Ehlers-Danlos症候群による大動脈弁輪拡張症に対する大動脈基部置換術の1例
症例は33歳,女性.23歳時に皮膚生検でEhlers-Danlos症候群と診断されていた.大動脈弁輪拡張症による大動脈弁閉鎖不全症ならびに上行大動脈瘤に対して大動脈基部置換術を施行した.手術の工夫として,末梢側吻合は残存大動脈に対する物理的侵襲を避けるために左大腿動脈送血による低体温循環停止・脳分離体外循環としてopen distal anastomosisとした.さらに中枢側吻合は大動脈壁を利用して大動脈弁輪を補強した.無輸血で手術を終了し,術後経過は良好であった.組織脆弱性がみられるEhlers-Danlos症候群においても,術式の工夫などで安全に開心術を行いうると考えられた....
Saved in:
| Published in | 日本心臓血管外科学会雑誌 Vol. 31; no. 4; pp. 288 - 291 |
|---|---|
| Main Authors | , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
特定非営利活動法人 日本心臓血管外科学会
15.07.2002
|
| Subjects | |
| Online Access | Get full text |
| ISSN | 0285-1474 1883-4108 |
| DOI | 10.4326/jjcvs.31.288 |
Cover
| Abstract | 症例は33歳,女性.23歳時に皮膚生検でEhlers-Danlos症候群と診断されていた.大動脈弁輪拡張症による大動脈弁閉鎖不全症ならびに上行大動脈瘤に対して大動脈基部置換術を施行した.手術の工夫として,末梢側吻合は残存大動脈に対する物理的侵襲を避けるために左大腿動脈送血による低体温循環停止・脳分離体外循環としてopen distal anastomosisとした.さらに中枢側吻合は大動脈壁を利用して大動脈弁輪を補強した.無輸血で手術を終了し,術後経過は良好であった.組織脆弱性がみられるEhlers-Danlos症候群においても,術式の工夫などで安全に開心術を行いうると考えられた. |
|---|---|
| AbstractList | 症例は33歳,女性.23歳時に皮膚生検でEhlers-Danlos症候群と診断されていた.大動脈弁輪拡張症による大動脈弁閉鎖不全症ならびに上行大動脈瘤に対して大動脈基部置換術を施行した.手術の工夫として,末梢側吻合は残存大動脈に対する物理的侵襲を避けるために左大腿動脈送血による低体温循環停止・脳分離体外循環としてopen distal anastomosisとした.さらに中枢側吻合は大動脈壁を利用して大動脈弁輪を補強した.無輸血で手術を終了し,術後経過は良好であった.組織脆弱性がみられるEhlers-Danlos症候群においても,術式の工夫などで安全に開心術を行いうると考えられた. |
| Author | 熊野, 浩 山口, 明満 丸橋, 裕之 加藤, 聡 木地, 達也 |
| Author_xml | – sequence: 1 fullname: 熊野, 浩 organization: 医真会八尾総合病院心臓センター外科 – sequence: 1 fullname: 山口, 明満 organization: 医真会八尾総合病院心臓センター外科 – sequence: 1 fullname: 木地, 達也 organization: 現奈良県立医科大学第3外科 – sequence: 1 fullname: 加藤, 聡 organization: 医真会八尾総合病院心臓センター外科 – sequence: 1 fullname: 丸橋, 裕之 organization: 医真会八尾総合病院心臓センター外科 |
| BookMark | eNpVkD1Lw1AYhS9SwVq7-TdS896P3GSUWj-g4KJzuEnutQ2xlaQIbqYKFjI4iEhxkEJBUaiTi8T-mZi2_guLFcHlnOE8nOFZRYVWuyURWge9Qgk2NnzfPY0qBCrYNJdQEUyTaBR0s4CKOjaZBpTTFVSOoqaj69jgoAMtIqfWCGQYaVuiFbSj6d1Vft6fjodZ_JJ1e1k3yYePeXI7u-zlaTxLnyfJIE_f5tgcyF_HWdz_xzy8f108TT9Gk-v72eAmi0fwOU7W0LISQSTLv11Ch9u1g-quVt_f2atu1jUfLEPXHKIMYNy1PMUlA9dUTBgeo4YCz7I8SlwuBQeFMXNcIhX3MHYsgglw5jmGR0qotvj1o444kvZJ2DwW4Zktwk7TDaT9IwgszmwCNl3E3NXf7jZEaPuCfANEkoMD |
| ContentType | Journal Article |
| Copyright | 特定非営利活動法人 日本心臓血管外科学会 |
| Copyright_xml | – notice: 特定非営利活動法人 日本心臓血管外科学会 |
| DOI | 10.4326/jjcvs.31.288 |
| DatabaseTitleList | |
| DeliveryMethod | fulltext_linktorsrc |
| Discipline | Medicine |
| EISSN | 1883-4108 |
| EndPage | 291 |
| ExternalDocumentID | article_jjcvs1975_31_4_31_4_288_article_char_ja |
| GroupedDBID | ALMA_UNASSIGNED_HOLDINGS JSF KQ8 OK1 RJT |
| ID | FETCH-LOGICAL-j1960-b3f6157c9df7e51c8f5a6d546f1d99d43c7ea71f225bc3ef7d22b9323175db6d3 |
