Background and aim of the study: The natural history of the unicuspid aortic valve (UAV) is poorly described in the literature. In order to study the association between UAV with any other cardiac or extra cardiac abnormalities, an evidence-based systematic review was carried out.
Methods: A computerized search was carried out of the medical literature published between 1st January 1966 and 1st September 2008 of the following databases: MEDLINE; EMBASE; Web of Science; and the Cochrane Database.
Results: A total of 231 cases of adult UAV was identified in 38 articles. The mean patient age was 42 years, and the most common presenting symptoms reported (in 52 cases) included dyspnea (44%; n=23), angina (21%; n=11), and dizziness or syncope (8%; n=4). The most common lesion in UAV was isolated aortic stenosis (AS) (41%; n=95) and AS with or without aortic regurgitation (28%; n=64). The preoperative diagnosis of UAV is rare, and 139 cases (60%) of UAV were reported at autopsy or by examination of surgically excised valves. Aortic valve replacement was performed in 166 cases (82%). Concomitant aortic surgery was performed in 47 of the UAV cases (23%), either for a dilated or aneurysmal aorta.
Conclusion: UAV shares many of the features of bicuspid aortic valve, including valvular dysfunction, aortic dilatation, aortic dissection, and dystrophic calcification, although these conditions develop at an earlier age and progress at a faster pace in UAV. Further investigations are warranted regarding the possibility of a familial incidence, associated histopathological changes in the aorta, preoperative diagnostic tools, ideal follow up and surgical intervention.