Unicuspid aortic valve in children: a systematic review spanning four decades.

BACKGROUND AND AIM OF THE STUDY: To date, the natural history of the unicuspid aortic valve (UAV) has been poorly described in the literature. To study the association between UAV with associated cardiac and extracardiac abnormalities, a systematic review was conducted.
METHODS: A computerized search was conducted of the medical literature published between 1st January 1966 and 1st September 2008, using the databases MEDLINE, EMBASE, Web of Science and Cochrane database.
RESULTS: Nine articles with 60 pediatric cases (age <15 years) were identified. The mean age at diagnosis was 14 months, the most common presentation of UAV was congestive heart failure due to congenital aortic stenosis, and the most common lesion was isolated aortic stenosis, reported in 19 cases (32%). Associated anomalies included 22 cases (37%) of aortic coarctation, seven (12%) with ventricular septal defect (VSD), three (5%) with patent ductus arteriosus (PDA), and three (5%) with aortic aneurysm. The preoperative diagnosis of UAV was rare, and 33 cases (55%) of pediatric UAV were reported at autopsy or at pathological examination of the surgically excised valves. A total of 26 cases (43%) was treated with either balloon valvoplasty or surgical valvotomy or commissurotomy.
CONCLUSION: UAV shares many of the features of bicuspid aortic valve, including valvular dysfunction, aortic dilatation, aortic dissection, and dystrophic calcification. The importance of an early diagnosis of UAV lies in its risk of sudden cardiac death and association with other congenital anomalies, such as PDA, VSD, anomalous coronary arteries, and coarctation of the aorta. Further investigations of UAV are warranted in the pediatric age group with regards to familial incidence, associated aortic histopathological changes, the ideal follow up to monitor complications, and surgical intervention.