Unicuspid unicommissural aortic valves: A surgical pathology analysis

Area of Science:

• Cardiovascular Pathology
• Congenital Heart Disease
• Surgical Pathology

Background:

• The unicuspid unicommisural aortic valve (UAV) is a rare congenital malformation.
• It typically manifests as aortic stenosis with or without regurgitation in adults aged 30-50.
• UAVs are often misdiagnosed clinically as more common conditions like bicuspid aortic valves or degenerative disease.

Purpose of the Study:

• To characterize the morphological features of surgically excised unicuspid aortic valves in adults.
• To correlate these pathological findings with clinical data.
• To improve diagnostic accuracy for this uncommon condition.

Main Methods:

• Analysis of clinical data and morphological features of 23 surgically excised unicuspid aortic valves over 10 years.
• Classification of patients based on valvular function.
• Detailed pathological examination of orifice shape, commissures, fibrous thickening, calcification, and associated complications.

Main Results:

• UAVs constituted 4.16% of excised diseased aortic valves, with a male predominance and mean age of 47.7 years.
• 22 patients had moderate to severe stenosis with regurgitation, often misattributed to other conditions.
• Morphological findings included rudimentary commissures/raphe (21 valves) and cuspal fibrosis/calcification (associated with ulceration, hemorrhage, vegetations).
• Ascending aorta aneurysm was noted in one patient.

Conclusions:

• The unicuspid unicommisural aortic valve is rare but clinically significant.
• Diagnostic challenges arise from calcification obscuring morphology, mimicking other valvular diseases.
• Gross pathological examination is often essential for definitive differentiation of UAVs.