| ISSN | 0285-1474 |
| IngestDate | Wed Sep 03 06:13:01 EDT 2025 |
| IsDoiOpenAccess | true |
| IsOpenAccess | true |
| IsPeerReviewed | true |
| IsScholarly | true |
| Issue | 4 |
| Language | Japanese |
| LinkModel | OpenURL |
| MergedId | FETCHMERGED-LOGICAL-j1960-b3f6157c9df7e51c8f5a6d546f1d99d43c7ea71f225bc3ef7d22b9323175db6d3 |
| OpenAccessLink | https://www.jstage.jst.go.jp/article/jjcvs1975/31/4/31_4_288/_article/-char/ja |
| PageCount | 4 |
| ParticipantIDs | jstage_primary_article_jjcvs1975_31_4_31_4_288_article_char_ja |
| PublicationCentury | 2000 |
| PublicationDate | 2002/07/15 |
| PublicationDateYYYYMMDD | 2002-07-15 |
| PublicationDate_xml | – month: 07 year: 2002 text: 2002/07/15 day: 15 |
| PublicationDecade | 2000 |
| PublicationTitle | 日本心臓血管外科学会雑誌 |
| PublicationTitleAlternate | 日心外会誌 |
| PublicationYear | 2002 |
| Publisher | 特定非営利活動法人 日本心臓血管外科学会 |
| Publisher_xml | – name: 特定非営利活動法人 日本心臓血管外科学会 |
| References | 3) Takahashi, T., Koide, T., Yamaguchi, H. et al.: Ehlers-Danlos syndrome with aortic regurgitation, dilation of the sinuses of Valsalva, and abnormal dermal collagen fibrils. Am. Heart J. 123: 1709-1712, 1992. 4) Tiller, G. E., Cassidy, S. B., Wensel, C. et al.: Aortic root dilatation in Ehlers-Danlos syndrome types I, II and III. A report of five cases. Clin. Genet. 53: 460-465, 1998. 14) Luciani, G. B., Casali, G., Tomezzoli, A. et al.: Recurrence of aortic insufficiency after aortic root remodeling with valve preservation. Ann. Thorac. Surg. 67: 1849-1852, 1999. 8) 高沢有史,橋本明政,青見茂之ほか:Ehlers-Danlos症候群における上行弓部,胸部下行大動脈人工血管置換術の1治験例.日胸外会誌43:1850-1853,1995. 11) Tucker, D. H., Miller, D. E. and Jacoby, W. J., Jr.: Ehlers-Danlos syndrome with a sinus of Valsalva aneurysm and aortic insufficiency simulating rheumatic heart disease. Am. J. Med. 35: 715-720, 1963. 5) Lichtenstein, J. R. and McKusick, V. A.: Ehlers-Danlos syndrome with aortic aneurysm. Birth Defects Orig. Artic. Ser. 11: 340, 1975. 16) Robicsek, F., Daugherty, H. K., Mullen, D. C. et al.: Long-range observations with external aortic grafts. J. Cardiovasc. Surg. (Torino) 17: 195-201, 1976. 13) Kamohara, K., Itoh, T., Natsuaki, M. et al.: Early valve failure after aortic valve-sparing root reconstruction. Ann. Thorac. Surg. 68: 257-259, 1999. 6) Hamano, K., Minami, Y., Fujimura, Y. et al.: Emergency operation for thoracic aortic aneurysm caused by the Ehlers-Danlos syndrome. Ann. Thorac. Surg. 58: 1180-1182, 1994. 1) Byers, P. H.: Ehlers-Danlos syndrome: recent advances and current understanding of the clinical and genetic heterogeneity. J. Invest. Dermatol. 