Background: Familial hypercholesterolemia (FH), is a recognized cause of premature aortic valve disease. This condition poses a real dilemma in young patients requiring aortic valve replacement (AVR). On one hand, mechanical valves entail lifelong anticoagulation with its associated thromboembolic and bleeding risks. On the other hand, bioprosthetic valves have limited durability. The Ross procedure has been associated with good long-term outcomes in young patients but there is limited data on its performance in patients with FH.
METHODS AND RESULTS:
Methods: This is a retrospective analysis of 16 adults with FH who underwent a Ross procedure between 1992 and 2024. The primary outcome is the cumulative incidence of autograft reintervention.
Results: The cohort included 8 males and 8 females, with a mean age of 41.0 ± 10.6 years. Four patients (25%) had previous cardiac operations. The main indication for surgery was aortic stenosis (12 patients, 75%). Four patients (25%) were operated due to severe aortic regurgitation. There was no in-hospital mortality, stroke, or new pacemaker implantation. Four patients required aortic valve reintervention during follow-up (3 underwent a biological Bentall and 1 patient had a mechanical AVR). At 10 years, the cumulative incidence of autograft reintervention is 13±9%. Three patients died during follow-up. Survival at 10 and 15 years was 94±6%.
Conclusion: In selected young patients with FH, the Ross procedure offers favorable mid-term outcomes. At 10 years, more than 85% of patients are free from reoperation. Long-term follow-up is mandated to assess the proposed benefits of this approach in the second and third decade.
