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A Three-years follow-up Case of Operated Aortic Regurgitation Secondary to Behçet's Disease:A case report and review of the literature Masafumi Enoki 1 , Toshinori Utsunomiya 1 , Takashi Tokushima 1 , Noriaki Suzuki 1 , Kohei Nagasawa 1 , Shuzo Matsuo 1 , Hisao Suda 2 , Masafumi Natsuaki 2 , Tsuyoshi Itoh 2 1Cardiology Division, Department of Internal Medicine, Saga Medical School 2Department of Cardiovascular Surgery, Saga Medical School Keyword: ベーチェット病 , 大動脈弁閉鎖不全症 , Behçet disease , aortic regurgitation pp.385-389
Published Date 1997/4/15
DOI https://doi.org/10.11477/mf.1404900031
  • Abstract
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A 40-year-old man was admitted with the chief com-plaint of dyspnea on Sep. 11, 1992. He had already been diagnosed as having an incomplete form of Behçet's disease, and salazosulfapyridine therapy has been continued. On auscultation, there were a grade III/VI diastolic high pitch decrescent murmur at the 2nd left sternal border and coarse crackles at the lower lung field. Laboratory data showed an increased white blood cell count of 11,800/mm3 and CRP of 5.3mg/ml. Moder-ate aortic regurgitation (AR) flow wasdetected by color Doppler and cine MRI. Sellers grade was III by DSA (digitized subtraction angiography). Steroid therapy was started. Because of intractable heart failure, aortic valve replacement (St Jude Medical Valve) was perfor-med on Oct 8, 1992. Operative findings showed thedestructed left coronary cusp and a fenestrated aneur-ysm of the left sinus of Valsalva. Pathological findings of aortic valve tissue showed fibrinoid degeneration, monocyte invasion and deposition of hemosiderin. Ten days later, however, AR of Sellers grade II reappeared. The second aortic valve replacement wasperformed on Nov. 2, 1992. After that, he had no symptoms. Labora-tory data showed normalized white bloodcell count and CRP. Steroid therapy was continued. For 3 years, he has had no cardiac symptoms. White blood cell has been 6,300-10,100/mm3. CRP was 0.36-4.53 mg/dl. We have reviewed 62 cases of Behçet's disease with AR in the literature. Most patients were male under the age of 50 years. Thirty-four cases (54%) underwent aortic valve replacement. An important complication was per- valvular leak. Steroid was used for long-term medical therapy in most cases. Only 4 cases survived over 2 years after aortic valve replacement. Careful control of inflammation may be required for long-term survival of Beheet's disease with AR.


Copyright © 1997, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1200 印刷版ISSN 0452-3458 医学書院

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