Superior Long-term Survival After Aortic Valve-sparing Root Surgery in Patients with Connective Tissue Disorder

Abstract

Objective: Most patients with connective tissue disorder present with aortic root dilatation that may require surgical intervention. In many specialized centres, aortic valve-sparing root surgeries have become the preferred surgical procedure to treat aortic root aneurysm in patients with connective tissue disorder. Methods: A total of 313 patients underwent aortic valve-sparing root surgery at our institution between 1997 and August 2019. Out of these, 83 patients had a connective tissue disorder and were included into further analysis. Follow-up protocol included clinical interview using a structured questionnaire and long-term echocardiographic follow-up. Time-to-event analyses were calculated using the Kaplan-Meier method. Primary endpoint was actual freedom from aortic valve (AV) re-intervention, secondary endpoints were survival and freedom from major adverse cardiac and cerebrovascular events (MACCE). Results: Mean age of the study cohort was 35.4 ± 12.4 years and 60% were male. Marfan syndrome was present in 95.2% and Loeys-Dietz syndrome in 4.8% of the patients. Reimplantation technique of the aortic valve was performed in 72 and remodeling technique of the aortic root in 11 patients. In-hospital and 30-day mortality was 0%. Mean follow-up was 69.6 ± 51.1 (range: 2 - 185 months). Overall survival was 92.1%, 77.5%, and 77.5% at 5, 10 and 15 years. Freedom from AV re-intervention at 5, 10 and 15 years was 92.8%, 78.5 and 78.5%, respectively. No further MACCE (e.g. endocarditis, stroke) occurred in this study cohort during follow-up. Conclusions: In specialized heart centers, aortic valve-sparing root surgery is a safe procedure with very low in-hospital mortality and favorable long-term results in patients with connective tissue disorder.