Valve-sparing aortic root replacement (David I procedure) in Marfan disease: single-centre 20-year experience in more than 100 patients†

Replacement of the aortic root with a prosthetic graft and valve in patients with Marfan's syndrome may prevent premature death from rupture of an aneurysm or aortic dissection. We reviewed the results of this surgical procedure at 10 experienced surgical centers. A total of 675 patients with Marfan's syndrome underwent replacement of the aortic root. Survival and morbidity-free survival curves were calculated, and risk factors were determined from a multivariable regression analysis. The 30-day mortality rate was 1.5 percent among the 455 patients who underwent elective repair, 2.6 percent among the 117 patients who underwent urgent repair (within 7 days after a surgical consultation), and 11.7 percent among the 103 patients who underwent emergency repair (within 24 hours 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 deaths (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 after surgery, then rapidly decreased to a constant level by the end of the first year. Elective aortic-root replacement has a low operative mortality. In contrast, emergency repair, usually for acute aortic dissection, is associated with a much higher early mortality. Because nearly half the adult patients with aortic dissection had an aortic-root diameter of 6.5 cm or less at the time of operation, it may be prudent to undertake prophylactic repair of aortic aneurysms in patients with Marfan's syndrome when the diameter of the aorta is well below that size.

Surgical treatment of aortic root aneurysm in Marfan syndrome (MFS) patients. To compare results of total root replacement versus valve-sparing aortic root replacement in MFS patients. PubMed, Embase and Cochrane library were searched from January 1966 until February 2010 looking for papers reporting on aortic root operations in MFS patients. 530 studies were retrieved. Finally, 11 publications were enrolled. Inclusion criteria were observational studies reporting valve-related morbidity and mortality after total root replacement (TTR) and/or valve-sparing root replacement (VSRR) in patients with MFS and study size n≥30, reflecting the centre's experience. Data obtained from papers reporting both TRR and VSRR cohorts were analysed separately. In case of multiple publications, the most recent and complete report was selected. If the total number of patient-years was not provided, we calculated it by multiplying the number of hospital survivors with the mean follow-up duration of that study. Overall, 1,385 patients were analysed (972 patients had TTR and 413 patients had VSRR). Reintervention rate was 0.3%/year (95% CI 0.1 to 0.5) versus 1.3%/year (95% CI 0.3 to 2.2) (p=0.02) and thromboembolic events rate was 0.7%/year (95% CI 0.5 to 0.9) versus 0.3%/year (95% CI 0.1 to 0.6) (p=0.01) after TRR and VSRR, respectively. When composite valve-related events were compared, no difference existed between the two surgical strategies (p=0.41). Among patients undergoing VSRR, reimplantation was associated with a reduced rate of reintervention compared with remodelling (0.7%/year vs 2.4%/year, p=0.02). VSRR may represent a valuable option for patients with MFS with aortic aneurysm. However, this technique should be used with caution in patients with valve characteristics at risk for decreased durability.