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Kokyu to Junkan Volume 47, Issue 7 （July 1999）

呼吸と循環 47巻7号（1999年7月発行）

Japanese English

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症例

成人後まで生存したEbstein奇形の2例と本邦報告例の文献的考察 Two Cases of Adult Ebstein's Anomaly: Case reports and review of the Japanese literature 吉村達 ¹ , 宇都宮俊徳 ¹ , 小川敏弘 ¹ , 松永萬成 ² , 龍俊宏 ¹ , 吉田和代 ¹ , 尾形徹 ¹ , 辻信介 ¹ , 松尾修三 ¹ Toru Yoshimura ¹ , Toshinori Utsunomiya ¹ , Toshihiro Ogawa ¹ , Moronari Matsunaga ² , Toshihiro Ryu ¹ , Kazuyo Yoshida ¹ , Toru Ogata ¹ , Shinsuke Tsuji ¹ , Shuzo Matsuo ¹ ¹佐賀医科大学内科 ²国立武雄病院 ¹Division of Cardiology, Department of Internal Medicine, Saga Medical School ²Takeo National Hospital キーワード： Ebstein奇形 , 成人症例 , 予後 , Ebstein's anomaly , adult case , prognosis Keyword: Ebstein奇形 , 成人症例 , 予後 , Ebstein's anomaly , adult case , prognosis pp.749-754

発行日 1999年7月15日 Published Date 1999/7/15

DOI https://doi.org/10.11477/mf.1404901938

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Abstract 文献概要
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　成人後まで生存したEbstein奇形を2例経験し，本邦の40歳以上の成人例の文献的考察も行った．症例1は60歳男性で，主訴は倦怠感．症例2は46歳女性で，主訴は動悸．両者とも近医を受診して，心拡大があり心エコーでEbstein奇形を疑われ，精査のため入院．MRI，CT，心血管造影検査で，心房中隔欠損症（ASD）などの合併奇形のないEbstein奇形と診断した．症状がないため手術は施行せず，外来観察中である．本邦における40歳以上のEbstein奇形の報告は本例を含め66例であった．最高年齢は94歳で，13例はASDを認め，9例にチアノーゼを伴っていた．NYHA心機能分類は12例がI〜II度，2例がIV度であった．若年者に比しASDの合併率は低く，NYHA IV度の症例も少ないことが，40歳以上まで生存できた理由と思われた．また，成人の心拡大（特に右心房拡大）ではEbstein奇形も考慮すべきであり，診断に心エコーやMRI検査が有用であった．

　We have reported 2 cases of adult Ebstein's anomaly. We also analyzed 64 cases of Japanese adult (age＞ 40 years old) Ebstein's anomaly in the literature.

　A 60-year-old man was admitted with general fatigue as his chief complaint. A 46-year-old woman was admitted with palpitation as her chief complaint. Neither of these patients had been suffered from cyanosis or congestive heart failure. They showed cardiomegaly on the chest X-ray, especially in the right atrium. Echocardiogram showed abnormal attachment of the tricuspid valve in the right ventricle. Ebstein's anomaly was diagnosed by echocardiography, CT, MRI and angiography. However, no cardiac anomalies were detected in either patient, except tricuspid regurgitation (TR). These patients are now being followed up without symptoms in our outpatient clinic.

　We reviewed 64 Japanese adult (age＞ 40 years old) patients with Ebstein's anomaly in the literature. Ages ranged from 40 to 94 years old. There were 28 men and 28 women, but sex was not listed in another 8 cases. Cardio-thoracic ratio (CTR) ranged from 47 to 90% on the chest X-ray. Atrial septal defect (ASD) was combined in 13/66 cases (20%), cyanosis in 9/66 cases (14%). The fewness of the complications of ASD and cyanosis may be the reason that adult patients Ebstein's anomaly were able to survive to ages over 40. In adult patients with cardiomegaly on chest X-ray, especially in the right atrium, Ebstein's anomaly should be considered as a possible diagnosis. Echocardiography was useful for diagnosing this anomaly.