Surgical therapy for Marfan Syndrome — Then and now

Bentall, H. H.

doi:10.1007/978-3-642-72508-1_6

Cited by 2 publications

(1 citation statement)

References 8 publications

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“…In cases with additional aortic valve defects and/or if the right or left coronary ostium originates from the aneurysmal area, a more complex repair seems advisable in order to avoid subsequent complications. Because of the particular conditions in our case, the repair was performed with replacement of the aortic valve and root using a composite graft employing a modified Bentall's technique [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

Sinus of valsalva aneurysm in Blau's syndrome

Mourad

Tang

2010

J Cardiothorac Surg

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Blau syndrome is a rare granulomatous disorder inherited in an autosomal dominant manner characterized by the early appearance of granulomatous arthritis, skin rash and anterior uveitis. There are very few data on the cardiovascular manifestations of Blau syndrome. Here we report the first case of sinus of valsava aneurysm in Blau syndrome. In isolated unruptured aneurysms of a sinus of Valsalva without compromise of the aortic valve and/or the coronary ostia, repair may be accomplished by simple placation of the aneurysm or excision of the aneurysm(s) and patch closure of the defect(s) between the aortic annulus and the sinu-vascular ridge. Because of the particular conditions in our case, the repair was performed with replacement of the aortic valve and root using a composite graft employing a modified Bentall's technique.

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Section: Discussionmentioning

confidence: 99%

Sinus of valsalva aneurysm in Blau's syndrome

Mourad

Tang

2010

J Cardiothorac Surg

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Early and Long-Term Results of a Valve-Sparing Operation for Marfan Syndrome

et al. 1999

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Background —We have previously described the experience, rationale, and development of a valve preserving technique, but its role in patients with Marfan syndrome has not previously been defined. Here, we attempt to determine the early and long-term results, timing, and determinants of outcome of this operation in patients with Marfan syndrome. Methods and Results —Since 1979, 82 patients (73.2% of all patients with Marfan syndrome undergoing resection of aneurysm of the ascending aorta) were operated on using this technique. Ages ranged from 2 to 69 years (mean, 33.9 years). In all, there were 4 early deaths (4.9%), 2 with acute dissection and 2 with chronic aneurysm operated on as emergencies. There were no early deaths in 67 patients operated on electively. Actuarial survival for patients operated for chronic aneurysm was 94.2%, 94.2%, and 94.2% at 1, 5, and 10 years, respectively; that for acute dissection was 72.7%, 63.6%, and 63.6%; and that for chronic dissection was 100%, 85.7%, and 75.0%. The probability of needing reoperation was 5.7%, 17.3%, and 17.3% at 1, 5, and 10 years. There were no instances of infective endocarditis or thromboembolic complications except in 2 patients operated on early in the series who had cusp extension. At the end of the follow-up, trivial or no aortic regurgitation was demonstrated in 33.3%, mild in 45.6%, moderate in 21.1%, and severe in 0. Conclusions —Valve-sparing operations are feasible in most patients with Marfan syndrome; they are applicable to patients with both dissection and chronic aneurysm. The early and long-term results are encouraging. Results are better in the absence of dissection, and prophylactic operation is warranted in some cases.

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Surgical therapy for Marfan Syndrome — Then and now

Cited by 2 publications

References 8 publications

Sinus of valsalva aneurysm in Blau's syndrome

Sinus of valsalva aneurysm in Blau's syndrome

Early and Long-Term Results of a Valve-Sparing Operation for Marfan Syndrome

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