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Kokyu to Junkan Volume 38, Issue 9 （September 1990）

呼吸と循環 38巻9号（1990年9月発行）

Japanese English

症例

3年間の経過観察中に右室流出路狭窄が顕性化した肥大型心筋症の1例 A case of hypertrophic cardiomyopathy with right ventricular outflow obstruction manifested during three-years follow-up 佐藤良二 ¹ , 岡田隆夫 ¹ , 老松寛 ¹ , 沢井圀郎 ¹ , 小川孝 ^1,2 , 土田哲人 ^1,2 Ryoji Satoh ¹ , Takao Okada ¹ , Hiroshi Oimatsu ¹ , Kunio Sawai ¹ , Takashi Ogawa ^1,2 , Akihito Tuchida ^1,2 ¹市立釧路総合病院循環器科 ²現札幌医科大学第二内科 ¹Department of Cardiology, Kushiro City General Hospital キーワード：肥大型閉塞性心筋症（obstructive hypertrophic cardiomyopathy） , 右室流出路狭窄（right ventricular outflow obstruction） , 超音波パルスドプラー法（pulsed Doppler echocardiography） Keyword: 肥大型閉塞性心筋症（obstructive hypertrophic cardiomyopathy） , 右室流出路狭窄（right ventricular outflow obstruction） , 超音波パルスドプラー法（pulsed Doppler echocardiography） pp.913-917

発行日 1990年9月15日 Published Date 1990/9/15

DOI https://doi.org/10.11477/mf.1404910016

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Abstract 文献概要
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　左室内圧較差を認めず，約3年間の経過中に右室内圧較差が顕性化した肥大型閉塞性心筋症の1例を経験した。

　患者は55歳女性，1984年（52歳時）心雑音，心電図異常精査のため入院。心カテーテル検査で左右両心室とも心室内圧較差は認めず，心尖部肥厚の造影所見より肥大型心筋症疑いにて経過観察。1987年労作時息切れを自覚し再入院。超音波心断層法で心室中隔壁の右室側への肥大突出が顕著となり，ドップラー断層法で右室流出路に乱流シグナルを，心カテーテル検査では左室内圧較差は認めず，右室-右室流出路間にのみ20mmHgの圧較差が検出され，造影で圧所見に一致する流出路狭窄を認めた。

　肥大型心筋症において，右室流出路に筋性狭窄を伴うことは少なくないが，左心系に圧較差を示さず右心系にのみ圧較差を生じる例は比較的まれである。しかも，本例は心室中隔の肥大進展過程，ならびに血行動態推移を観察しえた貴重な1例と考えられた。

A case is reported of a 55-year-old female with idiopathic hypertrophic cardiomyopathy, which was accompanied with outflow obstruction in the right ventricle developed during the previous 3 years without lesion of the left ventricle.

In 1984, she was admitted to our hospital to be examined for cardiac murmurs and abnormal electro-cardiogram including ST depression and inverted T. The findings of echocardiography and cardiac catheterization revealed non-obstructive hyper-trophic cardiomyopathy. She had been treated with sympathetic beta-blockade and calcium antagonist for 3 years until she complained of dyspnea on exertion, and she was readmitted to our hospital in 1987. Echocardiographic findings showed protrusion of the ventricular septum toward the right ven-tricle and systolic turbulent flow along the right ventricular outflow tract (by pulsed Doppler tech-nique). A pressure gradient of 20 mmHg across the protrusion was detected by the examination of the cardiac catheter. However, neither protrusion nor pressure gradient was observed in the left ventricular outflow tract as well as that in 1984.

Idiopathic hypertrophic cardiomyopathy has been described as involving both ventricles, and outflow obstruction is the usual hemodynamic finding in the left ventricle. However, right ventricular outflow obstruction with the left ventricular outflow tract intact has been very rare. In addition, in this case, the change of hemodynamic characteristics from non-obstructive to obstructive hypertrophic cardio-myopathy, and the development of these changes only in the right ventricle were observed during the last 3 years.