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[系统性淀粉样变性合并心脏受累患者的特征与演变]

[Features and evolution of patients with systemic amyloidosis and cardiac involvement].

作者信息

Carretero Marcelina, Aguirre Ma A, Villanueva Eugenia, Nucifora Elsa, Posadas-Martínez Ma Lourdes

机构信息

Servicio de Clínica Médica, Área de Investigación en Medicina Interna, Hospital Italiano de Buenos Aires, BA, Argentina.

Servicio de Clínica Médica, Hospital Italiano de Buenos Aires, BA, Argentina.

出版信息

Arch Cardiol Mex. 2022 Jan 3;92(1):60-67. doi: 10.24875/ACM.21000011.

DOI:10.24875/ACM.21000011
PMID:34187048
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8771037/
Abstract

OBJECTIVE

To estimate the prevalence of cardiac amyloidosis in patients with systemic amyloidosis. Compare survival rates based on whether they show cardiac involvement.

METHODS

A retrospective cohort study of patients with systemic amyloidosis from the Institutional Amyloidosis Registry of the Hospital Italian of Buenos Aires from 2010 to 2019. Heart involvement is considered to be the presence of symptoms and/or images consistent with amyloidosis, and there is no other reason to explain it. All deaths due to causes were evaluated. The survival rate was estimated by Kaplan-Meier. Cox regression model was used to evaluate factors related to mortality. Heart transplantation was evaluated in a competitive risk regression model.

RESULTS

The prevalence of heart involvement is 63%. For the group with heart damage, the death rate was 14/1,000 person-months, and for patients without damage, the death rate was 5/1,000 person-months. The 5-year overall survival rate for patients with heart involvement was 44%, while that for patients without damage was 67% (p = 0.02). The original HR for heart involvement was 2.09 (p = 0.02). Age showed that HRa was 1.06 (p <0.01). The sub-HR estimated by the competitive risk regression model are 1.86 (95% CI 0.99-3.49) p = 0.05.

CONCLUSION

Cardiac involvement is an important prognostic factor in patients with amyloidosis.

摘要

目的

评估系统性淀粉样变性患者中心脏淀粉样变性的患病率。比较根据是否存在心脏受累情况的生存率。

方法

对2010年至2019年布宜诺斯艾利斯意大利医院机构淀粉样变性登记处的系统性淀粉样变性患者进行回顾性队列研究。心脏受累被认为是存在与淀粉样变性一致的症状和/或影像，且没有其他原因可以解释。评估所有死因。采用Kaplan-Meier法估计生存率。使用Cox回归模型评估与死亡率相关的因素。在竞争风险回归模型中评估心脏移植情况。

结果

心脏受累的患病率为63%。对于有心脏损害的组，死亡率为14/1000人月，对于无损害的患者，死亡率为5/1000人月。有心脏受累的患者5年总生存率为44%，而无损害的患者为67%（p = 0.02）。心脏受累的原始风险比为2.09（p = 0.02）。年龄显示风险比为1.06（p <0.01）。竞争风险回归模型估计的亚风险比为1.86（95%置信区间0.99 - 3.49）p = 0.05。

结论

心脏受累是淀粉样变性患者的一个重要预后因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf97/8771037/38ebd74ca207/ACM-92-60-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf97/8771037/f33fa8c018bd/ACM-92-60-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf97/8771037/d2cac46d3a92/ACM-92-60-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf97/8771037/fbb4261c68ca/ACM-92-60-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf97/8771037/38ebd74ca207/ACM-92-60-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf97/8771037/f33fa8c018bd/ACM-92-60-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf97/8771037/d2cac46d3a92/ACM-92-60-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf97/8771037/fbb4261c68ca/ACM-92-60-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf97/8771037/38ebd74ca207/ACM-92-60-g004.jpg

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Amyloid cardiomyopathy in a large integrated health care system.大型综合医疗保健系统中的淀粉样心肌病。
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Cardiac amyloidosis: the need for early diagnosis.心脏淀粉样变性：早期诊断的必要性。
轻链（AL）型心脏淀粉样变性的多模态成像方法：一例报告
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