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英文誌(2004-)

Journal of Medical Ultrasonics

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2023 - Vol.50

Vol.50 No.06

Case Report(症例報告)

(0401 - 0407)

クッシング症候群の若年女性における術後心形態および心機能の変化

Change in postoperative cardiac structure and functions in young women with Cushing's syndrome

山根 彩, 加藤 雅也, 松井 翔吾, 永井 道明, 香川 英介, 國田 英司, 小田 登

Aya YAMANE, Masaya KATO, Syogo MATSUI, Michiaki NAGAI, Eisuke KAGAWA, Eiji KUNITA, Noboru ODA

地方独立行政法人広島市立病院機構広島市立北部医療センター安佐市民病院循環器内科

Department of Cardiology, Hiroshima City North Medical Center Asa Citizens Hospital

キーワード : Cushing's syndrome, left ventricular hypertrophy, secondary hypertension, concentric hypertrophy

クッシング症候群は左室肥大を来たし心血管リスクが高い疾患であり,その病態は未だ明らかになっていない点が多い.今回,副腎腺腫によるクッシング症候群患者における術前術後の心形態および心機能の経時的変化を経胸壁心臓超音波検査にて観察した1例を報告する.症例は30歳女性.難治性高血圧のため紹介受診した.心臓超音波検査では左室壁厚14 mm,左室心筋重量係数120g/m2 Relative wall thickness 0.76と著明な求心性肥大所見を認めた.二次性高血圧を疑い各種ホルモン検査を行った.血中副腎皮質刺激ホルモン(ACTH)は抑制され,血中コルチゾール値は正常範囲であった.入院精査を行ったところ夜間コルチゾール高値,副腎皮質刺激ホルモン放出ホルモン負荷試験にてACTH無反応,1 mgデキサメタゾン抑制試験にて血中コルチゾールは抑制されずコルチゾール自律性分泌を認めた.満月様顔貌,高血圧,月経異常,耐糖能異常のクッシング徴候を認め,クッシング症候群と診断した.また,腹部CTにて左副腎腺腫を疑う腫瘤を認めた.131I-アドステロールシンチを行い,左副腎腫瘍に局在する集積を認めたため,腹腔鏡下左副腎摘除術を行った.術後早期は心形態の改善に乏しく高血圧も持続していたが,徐々に心肥大所見の改善を認め,術後2年でほぼ正常化に至った.

Although Cushing's syndrome causes left ventricular hypertrophy and increases cardiovascular risk, its mechanism remains mostly unclear. We report a case of a patient with Cushing's syndrome caused by an adrenal adenoma, whose preoperative and postoperative cardiac morphology and function were observed over time using transthoracic echocardiography. The patient was a 30-year-old woman referred to us for refractory hypertension. Echocardiography revealed a left ventricular wall thickness of 14 mm and a relative wall thickness of 0.76, indicating significant concentric hypertrophy. We suspected secondary hypertension and performed various hormone tests. Blood adrenocorticotropic hormone (ACTH) was suppressed and blood cortisol was within the normal range. Detailed examinations revealed high nighttime cortisol levels, no response to ACTH in the adrenocorticotropic hormone-releasing hormone challenge test, and no inhibition of blood cortisol in the 1-mg dexamethasone suppression test, and autonomous cortisol secretion was observed. Cushing's sign of moon face, hypertension, abnormal menstruation, and glucose intolerance were observed. Based on the above, Cushing's syndrome was diagnosed. Abdominal computed tomography showed a tumor suspected to be a left adrenal adenoma. Adsterol scintigraphy was performed, which confirmed localization of the left adrenal gland tumor, so laparoscopic left adrenalectomy was performed. In the early postoperative period, there was little improvement in cardiac morphology and hypertension persisted. The findings of cardiac hypertrophy gradually improved, and her condition had mostly normalized 2 years after the operation.