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 QAVand 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.