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英文誌(2004-)

Journal of Medical Ultrasonics

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2016 - Vol.43

Vol.43 No.06

Case Report(症例報告)

(0739 - 0743)

症状発現から急速に悪化したカルチノイド腫瘍に伴う三尖弁逆流症の1例

A case of carcinoid heart disease with rapid progression of symptoms

須磨谷 いづみ1, 吉岡 賢二2, 鍵山 暢之3, 水上 暁2, 本間 浩一4, 大塚 喜人1, 田邉 大明5, 橋本 裕二2

Izumi SUMATANI1, Kenji YOSHIOKA2, Nobuyuki KAGIYAMA3, Akira MIZUKAMI2, Koichi HONMA4, Yoshihito OTSUKA1, Hiroaki TANABE5, Yuji HASHIMOTO2

1亀田総合病院臨床検査部, 2亀田総合病院循環器内科, 3心臓病センター榊原病院循環器内科, 4亀田総合病院臨床病理科, 5亀田総合病院心臓血管外科

1Department of Laboratory Medicine, Kameda Medical Center, 2Department of Cardiology, Kameda Medical Center, 3Department of Cardiology, The Sakakibara Heart Institute of Okayama, 4Department of Clinical Pathology, Kameda Medical Center, 5Department of Cardiovascular Surgery, Kameda Medical Center

キーワード : tricuspid regurgitation, carcinoid heart disease, cardiac surgery, echocardiography

症例は70歳女性.2年前に原発性卵巣腫瘍に対して開腹下付属器摘出術を施行され,病理所見から卵巣カルチノイドと確定診断された.その頃より重症三尖弁逆流症が指摘されていたが,自覚症状がなかったため経過観察されていた.入院1ヵ月前より下肢浮腫増強があり,利尿剤を開始したが改善せず,全身浮腫のため入院となった.入院5年前の心エコー図では三尖弁逆流症,肺動脈弁逆流症ともにほとんど認められなかったが,入院時の心エコー図では三尖弁,肺動脈弁ともに強い肥厚と短縮,可動性の低下を伴い,重症三尖弁逆流症,重症肺動脈弁逆流症,右房・右室拡大を認めた.利尿剤を増量したが心不全のコントロールがつかず,入院30日目に三尖弁置換術を施行した.術後,一時は車椅子移乗まで改善したが,その後敗血性ショックを来して入院39日目に永眠された.手術所見および剖検所見では三尖弁弁葉および弁下組織は高度に肥厚し,かつ短縮,硬化所見あり,弁尖の可動性は高度に低下していた.肺動脈弁も同様の所見であった.病理検査ではいずれの弁尖にも筋繊維芽細胞,平滑筋細胞,粘液基質の沈着を認め,カルチノイドによる変化と診断された.今回我々は明らかな症状が発現した後に急速な経過で不幸な転帰をたどった1例を経験した.一次性三尖弁逆流は不可逆性であり,症状発現後は病状が比較的急速に悪化することもある.症状の発現に注意し,適切な時期に手術を検討する必要が示唆された.

This case is a 70-year-old woman. Two years ago, she underwent surgery for a primary ovarian tumor, which was diagnosed as an ovarian carcinoid tumor based on the pathological findings. Asymptomatic severe tricuspid regurgitation was observed at that time. No medication was prescribed. One month prior to admission, she started to take diuretics for leg edema, and she was admitted to our hospital because of anasarca. Echocardiogram on admission demonstrated severely thickened and shortened tricuspid and pulmonary valves with severe regurgitation. The mobility was restricted. The right atrium and the right ventricle were dilated. She underwent tricuspid valve replacement on the 30th hospital day because of intractable heart failure. After the surgery, the symptoms and activity level improved to the level of using a wheelchair. However, she passed away because of septic shock on the 39th hospital day. Autopsy showed highly thickened, shortened and hardened tricuspid valve leaves and subvalvular tissue. Mobility of the leaflets was highly reduced. The pulmonary valve had similar findings. Pathological examination revealed deposition of myofibroblasts, smooth muscle cells, and mucus substrate in leaflets of both valves, and this denaturalization was diagnosed as carcinoid heart disease. We have experienced an unfortunate case that progressed rapidly after the obvious symptoms appeared. Primary tricuspid regurgitation is irreversible, and it might deteriorate rapidly. We need to pay attention to any signs of the symptoms, and we need to consider surgery at an appropriate time.