Neoaortic Dilation and Polyvalvar Dysfunction: The Coexistence of Marfan Syndrome with Hypoplastic Left Heart Syndrome

We present a unique case of progressive neoaortic root and neoascending aortic dilatation, with poly-valvar regurgitation in a patient with Marfan syndrome and hypoplastic left heart syndrome palliated to Fontan procedure. This progressive dilation and valvar dysfunction necessitated a modified Bentall procedure and tricuspid valve repair. Our case highlights the impact of both a primary and secondary aortopathy complicating the Fontan procedure and the need for mechanical aortic valve replacement.