Replacement of the aortic root in patients with Marfan's syndrome

Background Replacement of the aortic root with a prosthetic graft and valve in patients with Marfan's syndrome may prevent premature death from ruptur e of an aneurysm or aortic dissection. We reviewed the results of this surg ical procedure at 10 experienced surgical centers. Methods A total of 675 patients with Marfan's syndrome underwent replacemen t of the aortic root. Survival and morbidity-free survival curves were calc ulated, and risk factors were determined from a multivariable regression an alysis. Results The 30-day mortality rate was 1.5 percent among the 455 patients wh o underwent elective repair, 2.6 percent among the 117 patients who underwe nt urgent repair (within 7 days after a surgical consultation), and 11.7 pe rcent among the 103 patients who underwent emergency repair (within 24 hour s after a surgical consultation). Of the 675 patients, 202 (30 percent) had aortic dissection involving the ascending aorta. Forty-six percent of the 158 adult patients with aortic dissection and a documented aortic diameter had an aneurysm with a diameter of 6.5 cm or less. There were 114 late deat hs (more than 30 days after surgery); dissection or rupture of the residual aorta (22 patients) and arrhythmia (21 patients) were the principal causes of late death. The risk of death was greatest within the first 60 days aft er surgery, then rapidly decreased to a constant level by the end of the fi rst year. Conclusions Elective aortic-root replacement has a low operative mortality. In contrast, emergency repair, usually for acute aortic dissection, is ass ociated with a much higher early mortality. Because nearly half the adult p atients with aortic dissection had an aortic-root diameter of 6.5 cm or les s at the time of operation, it may be prudent to undertake prophylactic rep air of aortic aneurysms in patients with Marfan's syndrome when the diamete r of the aorta is well below that size. (N Engl J Med 1999;340:1307-13.) (C )1999, Massachusetts Medical Society.