103: 47s-52s, 1994. 12) Simon, A. P. and Stein, P. D.: Aortic insufficiency in Ehlers-Danlos syndrome. Angiology 25: 290-296, 1974. 2) Raman, J., Saldanha, R. F., Esmore, D. S. et al.: The Bentall procedure: a surgical option in Ehlers-Danlos syndrome. J. Cardiovasc. Surg. 29: 647-649, 1988. 7) Conte, S., Serraf, A., Lacour-Gayet, F. et al.: Successful repair of thoracic aortic aneurysm in a child with Ehlers-Danlos syndrome. J. Thorac. Cardiovasc. Surg. 113: 410-411, 1997. 15) Dhillon, J. S., Randhawa, G. K., Straehley, C. J. et al.: Late rupture after Dacron wrapping of aortic aneurysms. Circulation 74 (Suppl. I): I-11-I-14, 1986. 9) Ascione, R., Gomes, W. J., Bates, M. et al.: Emergency repair of type A aortic dissection in type IV Ehlers-Danlos syndrome. Cardiovasc. Surg. 8: 75-78, 2000. 10) Babatasi, G., Massetti, M., Bhoyroo, S. et al.: Pregnancy with aortic dissection in Ehlers-Danlos syndrome. Staged replacement of the total aorta (10-year follow-up). Eur. J. Cardio-thorac. Surg. 12: 671-674, 1997. |
| References_xml | – reference: 2) Raman, J., Saldanha, R. F., Esmore, D. S. et al.: The Bentall procedure: a surgical option in Ehlers-Danlos syndrome. J. Cardiovasc. Surg. 29: 647-649, 1988. – reference: 6) Hamano, K., Minami, Y., Fujimura, Y. et al.: Emergency operation for thoracic aortic aneurysm caused by the Ehlers-Danlos syndrome. Ann. Thorac. Surg. 58: 1180-1182, 1994. – reference: 13) Kamohara, K., Itoh, T., Natsuaki, M. et al.: Early valve failure after aortic valve-sparing root reconstruction. Ann. Thorac. Surg. 68: 257-259, 1999. – reference: 4) Tiller, G. E., Cassidy, S. B., Wensel, C. et al.: Aortic root dilatation in Ehlers-Danlos syndrome types I, II and III. A report of five cases. Clin. Genet. 53: 460-465, 1998. – reference: 1) Byers, P. H.: Ehlers-Danlos syndrome: recent advances and current understanding of the clinical and genetic heterogeneity. J. Invest. Dermatol. 103: 47s-52s, 1994. – reference: 10) Babatasi, G., Massetti, M., Bhoyroo, S. et al.: Pregnancy with aortic dissection in Ehlers-Danlos syndrome. Staged replacement of the total aorta (10-year follow-up). Eur. J. Cardio-thorac. Surg. 12: 671-674, 1997. – reference: 14) Luciani, G. B., Casali, G., Tomezzoli, A. et al.: Recurrence of aortic insufficiency after aortic root remodeling with valve preservation. Ann. Thorac. Surg. 67: 1849-1852, 1999. – reference: 7) Conte, S., Serraf, A., Lacour-Gayet, F. et al.: Successful repair of thoracic aortic aneurysm in a child with Ehlers-Danlos