Literature DB >> 10773564

Results of surgery for aortic root aneurysm in patients with the Marfan syndrome.

L Tambeur1, T E David, M Unger, S Armstrong, J Ivanov, G Webb.   

Abstract

OBJECTIVE: To evaluate the results of two operations, aortic valve-sparing and aortic root replacement, in patients with aortic root aneurysm and the Marfan syndrome.
METHODS: A retrospective review of 78 consecutive patients with aortic root aneurysm and the Marfan syndrome according to the Gent criteria indicated that 42 patients with normal aortic cusps had an aortic valve-sparing operation, and 36 patients had aortic root replacement (mechanical valve in 25 patients and biological valve in 11). The mean age was similar in both groups, but patients who had aortic root replacement had larger aneurysms, higher grade aortic insufficiency, worse left ventricular function and more cardiac co-morbidity than patients who had aortic valve-sparing operations. The mean follow-up was 41+/-35 months for patients who had aortic valve-sparing, and 65+/-50 months for those who had aortic root replacement.
RESULTS: Kaplan-Meyer estimates of survival at 5 years was 100% for patients who had aortic valve-sparing, and 88+/-6% for those who had aortic root replacement (P=0.04). Five patients who had aortic root replacement required seven aortic root re-replacements: three for endocarditis and four for valve failure (biological valves). There have been no reoperations in patients who had aortic valve-sparing operations and annual Doppler echocardiography revealed mild or no aortic insufficiency in 39 patients and moderate aortic insufficiency in three.
CONCLUSIONS: These data suggest that aortic valve-sparing operations are safe in patients with the Marfan syndrome and may provide better clinical outcomes than aortic root replacement. Since the size of the aneurysm often determines the feasibility of a valve-sparing procedure, we now recommend surgery when the diameter of the aortic root reaches 50 mm in patients with the Marfan syndrome who have echocardiographically normal aortic valve cusps.

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Year:  2000        PMID: 10773564     DOI: 10.1016/s1010-7940(00)00379-1

Source DB:  PubMed          Journal:  Eur J Cardiothorac Surg        ISSN: 1010-7940            Impact factor:   4.191


  4 in total

1.  Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: outflow tract obstruction, coarctation of the aorta, tetralogy of Fallot, Ebstein anomaly and Marfan's syndrome.

Authors:  Candice K Silversides; Marla Kiess; Luc Beauchesne; Timothy Bradley; Michael Connelly; Koichiro Niwa; Barbara Mulder; Gary Webb; Jack Colman; Judith Therrien
Journal:  Can J Cardiol       Date:  2010-03       Impact factor: 5.223

2.  Surgical treatment for cardiovascular lesions of patients with Marfan syndrome.

Authors:  Fumio Yamazaki; Mitsuomi Shimamoto; Shoji Fujita; Masanao Nakai; Tomoya Kono; Akihiro Aoyama; Fengshi Chen; Tomohiro Nakata
Journal:  Jpn J Thorac Cardiovasc Surg       Date:  2002-09

Review 3.  Large ascending aortic aneurysm and severe aortic regurgitation in a 7-year-old child with Marfan syndrome and a review of the literature. Marfan syndrome in childhood.

Authors:  Mehmet Yazici; Serdar Soydinç; Vedat Davutoğlu; Ilyas Akdemir; M Hakan Dinçkal
Journal:  Int J Cardiovasc Imaging       Date:  2004-08       Impact factor: 2.357

4.  Myocardial ischemia due to compression of an unruptured thoracic aortic aneurysm in a patient with Marfan syndrome.

Authors:  Hiroya Minami; Tatsuro Asada; Kunio Gan; Koichiro Abe; Satoshi Izumi
Journal:  Gen Thorac Cardiovasc Surg       Date:  2007-06
  4 in total

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