[Medical management of Marfan's syndrome and ascending aortic aneurysms in France: initial assessment].

Over the last 30 years, there has been considerable progress in the management of aneurysms of the ascending aorta whether isolated or related to Marfan's syndrome which has considerably increased the life expectancy of these patients. The aim of this study was to assess the management protocol proposed in France for this condition in order to establish recommendations. A questionnaire was sent to cardiologists, paediatric cardiologists and cardiac surgeons and also to patients diagnosed with Marfan's syndrome. Most of the 228 physicians who replied prohibited violent sporting activities and less than a quarter advised against all sports. There was a wide dispersion in the prescription of beta blockers in Marfan's syndrome as 57% proposed this treatment only in cases with dilatation of the aorta and 5% prescribed no treatment at all. On the other hand, beta blockers were widely prescribed (93%) after replacement of the ascending aorta. When poorly tolerated in adults, the most commonly prescribed option was a calcium antagonist (60%) followed by angiotensin converting enzyme inhibitors and therapeutic abstention. On the other hand, 60% of paediatric cardiologists preferred to withdraw treatment. These results were confirmed by the replies from patients with Marfan's syndrome: 58.3% were prescribed beta blockers with a higher proportion in children less than 12 years of age. This treatment was introduced late, about 7 years after diagnosis. The authors conclude that there is much disparity in the management of ascending aortic aneurysms whether or not associated with Marfan's syndrome, especially with regards the prescription of beta blockers, justifying the publication of recommendations.