An Adult Case of Unicommissural Unicuspid Aortic Valve Diagnosed Based on the Intraoperative Findings

Abstract

We herein report an adult case of unicommissural unicuspid aortic valve (UAV). A 59-year-old man, who was noted to have a cardiac murmur at 31 years of age, was admitted to our hospital due to acute heart failure. Severe calcification in the aortic valve with severe low-flow/low-gradient aortic stenosis and moderate aortic regurgitation was observed and thought to be the cause of heart failure, however, the etiology of aortic valve dysfunction was not clear. Aortic valve replacement was subsequently performed, and unicommissural UAV was diagnosed according to the intraoperative findings. UAV is very rare congenital aortic valve disease which is rarely diagnosed preoperatively.

Unicuspid Aortic Valve: A Rare Congenital Anomaly

“…The unicommissural form appears to have a less-aggressive course. It usually develops symptoms until adulthood since the formation of a commissure brings a relatively larger valvular orifice area compared to acommissural type [4, 5, 8, 12, 35, 39, 40]. Moreover, the valvular lesion would progress further with the aging, the accelerated leaflet calcification and fibrosis resulted from the aortic valve malformation [13, 38, 41].…”

“…UAV may also be accompanied by other abnormalities, such as aortopathy, patent ductus arteriosus, ventricular septal defect, and anomalous coronary artery [12, 40]. Concurrent aortopathy comprises aortic dissection, coarctation of the aorta, dilatation or aneurysm of the aortic annulus, aortic root or ascending aorta [38, 42].…”

“…Nearly half (48%) adult UAV patients coexist with ascending aortic dilatation which may be caused by a synergistic effect of hemodynamic stress and a congenitally inherited weakness of the aortic media. Whereas ascending aortic dilatation is less common in pediatric patients for the relatively short-term effect of hemodynamic stress [2, 40, 43]. Reconstruction of the aortic sinuses or replacement of the ascending aorta is recommended when the diameter of the aortic sinuses or ascending aorta is greater than 55 mm [44].…”

“…Some are palliative procedures and possibly associated with residual or recurrent valve dysfunction and reintervention. The acommissural type usually requires these treatments from neonatal period due to severe AS [1, 40, 66]. In adult population, the most common treatment modality is aortic valve replacement (AVR).…”