"standing of the genetic nature of the disease by the medical community, as well as increased availability of genetic testing , which is used more and more . Echocardiography is now readily available and aortic root follow-up is standardized , with clear recommendations being issued  . Medical care has been standardized, with an indication for beta-blockade in all patients with MFS, or calcium antagonists  when beta-blockers are not tolerated. "
[Show abstract][Hide abstract] ABSTRACT: To evaluate the evolution of surgical management in a large population of patients with Marfan syndrome.
This is a retrospective study of patients fulfilling the Ghent criteria for Marfan syndrome, who visited the Centre de référence national pour le syndrome de Marfan et apparentés and underwent a surgical event before or during follow-up in the centre.
One thousand and ninety-seven patients with Marfan syndrome, according to international criteria, came to the clinic between 1996 and 2010. Aortic surgery was performed in 249 patients (22.7%; 20 children and 229 adults), including the Bentall procedure in 140 patients (56%) and valve-sparing surgery in 88 patients (35%); a supracoronary graft was performed in 19 patients (7.6%), usually for aortic dissection. During the past 20 years, the predominant reason for aortic surgery has switched from aortic dissection to aortic dilatation, while age at surgery has tended to increase (from 32.4 ± 11.9 years to 35.2 ± 12.4 years; P=0.075). Mitral valve surgery was performed in 61 patients (5.6%; six children and 55 adults), including 37 valvuloplasties (60.6%) and 18 mitral valve replacements (29.5%). No significant difference was observed when comparing mitral valve surgery before and after 2000.
Surgery performed in patients with Marfan syndrome has switched from emergency surgery for aortic dissection to elective surgery for aortic dilatation; this is associated with surgery performed at an older age despite the indication for surgery having decreased from 60mm to 50mm. No significant evolution was observed for mitral valve surgery.
[Show abstract][Hide abstract] ABSTRACT: Aneurysms of the ascending aorta carry a high risk of aortic dissection or rupture in the absence of surgical treatment.
Aneurysms of the ascending aorta are classified into 2 distinct types according to their etiology and surgical treatment: aortic root aneurysms, in which the dilatation of the aorta involves the initial portion of the aorta and includes the sinuses of Valsalva and supravalvular aortic aneurysms, which involve the ascending thoracic aorta above the sinuses of Valsalva.
Echocardiography and CT scan are the main exams in the evaluation of the diameter of the ascending aorta and its follow up.
Conservative treatment by par ß-blockers and follow up by echocardiography once or twice a year is recommended for asymptomatic patients. ß-blockers have demonstrated a slowing effect on dilatation of the aortic root and improve survival especially in patients with Marfan's syndrome.
Beyond 50 mm, an aneurysm is at risk for complications. Supravalvular aortic aneurysms can be treated by a simple supracoronary tube graft unlike aortic root aneurysms in which replacement or repair of the aortic valve is often necessary.
A family history and investigation of other members is essential in patients with aortic aneurysms.
La Presse Médicale 01/2010; 39(1):26-33. DOI:10.1016/j.lpm.2009.01.026 · 1.08 Impact Factor
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