Marfan Syndrome Research - Genetics, Causes, Symptoms, Treatment

Marfan Syndrome Research Today is a free monthly online journal that collates and summarizes the latest research about Marfan Syndrome, including details on genetics, causes, symptoms, treatment.


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Chronic dissection of the ascending aorta: surgical results during a 20-year period (previous surgery excluded).

Jault F, Rama A, Lievre L, Bonnet N, Leprince P, Pavie A, Gandjbakhch I

Department of Cardiovascular and Thoracic Surgery, Hôpital de la Pitié Salpetrière, service du Pr Gandjbakhch, 83 boulevard de l'Hôpital, 75013 Paris, France. frederique.jault@psl.ap-hop-paris.fr

OBJECTIVE: We study here the surgical results of chronic dissection involving the ascending aorta over the last 20 years. Patients with previous cardiac surgery, or proximal aortic repair, were excluded. The patients survived an acute dissection, undiagnosed as pauci- or asymptomatic. The aorta was normal or pathological (atheromatous aneurysm in 15 cases, Marfan's disease in 12 cases, and annuloectasic disease in 18 cases). Two patients had a bicuspid aortic valve. METHODS: Between January 1981 and December 2001, 77 patients (mean age 48+/-15) underwent surgery for chronic dissection of the ascending aorta; 60 patients had severe aortic regurgitation, 12 had Marfan syndrome, and 18 had annuloaortic ectasia. Only the ascending aorta was dissected in 37 patients, the ascending aorta and arch in 26, and the whole aorta in 14. Coronary artery disease occurred in five patients. Statistical analysis was performed using SAS software. Different surgical procedures were used. The aortic arch was repaired in 40 cases; selective antegrade cerebral perfusion and partial circulatory arrest were used. Total aortic replacement was performed on four patients. RESULTS: In-hospital mortality was 10%. The only risk factor was the extent of the dissection. The rate of neurologic stroke was 2.5%. Late survival rate was 42+/-7.5% at 12 years for all the patients; it was 71+/-10% when only the ascending aorta was dissected, 44+/-11% when the ascending aorta and arch were dissected, and 33+/-15% when the whole aorta was dissected (p = 0.0329). The extent of the dissection was the only risk factor for late mortality. Reoperation was required for one proximal and five distal problems. CONCLUSION: In chronic aortic dissection, in-hospital and late mortality were related to the extent of the dissection; in-hospital mortality remained unchanged during the operative period.

Published 23 May 2006 in Eur J Cardiothorac Surg, 29(6): 1041-5.
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Marfan Syndrome Research Today Archive:

Volume 1 (2005)
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Volume 2 (2006)
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