Key result
Prophylactic aortic root repair in Marfan syndrome links to better long-term outcomes than post-dissection repair.
Why the study?
Does prophylactic aortic root repair improve survival and freedom from adverse events compared to repair after dissection in patients with Marfan syndrome?
Observational (n=50)
Does prophylactic aortic root repair improve survival and freedom from adverse events compared to repair after dissection in patients with Marfan syndrome?
Prophylactic aortic root repair in Marfan syndrome reduces overall adverse events compared to repair after dissection, though it does not guarantee prevention of future distal dissections.
Supports prophylactic repair in Marfan syndrome; leaves open need for lifelong distal surveillance.
We reviewed 50 patients with Marfan syndrome who underwent surgery for aortic root pathologies comprising a root aneurysm without (n = 25; group A) and with (n = 25; group B) dissection. Aortic root repair included Bentall (n = 37) and valve-sparing (n = 13) procedures. Hospital mortality was 4.0%. Twenty-two patients required 36 repeat surgeries on the distal aorta. The main indication for re-intervention was the dilation of the false lumen. In group A, the distal aorta was stable for up to 7 years, but new dissection developed in 5 (33.3%) of the 15 patients who were followed up for >7 years after the root repair. Actuarial survival including operative mortality was 88.1 and 65.0% at 10 and 20 years, respectively; groups A and B did not significantly differ. Rates of freedom from all-cause death, new dissection or repeated aortic surgery were 60.1, 44.5 and 26.0% at 5, 10 and 15 years, respectively. Group A was significantly better than group B. Prophylactic aortic root repair apparently reduces the likelihood of overall adverse events, but it cannot guarantee the prevention of further aortic dissection. A multidisciplinary approach is needed for patients with Marfan syndrome.
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Shimizu et al. (2011) conducted an observational in Marfan syndrome with aortic root pathologies (n=50). Prophylactic aortic root repair (without dissection) vs. Aortic root repair with dissection was evaluated on Freedom from all-cause death, new dissection or repeated aortic surgery. Prophylactic aortic root repair in Marfan syndrome without dissection had significantly better long-term outcomes than repair with dissection, though 33.3% developed new dissections after 7 years.
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