Dr K Niwa from Japan showed that medial abnormalities in the ascending Aorta are prevalent not only in Marfan syndrome, but also in a variety of other congenital heart disease (bicuspid aortic valve, coarctation of aorta, unrepaired and also repaired tetralogy of Fallot, pulmonary atresia etc.).
Typical histological changes (so called cystic medial necrosis, elastic fibre fragmentation, smooth muscle cell apoptosis) predispose to the dilatation of the aorta out of proportion to aortic aneurysm and to dissection.
Dr J. Roos-Hesselink from the Netherlands gave an excellent clinical overview. The most frequent causes of aortic dilatation and dissection were Marfan syndrome, bicuspid aortic valve and familial aortic aneurysms. These patients need a serial follow-up for prevention of complications using imaging modalities (CT, MRI, echo).
Patients with aortic dilatation, particularly with Marfan syndrome, may profit from medical prophylaxis (beta-blockers, probably also ACEI and A-II blockers). Surgical treatment is indicated for symptomatic aortic aneurysms. Asymptomatic patients should undergo surgery if the diameter of aortic root or ascending aorta exceeds 50 – 55 mm, or even earlier in Marfan syndrome and bicuspid aortic valve with a family history of aortic dissection or in women contemplating pregnancy. The risk of dissection increases dramatically at 60 mm or in the case of rapid progression of the dilatation ≥ 5 mm/year.
Dr O Oto from Turkey talked about the surgical techniques. The valve sparing surgery (re-implantation technique) seems to be preferable, if appropriate.
The last presentation was given by Dr C A Pedra from Brazil. He showed that besides the surgical option, there is an increased use of stenting of the aorta in adults with aortic coarctation or recoarctation. Even if no randomised multicentre studies exist, it may be a realistic solution for young adults.