Author(s)

Dilesha Dilangi Kumanayaka^1*, Alex Otto², Mit Chauhan¹, Vadim Belinschi¹, Noreen Mirza¹, Mohammad Nabil Rayad¹, Addi Suleiman¹

¹Department of Internal Medicine, New York Medical College at Saint Michael’s Medical Center, Newark, NJ, USA.
²Department of Medical Education, University of New England College of Osteopathic Medicine, Portland, Maine, USA.

The most common congenital heart defect is the bicuspid aortic valve (BAV) occurring with fusion of one of the three-valve commissures. A rarer valvular phenomenon is the unicuspid aortic valve (UAV) which has an estimated prevalence of 0.02% in the adult population. Two morphologic subtypes of UAV exist: the earlier presenting, acommissural UAV and the later appearing unicommissural. To better characterize and manage patients with UAVs, a systematic review of this rare phenomenon was performed. The objective of our study was to conduct a systematic review of adequate case studies involving UAV patients to describe patient characteristics, clinical presentation, management, and common sequela featured there within. The mean age of presentation in this review was 35.6 years. When diagnosing UAV, TEE was the most utilized diagnostic modality (65%) followed by intraoperative diagnosis (17%) discovered with valve repair or incidentally during another procedure. TTE was utilized to diagnose only 7% of the cases reviewed likely owing to the difficulty and inaccuracies in identifying UAV with this method that were previously established by multiple series. Interventional options for UAV are balloon versus surgical valvotomy, aortic valve replacement, Ross procedure and aortic root replacement. They are performed on an individual basis and all cases ultimately require aortic valve replacement (AVR) or Ross procedure (to avoid anticoagulation). Aortic root replacement is additionally required if aortic root/ascending aorta diameter exceeds 4.5 cm. In this review, Aortic aneurysm (16%) cases resulted in surgical interventions. There may be a need to implant permanent pacemaker (in 3% - 8%) during AVR when calcified UAV has calcification extending into interventricular septum.

Unicuspid Aortic Valve, Bicuspid Aortic Valve, Heart Defects, Unicommisural Valve

Kumanayaka, D. , Otto, A. , Chauhan, M. , Belinschi, V. , Mirza, N. , Rayad, M. and Suleiman, A. (2023) Unicuspid Aortic Valve, an Extremely Rare Congenital Anomaly in Adults: A Systemic Review. World Journal of Cardiovascular Diseases, 13, 283-288. doi: 10.4236/wjcd.2023.136025.