Unicuspid unicommissural aortic valves: A surgical pathology analysis

ABSTRACT The unicuspid unicommisural aortic valve is an uncommon congenital malformation that often manifests as stenosis with or without regurgitation in adults in their third to fifth decades of life. This report characterizes the morphological features of surgically excised unicuspid valves in adults with clinical correlation. Among the surgically excised aortic valves over a period of 10 years, the clinical data and morphological features of unicuspid aortic valves were analyzed. The patients were grouped by the type of valvular function. Pathological features noted were the shape of the orifice, the status of the commissure and raphe, presence of fibrous thickening, calcification, and other complications. Twenty-three UAVs, excised over a 10-year period, represented 4.16% of the excised diseased aortic valves. There was a male preponderance with a mean age of 47.7 years. Majority of the patients (22 cases) had moderate to severe stenosis with varying degrees of regurgitation, and the valvular disease had been clinically attributed to rheumatic heart disease, bicuspid aortic valve, or senile degenerative changes. Most of the valves (18) had been cut at their commissural regions, and 2 rudimentary commissures or raphes were seen in 21 valves. Cuspal fibrosis/calcification was often associated with complications like ulceration, hemorrhage, and bland vegetations. Aneurysm of the ascending aorta had been present in 1 patient. The unicuspid unicommisural aortic valve while rare is usually clinically classified with the more common, congenitally bicuspid aortic valve. Clinical or imaging diagnosis can be challenging since calcification may obscure the morphology creating difficulties in distinguishing such valves from other congenital or acquired valvular pathologies, especially in older patients. Often it is only the gross examination that leads to the differentiation as was our observation.