Characterization and risk factors for aortic dilatation in pediatric patients with bicuspid aortic valve

Abstract

Introduction and objectives: Dilatation of the ascending aorta associated with bicuspid aortic valve is a major cause of morbidity and mortality in adults. The main objective was to recognize the aortic involvement in children, its characteristics and risk factors.

Methods: Aortic measures of all pediatric patients with bicuspid aortic valve followed in a tertiary pediatric hospital between 1997 and 2015 were retrospectively taken. Patients with syndromes associated with aortic dilatation were excluded (n=17).

Results: Two hundred and six patients were included, 67.9% males. The commonest opening pattern was horizontal: 137 (66.7%). Half of the patients (101) had a history of surgical aortic coarctation, 46 (22.3%) had≥moderate aortic valve stenosis and 13 (6%) had≥moderate aortic insufficiency. Mean follow-up time was 6.1 (4.9) years; diagnosis of aortic dilatation was made during the first year of follow-up. Progression of the dilatation of the ascending aorta was noted in 17.1%, and of the aortic root in 2.5%. More than one-third (80/206) had aortic dilatation (z-score>2). The ascending aorta was exclusively affected in 70/80 patients, with sparing of the aortic root. In the multivariate analysis, patients with dilatation of the ascending aorta were associated with absence of coarctation (P=.001) and vertical opening pattern (P=.007).

Conclusions: Pediatric patients with bicuspid aortic valve warrant medical follow-up for the frequent association with valve impairment and/or dilatation of the ascending aorta.

Keywords: Aneurisma aórtico; Aortic aneurysm; Bicuspid aortic valve; Congenital heart disease; Dilatación; Dilatation; Enfermedad cardiaca congénita; Pediatrics; Pediatría; Senos de Valsalva; Sinus of Valsalva; Válvula aórtica bicúspide.