syndrome. J. Thorac. Cardiovasc. Surg. 113: 410-411, 1997. – reference: 16) Robicsek, F., Daugherty, H. K., Mullen, D. C. et al.: Long-range observations with external aortic grafts. J. Cardiovasc. Surg. (Torino) 17: 195-201, 1976. – reference: 9) Ascione, R., Gomes, W. J., Bates, M. et al.: Emergency repair of type A aortic dissection in type IV Ehlers-Danlos syndrome. Cardiovasc. Surg. 8: 75-78, 2000. – reference: 11) Tucker, D. H., Miller, D. E. and Jacoby, W. J., Jr.: Ehlers-Danlos syndrome with a sinus of Valsalva aneurysm and aortic insufficiency simulating rheumatic heart disease. Am. J. Med. 35: 715-720, 1963. – reference: 15) Dhillon, J. S., Randhawa, G. K., Straehley, C. J. et al.: Late rupture after Dacron wrapping of aortic aneurysms. Circulation 74 (Suppl. I): I-11-I-14, 1986. – reference: 12) Simon, A. P. and Stein, P. D.: Aortic insufficiency in Ehlers-Danlos syndrome. Angiology 25: 290-296, 1974. – reference: 8) 高沢有史,橋本明政,青見茂之ほか:Ehlers-Danlos症候群における上行弓部,胸部下行大動脈人工血管置換術の1治験例.日胸外会誌43:1850-1853,1995. – reference: 5) Lichtenstein, J. R. and McKusick, V. A.: Ehlers-Danlos syndrome with aortic aneurysm. Birth Defects Orig. Artic. Ser. 11: 340, 1975. – reference: 3) Takahashi, T., Koide, T., Yamaguchi, H. et al.: Ehlers-Danlos syndrome with aortic regurgitation, dilation of the sinuses of Valsalva, and abnormal dermal collagen fibrils. Am. Heart J. 123: 1709-1712, 1992. |
| SSID | ssib002671014 ssib005879759 ssib002670241 ssib002223857 ssib023160781 ssib000936894 ssib058493921 ssj0069030 |
| Score | 1.58125 |
| Snippet | 症例は33歳,女性.23歳時に皮膚生検でEhlers-Danlos症候群と診断されていた.大動脈弁輪拡張症による大動脈弁閉鎖不全症ならびに上行大動脈瘤に対して大動脈基部置換術を施... |
| SourceID | jstage |
| SourceType | Publisher |
| StartPage | 288 |
| SubjectTerms | Ehlers-Danlos症候群 大動脈基部置換術 大動脈弁輪拡張症 |
| Title | Ehlers-Danlos症候群による大動脈弁輪拡張症に対する大動脈基部置換術の1例 |
| URI | https://www.jstage.jst.go.jp/article/jjcvs1975/31/4/31_4_288/_article/-char/ja |
| Volume | 31 |
| hasFullText | 1 |
| inHoldings | 1 |
| isFullTextHit | |
| isPrint | |
| ispartofPNX | 日本心臓血管外科学会雑誌, 2002/07/15, Vol.31(4), pp.288-291 |
| journalDatabaseRights | – providerCode: PRVAFT databaseName: Open Access Digital Library customDbUrl: eissn: 1883-4108 dateEnd: 99991231 omitProxy: true ssIdentifier: ssj0069030 issn: 0285-1474 databaseCode: KQ8 dateStart: 19750101 isFulltext: true titleUrlDefault: http://grweb.coalliance.org/oadl/oadl.html providerName: Colorado Alliance of Research Libraries |
