Long-Term Outcome after Aortic Valve-Sparing Root Surgery in Patients with Connective Tissue Disorder

Objectives: Most patients with connective tissue disorder present with aortic root dilatation that may require surgical intervention. In many specialized centers, 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) reintervention, and 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 rates were 92.1, 77.5, and 77.5% at 5, 10, and 15 years, respectively. Rates of freedom from AV reintervention at 5, 10, and 15 years were 92.8, 78.5, and 78.5%, respectively. No further MACCE (e.g., endocarditis, stroke) occurred in this study cohort during follow-up.

Conclusion: 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.