Natural Progression of Aortic Insufficiency in Children

Purpose: The scientific literature is deficient in data regarding the natural history of aortic valve insufficiency (AI) in the pediatric population. As a result, the clinical indications and appropriate timing for surgical intervention are unknown. In the absence of natural history and surgical outcomes data, clinicians are forced to make decisions based solely on past experience, which results in significant practice variation. Methods: The UVA Pediatric Cardiology Database was queried to identify patients with aortic insufficiency who have been evaluated from January 1, 1990 to present. We excluded patients with Marfan syndrome, rheumatic heart disease, complex anatomy, or less than 6 months of follow up. We evaluated echocardiographic data including M-mode left ventricle measurements and the Doppler-determined degree of insufficiency. Serial echocardiograms were used including the first and most recent exam at least 6 months apart. In patients who had surgical therapy, we also included data just prior to surgery and immediately following. Results: In total, 59 patients had aortic insufficiency and 10 ultimately required aortic valve replacement (AVR) or Ross procedure. Of the 10 who required 2 operation, 4 had previous balloon valvuloplasty, and 2 were repaired due to worsening aortic stenosis. The 49 who did not progress to operation were followed for an average of 5.3 years. Of these, 34 (69%) were male and 29 (59%) had bicuspid valves. At initial visit, 14 (28%) had trivial or trace aortic insufficiency, 33 (67%) had mild, and 2 (4%) had moderate. In follow up 37 (76%) had mild insufficiency and only 1 (2%) had moderate. In contrast, of those who required operation, all 4 presented with moderate or severe AI. Conclusion: Isolated aortic valve insufficiency of trivial, trace or mild disease rarely progresses during childhood.

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