Outcomes after repair or replacement of dysfunctional quadricuspid aortic valve

Objective Quadricuspid aortic valve (QAV) morphology is rare (0.008%) but often dysfunctional, manifesting early or late in life. No large series have been evaluated. Current objectives are to characterize these patients, and assess repair possibilities and outcomes. Methods From 1989 to 2010, a total of 19,722 patients underwent aortic valve surgery at Cleveland Clinic. Thirty-one (0.0016%) patients had dysfunctional QAV and underwent repair or replacement for moderate to severe aortic regurgitation (n = 21), stenosis (n = 5), or both (n = 4). One additional patient with functional QAV required excision of fibroelastoma. The mean age was 58 ± 18 years. Ascending aortic diameter was>4 cm in 13 (42%) patients, and 7 required ascending repair (mean diameter: 4.8 ± 0.4 cm). Three patients had anomalous origin of coronary artery, and 1 required repair. Results The aortic valve was repaired in 7 (23%) patients and replaced in 23 (73%). The decision of which procedure to use was based on intraoperative findings. The Ross procedure was performed in 1 patient who had endocarditis. Most patients in the repair group had leaflet prolapse that was repaired with accessory cusp excision and commisuroplasty. The mean gradient after repair was 14 ± 5 mm Hg. Bioprostheses were used in all replacements; median valve size was 25 mm (range: 21-27 mm). No operative mortality occurred. One patient suffered nonpermanent stroke after aortic valve replacement. There was no myocardial infarction, renal failure, respiratory failure, or reoperation for bleeding. The median follow-up time was 38 months; 1 patient required replacement 13 years after previous repair for recurrent regurgitation and stenosis. Conclusions Quadricuspid aortic valve dysfunction includes both regurgitation and stenosis; repair may be feasible in some patients with regurgitation, but most require replacement. Aortic root and ascending dilatation are frequent, and further studies are needed.