Outcome of isolated bicuspid aortic valve in childhood

To evaluate the outcomes associated with isolated bicuspid aortic valve (BAV) during childhood and adolescence.

Analysis of a large single institutional cohort of children (n=981) with isolated BAV was undertaken to determine the prevalence of significant ascending aortic dilation and risk of cardiac events. Subjects with known genetic disorders, critical aortic stenosis (intervention required in infancy), or additional lesions such as coarctation of the aorta were excluded. Aortic dimensions were derived from echocardiography, and values were plotted as Z scores. Clinical outcomes included death, aortic dissection, balloon aortic valvuloplasty, or cardiac surgery.

The median age of the subjects at diagnosis was 8.3 years. At the time of the last pediatric follow-up, 7% of the subjects had moderate aortic regurgitation or greater, and the median Z score for the ascending aorta was +2.31. There were 9427 patient years of follow-up. Primary cardiac events occurred in 38 subjects, yielding an event rate of 0.004 per patient year. Eleven subjects (1.1%) underwent aortic valve surgery. Thirty subjects (3.0%) underwent balloon dilatation of the aortic valve. There was a single case of endocarditis. There were no cardiac-related deaths and no cases of aortic dissection.

The incidence of primary cardiac events in children with BAV is relatively low, approximately 3-fold lower than in young adults, and is generally related to aortic stenosis amenable to balloon dilatation. Although mild ascending aortic dilation is common in children, the clinical course is relatively benign. In this series, aortic dissection did not occur. Whether elective surgery for the dilated aorta has a role in children remains unknown.