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

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.

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.

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.

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.