Quadricuspid aortic valve repair: Results of a phenotype-based approach.

Objective: Quadricuspid aortic valve (QAV) anatomy is a rare congenital anomaly. Patients develop relevant aortic regurgitation (AR), commonly between the fourth and sixth decades of life. Various approaches to repair have been proposed, but mid-term data are lacking. The present study aimed to investigate late results of QAV repair using different repair concepts. Methods: Between 2004 and 2023, 19 patients (32% male; mean age, 46 ± 12 years; range, 26-60 years) underwent QAV repair. The mean duration of follow-up was 6.3 ± 5 years (range, 4 months to 19 years), and follow-up was 96% complete. Patients underwent surgery for isolated AR (n = 18) or combined valve disease (n = 1). Three patients (16%) had concomitant ascending aortic dilatation. Results: The majority of patients underwent design change-tricuspidization (n = 13; 68%) or bicuspidization (n = 3; 16%)-of the QAV; the valve was left quadricuspid in 3 patients (16%). Sinotubular junction remodeling was performed by adding a sinotubular suture (n = 7; 37%) or by ascending aortic replacement (n = 3; 16%). All patients were alive at 5 years and 10 years postoperation; 2 required late aortic valve reoperation. Freedom from reoperation was 82% at 12 years (86% after tricuspidizdation and 67% after bicuspidization). The 3 patients whose valve remained quadricuspid did not require reoperation after 2 years, 3 years, and 5 years. Overall freedom from AR >2 was 76% at 12 years. At last follow-up, 2 patients had a mean gradient of 21 mm Hg, both of whom had undergone bicuspidization. Conclusions: QAVs can be repaired by different methods, including tricuspidization and bicuspidization. If the quadricuspid morphology is preserved, stabilization of annular and sinotubular dimensions may achieve a stable result.