Sakihara Satoru, Kageyama Kazunori, Nigawara Takeshi, Kidani Yukie, Suda Toshihiro
Department of Endocrinology and Metabolism, Hirosaki University School of Medicine, 5 Zaifu-cho, Hirosaki, Aomori 036-8562, Japan.
Endocr J. 2007 Aug;54(4):631-6. doi: 10.1507/endocrj.k07-012. Epub 2007 Jul 31.
We describe here a case of reversible ampulla (takotsubo) cardiomyopathy caused by secondary adrenal insufficiency in ACTH isolated deficiency. A 53-year-old woman was referred to our department for evaluation and treatment of unconsciousness. On admission, her plasma glucose level was 34 mg/dL, suggesting loss of consciousness due to hypoglycemia. Basal levels of ACTH, cortisol, and dehydroepiandrosterone sulfate in blood, and urinary free cortisol levels were all decreased. ACTH and cortisol levels were not adequately increased in response to CRH administration and the insulin tolerance test. Electrocardiography showed ST segment elevation and T wave inversion in leads V 1-6. The coronary arteries were free of organic stenosis, and a left ventriculogram revealed severe hypokinesis, particularly in the anterior and posterior walls. Based on a diagnosis of adrenocortical insufficiency caused by ACTH isolated deficiency, hydrocortisone was administered. Two weeks after treatment, ultrasound studies of the heart showed recovery of left ventricular wall motion. Activation of the sympathetic nervous system, adrenocortical failure, and hypoglycemic attack were considered to be triggering factors for the takotsubo cardiomyopathy. Careful monitoring of cardiac function and appropriate treatments for both cardiomyopathy and adrenocortical failure are required to recover cardiac dysfunction.
我们在此描述一例由促肾上腺皮质激素（ACTH）孤立性缺乏导致继发性肾上腺功能不全引起的可逆性壶腹样（应激性）心肌病。一名53岁女性因意识丧失被转诊至我院进行评估和治疗。入院时，她的血浆葡萄糖水平为34mg/dL，提示因低血糖导致意识丧失。血液中ACTH、皮质醇和硫酸脱氢表雄酮的基础水平以及尿游离皮质醇水平均降低。给予促肾上腺皮质激素释放激素（CRH）和胰岛素耐量试验后，ACTH和皮质醇水平未充分升高。心电图显示V1 - 6导联ST段抬高和T波倒置。冠状动脉无器质性狭窄，左心室造影显示严重运动减弱，尤其是在前壁和后壁。基于ACTH孤立性缺乏导致肾上腺皮质功能不全的诊断，给予氢化可的松治疗。治疗两周后，心脏超声检查显示左心室壁运动恢复。交感神经系统激活、肾上腺皮质功能衰竭和低血糖发作被认为是应激性心肌病的触发因素。为恢复心脏功能，需要仔细监测心脏功能并对心肌病和肾上腺皮质功能衰竭进行适当治疗。
