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转甲状腺素蛋白心脏淀粉样变中的白蛋白尿:患病率、进展和预后意义。

Albuminuria in transthyretin cardiac amyloidosis: Prevalence, progression and prognostic importance.

机构信息

National Amyloidosis Centre, University College London, Royal Free Campus, London, UK.

Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste, Italy.

出版信息

Eur J Heart Fail. 2024 Jan;26(1):65-73. doi: 10.1002/ejhf.3094. Epub 2023 Dec 18.

Abstract

AIMS

Transthyretin cardiac amyloidosis (ATTR-CA) is an infiltrative cardiomyopathy that commonly presents with concomitant chronic kidney disease. Albuminuria is common in heart failure and associated with worse outcomes, but its prevalence and relationship to outcome in ATTR-CA remains unclear.

METHODS AND RESULTS

A total of 1181 patients with ATTR-CA were studied (mean age 78.1 ± 7.9 years; 1022 [86.5%] male; median estimated glomerular filtration rate 59 ml/min/1.73m [interquartile range: 47-74]). Albuminuria was present in 563 (47.7%) patients (499 [88.6%] with microalbuminuria and 64 [11.4%] with macroalbuminuria). Patients with albuminuria had a more severe cardiac phenotype evidenced by higher serum cardiac biomarkers (median N-terminal pro-B-type natriuretic peptide [NT-proBNP]: 4027 ng/L [2173-6889] vs. 1851 ng/L [997-3209], p < 0.001; median troponin T: 69 ng/L [46-101] vs. 48 ng/L [34-68], p < 0.001) and worse echocardiographic indices of systolic (longitudinal strain: -10.0 ± 3.6% vs. -11.6 ± 3.8%, p < 0.001) and diastolic function (E/e': 17.5 ± 6.4 vs. 16.4 ± 6.7, p < 0.001) than those with a normal urinary albumin to creatinine ratio (UACR). Microalbuminuria and macroalbuminuria were independently associated with mortality in the overall population (hazard ratio [HR] 1.47, 95% confidence interval [CI] 1.13-1.92, p = 0.005 and HR 1.87, 95% CI 1.15-3.05, p = 0.012, respectively). In a subgroup of patients (n = 349) without concomitant hypertension, diabetes mellitus or chronic kidney disease, albuminuria was also associated with mortality (HR 2.98, 95% CI 1.72-5.17, p < 0.001). At 12 months, 330 patients had a repeat UACR measurement; those in whom UACR increased by 30% or more (n = 148, 44.8%) had an increased risk of mortality (HR 1.84, 95% CI 1.06-3.19, p = 0.030).

CONCLUSIONS

Albuminuria is common in patients with ATTR-CA, and more prevalent in those with a more severe cardiac phenotype. Albuminuria at diagnosis and a significant increase in UACR during follow-up are associated with mortality.

摘要

目的

转甲状腺素蛋白心脏淀粉样变性(ATTR-CA)是一种浸润性心肌病,常伴有慢性肾脏病。蛋白尿在心力衰竭中很常见,并与更差的预后相关,但在 ATTR-CA 中的患病率及其与预后的关系仍不清楚。

方法和结果

共研究了 1181 例 ATTR-CA 患者(平均年龄 78.1±7.9 岁;1022 例[86.5%]为男性;中位估计肾小球滤过率 59ml/min/1.73m[四分位间距:47-74])。563 例(47.7%)患者存在蛋白尿(499 例[88.6%]为微量白蛋白尿,64 例[11.4%]为大量白蛋白尿)。有蛋白尿的患者心脏表型更严重,表现为更高的血清心脏生物标志物(中位 N 末端脑钠肽前体[NT-proBNP]:4027ng/L[2173-6889] vs. 1851ng/L[997-3209],p<0.001;中位肌钙蛋白 T:69ng/L[46-101] vs. 48ng/L[34-68],p<0.001)和更差的超声心动图收缩功能(纵向应变:-10.0±3.6% vs. -11.6±3.8%,p<0.001)和舒张功能(E/e':17.5±6.4 vs. 16.4±6.7,p<0.001)。微量白蛋白尿和大量白蛋白尿与全人群的死亡率独立相关(风险比[HR] 1.47,95%置信区间[CI] 1.13-1.92,p=0.005 和 HR 1.87,95% CI 1.15-3.05,p=0.012)。在无合并高血压、糖尿病或慢性肾脏病的亚组患者(n=349)中,白蛋白尿也与死亡率相关(HR 2.98,95% CI 1.72-5.17,p<0.001)。在 12 个月时,330 例患者进行了重复 UACR 测量;其中 UACR 增加 30%或以上的患者(n=148,44.8%)死亡风险增加(HR 1.84,95% CI 1.06-3.19,p=0.030)。

结论

白蛋白尿在 ATTR-CA 患者中很常见,且在心脏表型更严重的患者中更为常见。诊断时的白蛋白尿和随访期间 UACR 的显著增加与死亡率相关。

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