Management of aortic valve incompetence in patients with Marfan syndrome
Follwoing the original description of the skeletal manifestation of Marfan syndrome (1, 2), a relatively long time elapsed before recognition of the potentially lethal cardiovascular manifestations of the disease (3–5), and only recently has it become evident that these are the main determinants of prognosis of affected individuals (6). If uncorrected, these manifestations can lead to severe disability or death at a young age. Although prolapse of the mitral valve is the most common cardiovascular manifestation of the condition, pathology of the ascending aorta and root is frequently seen and is the most common manifestation requiring surgical intervention. Aortic regurgitation may result from aneurysmal dilitation or dissection. The purpose of this chapter is to describe the management of aortic regurgitation in Marfan syndrome with particular reference to the use of a valve-conserving operation combined with radical excision of the sinuses, a technique introduced by us 15 years ago, and the place of prophylactic surgical treatment which, in our view, is closely linked to valve repair.
KeywordsVortex CHROMe Hematoxylin Eosin Dura
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- 1.Marfan A-B (1896): Un cas de deformation congenitale des quatre membres plus prononcee aux extremites caracterisee par l’allongement des os avec un certain degre d’amincissement. Bull Mem Soc Med Hop Paris (ser 3) 13: 220–226Google Scholar
- 2.Marfan A-B (1938): La dolichostenomelie (dolichomelie, arachnodactylie). Ann Med 44: 5–29Google Scholar
- 4.Baer RW, Taussig HB, Oppenheimer EH (1943): Congenital aneurysmal dilatation of the aorta associated with arachnodactyly. Bull Johns Hopkins Hosp 72: 309–331Google Scholar
- 7.Jennifer Shores, Kenneth R Berger, Edmond A Murphy, Reed E Pyeritz (1994): Progression of aortic dilatation and the benefit of long-term B-adrenergic blockage in Marfan’s Syndrome.Google Scholar
- 9.Nicholas T Kouchoukos, Thomas H Wareing, suzan F Murphy, Johanna B Perrillo (1991): Sixteen-year experience with aortic root replacement. Annals of Surgery 308–320Google Scholar
- 11.Gula G, Ahmed M, Thompson R, Radley-Smith R, Yacoub M: Combined homograft replacement of the aortic valve and aortic root with reimplantation of the coronary arteries. Circulation 1976, 54: II 150.Google Scholar
- 12.Yacoub M, Rasmi N, Sundt T, Lund O, Boyland E, Radley-Smith R, Khaghani A, Mitchell A: Fourteen year experience with homovital homografts for aortic valve replacement. J Thor Cardiovasc Surg — manuscript submitted April 1994Google Scholar
- 13.Sundt TM, Rasmi N, Wong K, Radley-Smith R, Khaghani A, Yacoub M: Aortic valve reoperation following homograft root replacement: Surgical options and results. Annals of Thor Surgery — manuscript submitted July 1994.Google Scholar
- 14.14.Magdi Yacoub, Albert Fagan, Paolo Stassano, Rosemary Radley-Smith: Results of valve conserving operations for aortic regurgitation. Circulation 1983, 68: III, 111–321Google Scholar