Surgical Management of Aortic Root Disease in Marfan Syndrome: A Systematic Review and Meta-Analysis

Study Questions:

How do the results of total root replacement compare with valve-sparing aortic root replacement in patients with Marfan syndrome?


A meta-analysis was performed, searching PubMed, Embase, and Cochrane library for papers published between January 1966 and February 2010 reporting on aortic root operations in patients with Marfan syndrome. Of the 530 studies retrieved, 11 were utilized (observational studies with sample size ≥30 that reported valve-related morbidity and mortality after total root replacement and/or valve-sparing root replacement in patients with Marfan syndrome). The two surgical procedures were analyzed separately. The most recent report was selected in the case of multiple publications. If not provided, patient-years of follow-up were estimated as the product of the number of hospital survivors and the mean follow-up duration of that study.


Overall, 1,385 patients were analyzed (972 patients had total root replacement and 413 patients had valve-sparing root replacement). Reintervention rate was 0.3%/year (95% confidence interval [CI], 0.1-0.5) versus 1.3%/year (95% CI, 0.3-2.2; p = 0.02) and the thromboembolic event rate was 0.7%/year (95% CI, 0.5-0.9) versus 0.3%/year (95% CI, 0.1-0.6; p = 0.01) after total root replacement and valve-sparing root replacement, respectively. When composite valve-related events were compared, no difference existed between the two surgical strategies (p = 0.41). Among patients undergoing valve-sparing root replacement, reimplantation was associated with a reduced rate of reintervention compared with remodelling (0.7%/year vs. 2.4%/year, p = 0.02).


Valve-sparing root replacement may represent a valuable option for patients with Marfan syndrome who require surgery for aortic aneurysm. However, this technique should be used with caution in patients with valve characteristics at risk for decreased durability.


As with mitral valve repair, a well-performed aortic valve repair might be better than aortic valve replacement. However, a well-performed valve replacement probably is better than a poorly performed valve repair. Aside from all the usual issues with meta-analyses, it remains unwise to extrapolate the published experience of a few higher-volume surgeons to other surgeons with potentially less experience and less expertise in this specialized field. If valve-sparing aortic root replacement is better than total root replacement, then there is a rationale for referral of patients with Marfan syndrome and aortic root aneurysm to a surgeon and a center where valve-sparing root repair is routinely performed.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Quality Improvement

Keywords: Survivors, Heart Valve Prosthesis, Follow-Up Studies, Marfan Syndrome, Mitral Valve

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