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孤立性促肾上腺皮质激素缺乏所致肾上腺危象相关的严重可逆性心肌病:一例报告

Severe reversible cardiomyopathy associated with adrenal crisis caused by isolated adrenocorticotropin deficiency: a case report.

作者信息

Wang Li, Bu Fangfang, He Lanjie, Yao Guihua

机构信息

Department of Cardiology, Qilu Hospital (Qingdao), Shandong University, Qingdao, China.

Department of Endocrinology, Qilu Hospital (Qingdao), Shandong University, Qingdao, China.

出版信息

Front Cardiovasc Med. 2025 Jan 28;12:1451635. doi: 10.3389/fcvm.2025.1451635. eCollection 2025.

Abstract

Adrenal crisis, also known as acute adrenal insufficiency, is an endocrine emergency that is associated with high mortality rates. Reversible cardiomyopathy with severe heart failure is a rare complication of adrenal crisis. Isolated adrenocorticotropin deficiency (IAD) is a rare condition of pituitary adrenal insufficiency. In this case report, we describe a 74-year-old male patient who was in good physical health and was admitted to our hospital with a sudden onset of fever and confusion that was complicated by hyponatremia and hypotension. Cardiac ultrasound showed significantly reduced left ventricular ejection fraction (LVEF; 10%). The patients was initially diagnosed with "septic shock" because of elevated inflammatory indicators and treated with mechanical circulatory support, antibiotics, fluid resuscitation, and intravenous administration of 50 mg hydrocortisone every 6 h for 2 days (400 mg in total). The symptoms of the patient improved significantly by this treatment in 6 days. The LVEF improved from 10% to 40%. However, the initial treatment did not alleviate hypotension and confusion. Therefore, the status of adrenal function was analyzed using blood and urine cortisol tests. Blood and urinary cortisol levels were significantly reduced, but concurrent increase in the ACTH levels were not observed. This indicated adrenal crisis. Subsequently, the patient was initially administered intravenous injection of hydrocortisone (50-150 mg/day) for 5 days, and then transitioned to a physiological supplement dose orally. The LVEF value improved further to 52%. Finally, the patient was diagnosed with adult isolated ACTH deficiency. The patient was prescribed regular oral hydrocortisone. The patient has not shown any signs of heart failure during follow up for more than half a year. In summary, we described a rare and severe case of adrenal crisis complicated with reversible cardiomyopathy that was caused by isolated ACTH deficiency. In such a case, conventional guideline directed medical therapy (GDMT) for heart failure was not considered suitable because of the underlying hypotension, hypoglycemia, and hyponatremia. Our study showed that timely supplementation of glucocorticoids achieved better therapeutic effects in patients with adrenal crises complicated by severe cardiomyopathy.

摘要

肾上腺危象,又称急性肾上腺皮质功能减退,是一种内分泌急症,死亡率较高。伴有严重心力衰竭的可逆性心肌病是肾上腺危象的一种罕见并发症。孤立性促肾上腺皮质激素缺乏症(IAD)是一种罕见的垂体肾上腺功能不全病症。在本病例报告中,我们描述了一名74岁身体健康的男性患者,他因突然发热和意识模糊入院,并发低钠血症和低血压。心脏超声显示左心室射血分数(LVEF)显著降低(10%)。由于炎症指标升高,该患者最初被诊断为“感染性休克”,并接受了机械循环支持、抗生素、液体复苏治疗,以及每6小时静脉注射50毫克氢化可的松,持续2天(共400毫克)。经过6天的治疗,患者症状明显改善。LVEF从10%提高到了40%。然而,初始治疗并未缓解低血压和意识模糊症状。因此,通过血液和尿液皮质醇检测分析肾上腺功能状态。血液和尿液皮质醇水平显著降低,但促肾上腺皮质激素水平未同时升高。这表明存在肾上腺危象。随后,患者最初静脉注射氢化可的松(50 - 150毫克/天),持续5天,然后过渡到口服生理补充剂量。LVEF值进一步提高到52%。最终,患者被诊断为成人孤立性促肾上腺皮质激素缺乏症。患者开始规律口服氢化可的松。在半年多的随访中,患者未出现任何心力衰竭迹象。总之,我们描述了一例罕见且严重的肾上腺危象合并可逆性心肌病病例,其由孤立性促肾上腺皮质激素缺乏引起。在这种情况下,由于潜在的低血压、低血糖和低钠血症,传统的心力衰竭指导性药物治疗(GDMT)被认为不合适。我们的研究表明,及时补充糖皮质激素对合并严重心肌病的肾上腺危象患者能取得更好的治疗效果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af2f/11810951/cad6d800df76/fcvm-12-1451635-g001.jpg

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