{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T11:33:03Z","timestamp":1791286383055,"version":"4.1.0"},"reference-count":5,"publisher":"IMR Press","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["HSF"],"abstract":"<jats:p>Patients with Ehlers-Danlos syndrome (EDS) type IV, an inherited connective tissue disorder, are predisposed to vascular and digestive ruptures, and arterial ruptures account for the majority of deaths. A 31-year-old man with EDS presented with an intramural aortoatrial fistula, severe aortic regurgitation, mitral valve prolapse, and severe tricuspid valve insufficiency combined with a severely dilated left ventricle. Determining the best surgical option for the patient was not easy, especially regarding the course of action for the aortic root with a tear in the sinus of Valsalva. The fistula tract was closed at the aorta with suture and with a patch in the right atrium, the mitral valve was repaired with edge-to-edge suture and then annuloplasty with a Cosgrove ring, the aortic valve was replaced with a mechanical prosthesis, and a modified De Vega technique was used for the tricuspid valvuloplasty. The postoperative course was uncomplicated, and the patient was discharged 2 weeks later. The considerations made to arrive at the chosen surgical course of action in this complex case are reviewed.<\/jats:p>","DOI":"10.1532\/hsf98.20111133","type":"journal-article","created":{"date-parts":[[2012,6,15]],"date-time":"2012-06-15T22:54:46Z","timestamp":1339800886000},"page":"156","source":"Crossref","is-referenced-by-count":2,"title":["Successful Surgical Treatment of Intramural Aortoatrial Fistula, Severe Aortic Regurgitation, Mitral Prolapse, and Tricuspid Insufficiency in a Patient with Ehlers-Danlos Syndrome Type IV"],"prefix":"10.31083","volume":"15","author":[{"given":"Shengli","family":"Jiang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Changqing","family":"Gao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chonglei","family":"Ren","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tao","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"16242","published-online":{"date-parts":[[2012,6,14]]},"reference":[{"key":"1","doi-asserted-by":"crossref","first-page":"1210","DOI":"10.1067\/mtc.2001.111644","article-title":"Surgical treatment of multiple aneurysms in a patient with Ehlers-Danlos syndrome","volume":"121","author":"N. Oka","year":"2001","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1056\/NEJM200003093421001","article-title":"Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type","volume":"342","author":"M. Pepin","year":"2000","journal-title":"N Engl J Med"},{"key":"3","doi-asserted-by":"crossref","first-page":"308","DOI":"10.1111\/j.1610-0387.2006.05958.x","article-title":"Ehlers-Danlos syndrome-20 years experience with diagnosis and classification at the university skin clinic of Heidelberg [in German]","volume":"4","author":"S. Proske","year":"2006","journal-title":"J Dtsch Dermatol Ges"},{"key":"4","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1016\/j.athoracsur.2009.06.033","article-title":"Successful surgical treatment of atrial fibrillation, mitral regurgitation, and aortic root aneurysm in a patient with classical type Ehlers-Danlos syndrome","volume":"89","author":"M. Sauer","year":"2010","journal-title":"Ann Thorac Surg"},{"key":"5","doi-asserted-by":"crossref","first-page":"680","DOI":"10.1016\/j.jtcvs.2004.07.041","article-title":"Acute mitral regurgitation caused by papillary muscle rupture in the immediate postpartum period revealing Ehlers-Danlos syndrome type IV","volume":"129","author":"P. S\u00e8ve","year":"2005","journal-title":"J Thorac Cardiovasc Surg"}],"container-title":["The Heart Surgery Forum"],"original-title":[],"link":[{"URL":"http:\/\/journal.hsforum.com\/index.php\/HSF\/article\/viewFile\/80\/79","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,2]],"date-time":"2025-10-02T18:22:30Z","timestamp":1759429350000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.imrpress.com\/journal\/HSF\/15\/3\/10.1532\/HSF98.20111133"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,6,14]]},"references-count":5,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2012,6,1]]}},"URL":"https:\/\/doi.org\/10.1532\/hsf98.20111133","relation":{},"ISSN":["1522-6662","1098-3511"],"issn-type":[{"value":"1522-6662","type":"electronic"},{"value":"1098-3511","type":"print"}],"subject":[],"published":{"date-parts":[[2012,6,14]]}}}