PDF(Pubmed)
Abstract：
Background: Endogenous Cushing\'s syndrome (CS), also called hypercortisolism, leads to a significant increase in mortality due to excessive cortisol production, which is mainly due to cardiovascular disease. CS complicated with cardiomyopathies, which is a rare and severe condition, has rarely been reported in the literature. Objective: To investigate the clinical characteristics of CS complicated with cardiomyopathies, we retrospectively reviewed the clinical manifestations, laboratory results, cardiac imaging results and prognosis to further understand the diagnosis, treatment, and management of these cases. Methods: The clinical data of patients diagnosed with CS complicated with cardiomyopathies obtained from discharge sheets from Peking Union Medical College Hospital from January 1986 to August 2021 were collected. Case reports of CS complicated with cardiomyopathies were retrieved from PubMed. In addition, Cushing\'s disease (CD) patients without cardiomyopathies were collected as controls to compare the clinical features. Results: A total of 19 cases of CS complicated with cardiomyopathies and cases of CD without cardiomyopathies (n = 242) were collected. The causes of CS included pituitary adenoma (n = 8, 42.11%), adrenal adenoma (n = 7, 36.84%), ectopic adrenocorticotropic hormone (ACTH) tumor (n = 2, 10.53%) and unclear causes (n = 2, 10.53%) in the CS complicated with cardiomyopathies group. The types of cardiomyopathies were dilated cardiomyopathies (n = 15, 78.94%) and hypertrophic cardiomyopathies (n = 4, 21.05%). The serum sodium concentration was significantly higher [145.50 (140.50-148.00) mmol/L vs. 141.00 (140.00-143.00) mmol/L], while the serum potassium concentration was significantly lower [2.70 (2.40-3.60) mmol/L] vs. 3.90 (3.50-4.20 mmol/L)] in the CS complicated with cardiomyopathies group compared to the CD patients without cardiomyopathies. There were no significant differences between the CS complicated with cardiomyopathies group and the CD patients without cardiomyopathies in the serum cortisol concentration and 24-h urine free cortisol, but a significant difference in the adrenocorticotropic hormone level [109.00 (91.78-170.30) pg/ml vs. 68.60 (47.85-110.00) pg/ml]. Twelve/16 (75.0%) patients showed significant improvement or even a complete healing of the heart structure and function after remission of hypercortisolemia after treatment with CS. Conclusions: CS complicated with cardiomyopathies is a very rare clinical entity, in which cortisol plays an important role and it can be greatly improved after remission of hypercortisolemia.
摘要：
背景：内源性库欣综合征（CS），也被称为皮质醇增多症，导致由于皮质醇产生过多而导致的死亡率显着增加，这主要是由于心血管疾病。CS并发心肌病，这是一种罕见而严重的情况，在文献中很少有报道。目的:探讨CS合并心肌病的临床特点,我们回顾性回顾了临床表现，实验室结果,进一步了解心脏影像学检查结果和预后,治疗,以及这些案件的管理。方法:收集1986年1月至2021年8月北京协和医院出院单诊断为CS合并心肌病患者的临床资料。从PubMed检索CS并发心肌病的病例报告。此外,收集没有心肌病的库欣病（CD）患者作为对照，以比较其临床特征。结果：共收集了19例CS合并心肌病和CD无心肌病的病例（n=242）。CS的病因包括垂体腺瘤（n=8，42.11%），肾上腺腺瘤（n=7，36.84%），CS合并心肌病组异位促肾上腺皮质激素（ACTH）肿瘤（n=2，10.53％），原因不明（n=2，10.53％）。心肌病的类型为扩张型心肌病（n=15，78.94％）和肥厚型心肌病（n=4，21.05％）。血清钠浓度显著高于[145.50(140.50-148.00)mmol/Lvs.141.00(140.00-143.00)mmol/L],而血清钾浓度显着降低[2.70（2.40-3.60）mmol/L]。与无心肌病的CD患者相比，CS并发心肌病组的3.90（3.50-4.20mmol/L）]。CS合并心肌病组与CD无心肌病患者的血清皮质醇浓度和24h尿游离皮质醇差异无统计学意义。但是促肾上腺皮质激素水平[109.00（91.78-170.30）pg/ml与68.60（47.85-110.00）pg/ml]。12/16（75.0％）患者经CS治疗后，皮质醇血症缓解后，心脏结构和功能显着改善甚至完全愈合。结论：CS并发心肌病是一种非常罕见的临床实体，皮质醇在其中起重要作用，并且在高皮质醇血症缓解后可以大大改善。