| link | http://utb.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwtV1LaxRBEB5CBPEiPvFNDvZJZt3e7pnpvggzm1mCEkFIILdlnuAiiZjEgyc3CgYW9CAiwYMEAopCPHmRmD-zbhL_hV93zya94iFRhGXo7amu-qpqZrqq6YfjXC-lyPDZy9wgT0qXB0hQ0iBlLk3TelEIkeSlWo08fdefmuW357y5sfGX1qyl5aW0lj3547qSv_Eq6uBXtUr2CJ7dZ4oKlOFfXOFhXA_l41gdXbzownMPFhZJHBAZECT3safnL0hVE8Uk5CRmiBhJGOlCgwgxLESKGAShaRUR6ZFYEME1jUeipmqIGhTCkMS-oglpdSvyLKH7IsCwpeSaGgXjcLIkWkGEJIKRUGjwkySMtdAWkZEihmjJhrJi4OBKkojsGFs1AKbQ0wXgbmq4LcVXyQs0C80LZoIYyDAqAZz0dQ1oTA0Q-FpKk0gNDjiktgjMIfaHmFUjAZwGP8p6kqkCABuF8oAMQKj6KQu0SMiGZFKoNooebOsH9EYDYx8YvG7opbYs18AAqWXzB4awrgAqG3BDL7S96MgQj54ObBa56pdSqyAVP6V3PFR3kshYSwc0X_OH6lIjhfDIciVqmC5wpUQU3vh_jrC6r4bwXMrNGUy1wnSvQjCX07qw-98qCrhvDy6ZztQc-FjFZQ1zqtvvXT5n-pDSTid7vFhjtLbfaGQT9eoVbWsyKgOvzWibmwtatIf31VLHdgf51rEGogR1FMyde1a6IpkvRoZDEPB69nZ8AUJeav-n9iwFTwTS2l4TqZVv77WFXEAyvT2hiSR9WWdmjLiypFm4ozS-aeuL4LmDVHI4DVVHxjOnnJNVSjsRGuVOO2Od5IxzfLqatHPWSUc-U7tvXwyeru1ub_S7n_srq_2V3mDjw6D3Zu_56mCru7f1aae3Ptj6CjIQDL5s97trIzTvv_189nH3--bOq3d766_73U36Y7t3zpltxTPNKbc62sXtoMtHf8BKpFJBJvMyKDyaidJL_NzjfklzKXPOsqBIAloi2kgzVpRB3mikSDVVtpOnfs7OO-PzC_PFBWdCopNJuO-VaYNymvKkQA6S5IVf-FlW1vlF55axTvuh2b-nfcSH4dK_MrjsnDh4q68440uPlourSGWW0mv6-foFqBQG6w |
| linkProvider | Colorado Alliance of Research Libraries |
| openUrl | ctx_ver=Z39.88-2004&ctx_enc=info%3Aofi%2Fenc%3AUTF-8&rfr_id=info%3Asid%2Fsummon.serialssolutions.com&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Ehlers-Danlos%E7%97%87%E5%80%99%E7%BE%A4%E3%81%AB%E3%82%88%E3%82%8B%E5%A4%A7%E5%8B%95%E8%84%88%E5%BC%81%E8%BC%AA%E6%8B%A1%E5%BC%B5%E7%97%87%E3%81%AB%E5%AF%BE%E3%81%99%E3%82%8B%E5%A4%A7%E5%8B%95%E8%84%88%E5%9F%BA%E9%83%A8%E7%BD%AE%E6%8F%9B%E8%A1%93%E3%81%AE1%E4%BE%8B&rft.jtitle=%E6%97%A5%E6%9C%AC%E5%BF%83%E8%87%93%E8%A1%80%E7%AE%A1%E5%A4%96%E7%A7%91%E5%AD%A6%E4%BC%9A%E9%9B%91%E8%AA%8C&rft.au=%E7%86%8A%E9%87%8E%2C+%E6%B5%A9&rft.au=%E5%B1%B1%E5%8F%A3%2C+%E6%98%8E%E6%BA%80&rft.au=%E6%9C%A8%E5%9C%B0%2C+%E9%81%94%E4%B9%9F&rft.au=%E5%8A%A0%E8%97%A4%2C+%E8%81%A1&rft.date=2002-07-15&rft.pub=%E7%89%B9%E5%AE%9A%E9%9D%9E%E5%96%B6%E5%88%A9%E6%B4%BB%E5%8B%95%E6%B3%95%E4%BA%BA+%E6%97%A5%E6%9C%AC%E5%BF%83%E8%87%93%E8%A1%80%E7%AE%A1%E5%A4%96%E7%A7%91%E5%AD%A6%E4%BC%9A&rft.issn=0285-1474&rft.eissn=1883-4108&rft.volume=31&rft.issue=4&rft.spage=288&rft.epage=291&rft_id=info:doi/10.4326%2Fjjcvs.31.288&rft.externalDocID=article_jjcvs1975_31_4_31_4_288_article_char_ja |
| thumbnail_l | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/lc.gif&issn=0285-1474&client=summon |
| thumbnail_m | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/mc.gif&issn=0285-1474&client=summon |
| thumbnail_s | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/sc.gif&issn=0285-1474&client=summon |