Introduction:

The survival of patients with Marfan syndrome and aortic root involvement has increased over the past decades due to the introduction of valve-sparing aortic root replacement techniques.

Objective:

To identify and characterize the short- and long-term outcomes of patients with Marfan syndrome managed with aortic root replacement with aortic valve reimplantation (Tirone David procedure).

Materials and method:

A case series of patients diagnosed with Marfan syndrome who underwent the surgical procedure between 2002 and 2020. Kaplan-Meier curves were created to evaluate aortic reintervention-free time and survival.

Results:

18 patients were included, the average age was 29 years; disease progression was identified in 35.3%, determined by reintervention in five patients, three patients had type B aortic dissection, two had severe aortic insufficiency and five had chronic thoracic or abdominal aneurysms. There were three deaths, not directly related to disease progression.

Conclusions:

In patients with Marfan syndrome and aortic root disease, aortic root replacement with aortic valve reimplantation is the ideal technique due to its results in results in avoiding the need for reintervention and improving survival and quality of life. However, surgery does not resolve distal aortic involvement, which is the main factor in disease progression.

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