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Kokyu to Junkan Volume 33, Issue 6 （June 1985）

呼吸と循環 33巻6号（1985年6月発行）

Japanese English

症例

非対称性心室中隔肥厚，僧帽弁前尖の収縮期前方運動ならびに左室内圧較差を伴った大動脈炎症候群の一例 A case of aortitis syndrome with asymmetric septal hypertrophy, systolic anterior motion of anterior mitral leaflet and pressure gradient within the left ventricle 野口祐一 ¹ , 小川剛 ¹ , 関口達彦 ¹ , 富沢巧治 ¹ , 杉下靖郎 ¹ , 伊藤巌 ¹ , 山口徹 ² , 来栖武雄 ³ , 広江道昭 ⁴ , 関口守衛 ⁴ Yuichi Noguchi ¹ , Takeshi Ogawa ¹ , Tatuhiko Sekiguchi ¹ , Takuji Tomizawa ¹ , Yasuro Sugishita ¹ , Iwao Ito ¹ , Tetsu Yamaguchi ² , Takeo Kurusu ³ , Michiaki Hiroe ⁴ , Morie Sekiguchi ⁴ ¹筑波大学臨床医学系内科 ²三井記念病院循環器センター ³小川町国保中央病院（茨城県）内科 ⁴東京女子医大心研内科 ¹Department of Internal Medicine, Institute of Clinical Medicine, The University of Tsukuba ²Center for Cardiovascular Disease, The Mitsui Memorial Hospital ³Department of Internal Medicine, Ogawa-Machi National Insurance Hospital ⁴The Heart Institute of Japan, Tokyo Women's Medical College pp.817-821

発行日 1985年6月15日 Published Date 1985/6/15

DOI https://doi.org/10.11477/mf.1404204697

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Abstract 文献概要
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　従来より非対称性心室中隔肥厚（asymmetric septalhypertrophy,ASH）ならびに僧帽弁前尖の収縮期前方運動（systolic anterior motion, SAM）は閉塞性肥大型心筋症（hypertrophic obstructive cardiomyopathy,HOCM）に特徴的な所見であるとされてきたが^1〜3），近年これらがHOCM以外の各種疾患^3〜5）にも出現することが報告されている。

　著者らはASH,SAMならびに左室内圧較差を伴った大動脈炎症候群の一例を経験したので報告する。

A case of aortitis syndrome with asymmetric septal hypertrophy (ASH), systolic anterior motion of the anterior mitral leaflet (SAM) and a pressure gradient within the left ventricle is reported. Weak pulsation of right radial artery and hypertension were pointed out at age 28 years and dyspnea on exertion, neck and back pain appeared at age 69 years. Her elder sister died suddenly, although the cause of death was not known and younger brother has suffered from hypertension. On physical exami-nation blood pressure in the right arm was 120/86 mmHg and that in the left arm 196/60 mmHg. Systolic ejection murmur was audible at the apex and bruits at the neck and abdomen. ECG showedhigh voltage of the left ventricle. Chest x-ray re-vealed cardiomegaly and calcification of the aortic wall. The systolic ejection murmur at the apex became louder after inhalation of amyl nitrate. UCG showed ASH and SAM. Catheterization data indicated a pressure gradient within the left ven-tricle and between the descending and abdominal aorta. Angiography showed obstruction of the right subclavian artery and stenosis of the abdominal aorta and left renal artery. Pulmonary scintigram demonstrated multiple perfusion defects. Endo-myocardial biopsy of the right ventricle revealed the finding of "bizarre myocardial hypertrophy with disorganization" in only one of three speci-mens, which did not support strongly the complica-tion of hypertrophic cardiomyopathy (HCM). The diagnosis of aortitis syndrome was made and fur-thermore two possibilities, hypertension due to aortitis syndrome or coexistence of HCM, were considered as the cause of left ventricular outflow tract obstruction.