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Dilatation of the aortic root leading to aortic dissection and rupture is the leading cause of premature death in patients with Marfan syndrome, which on average may shorten life expectancy by a third.1 ,2 Remarkably, over the last 40 years, life expectancy in Marfan syndrome has been restored to near normal through earlier recognition of affected patients, antihypertensive medical therapy and prophylactic aortic replacement. Prophylactic aortic root surgery in many surgical series from expert centres has carried a 1% risk or less of operative mortality.3 ,4 Furthermore, in the current era, valve preservation is usually possible, so most patients avoid valve prostheses and anticoagulation, although at the price of a median sternotomy and a short period of cardiopulmonary bypass, which are well tolerated in this young population.5 ,6
With such a high benchmark of surgical success, what is to be achieved by revisiting an approach to aneurysmal disease that has been abandoned by most cardiothoracic surgeons and …
Competing interests None.
Provenance and peer review Commissioned; internally peer reviewed.