David-V Procedure in a Patient with Aortic Dilation and Competent Quadricuspid Aortic Valve: Are Genetics to Blame?

Quadricuspid aortic valves (QAVs) are extremely rare. In this case study, we report a David-V valve-sparing aortic root replacement with reimplantation of a native QAV in a patient with aortic dilation, normal valve function, and a family history of aortic dissection. Microscopic pathological examination of the excised section of the aorta revealed scattered small foci of cystic medical degeneration throughout. A genetic predisposition for aortic dilation may be present in patients with QAV, even in the setting of a competent valve. Regular screening for aortic dilation in patients with known QAV should therefore be considered.