）（24.4%）、高血压性心脏病（HHD）（14.6%）、肥厚型心肌病（HCM）（5.1%）和心脏瓣膜病（VHD）（3.4%）。中位随访时间为7.1年（四分位间距3.4 - 11.3年）。整个队列的5年总生存率为80.1%。在多变量分析中，与dCMP患者相比，淀粉样心肌病患者死于任何原因的可能性高3.74倍[95%置信区间（CI）2.72 - 5.14；P < 0.001]。与ATTRwt-CM相比，AL-CM的死亡率更高[风险比（HR）2.88；95% CI 1.48 - 5.58；P = 0.002]。ATTRwt-CM患者的死亡率高于dCMP患者（HR 1.96；95% CI 1.24 - 3.22；P = 0.007）、HCM患者（HR 2.94；95% CI 1.28 - 6.67；P = 0.011）、HHD患者（HR 2.08；95% CI 1.27 - 3.45；P = 0.004）、VHD患者（HR 2.38；95% CI 1.30 - 4.35；P = 0.005）或左心室射血分数≥40%的患者（HR 1.99；95% CI 1.12 - 3.52；P = 0.018）。
我们的研究表明，淀粉样心肌病与各种心力衰竭病因患者的不良生存独立相关。ATTRwt-CM的长期预后优于AL-CM，但与dCMP、HCM、HHD、VHD以及射血分数保留或中等范围的心力衰竭相比，其死亡率更高。
