Abstract 9363: Ascending Aorta Dilation is Less Frequent in Bicuspid Aortic Valve With Coarctation of Aorta Compared to Isolated Bicuspid Aortic Valve

Abstract

Listen

Translate article

Introduction: Bicuspid aortic valve (BAV) is known to be associated with aortopathy and aortic valve dysfunction. There is paucity of data in the pediatric population comparing ascending aorta (AA) dilation related to BAV in patients with and without coarctation of aorta (CoA). Hypothesis: We assess the hypothesis that BAV is associated with decrease in incidence of AA dilation in patients with CoA when compared to patients with isolated BAV. Methods: A retrospective cross-sectional study was done for all BAV patients 0-26 years of age seen from 2012 through 2021 in the outpatient pediatric cardiology clinic. The patients were subdivided into two groups: associated CoA (cBAV) and isolated BAV (iBAV) . Exclusion criteria were pregnancy, connective tissue disorders and/or aortic valve interventions. Patients with late repair of CoA ( ≥ 9 years) were excluded from the analysis of AA diameters because persistent hypertension, associated with late repair, could be a risk factor for AA dilation. Aortic insufficiency and aortic stenosis were defined by color Doppler and Doppler velocity by echocardiogram. Aortic dilation was defined by Z-score above 2.0. 95% score confidence intervals were used to compare the proportion of patients with an outcome in the two groups. Results: 345 patients with bicuspid aortic valve were included (67.5% male, median age 13). CoA history was present in 32 patients (9.3%). Of the 27 patients with CoA included to assess AA dilation, two patients did not need intervention for CoA. Incidence of AA dilation was 11.1% in cBAV and 28.7% in iBAV (CI: [-26.5%, 0%]; p= 0.05). AS was noted in 63 (18.3%) and AI in 184 (53.3%) of the 345 patients. AI was less common in cBAV at 34.4% than in iBAV at 55.3% (CI: [-35.9%, -2.7%]; p= 0.024). Incidence of AS was 9.4% in cBAV and 19.2% in iBAV (CI: [-17.5%, 5.6%]; p= 0.17). Conclusions: In conclusion, our study suggests that the ascending aorta in pediatric patients with BAV and CoA (who underwent early repair or did not meet criteria for intervention) may have less frequent dilation than in those with isolated BAV. Patients with BAV and CoA also have lower risk for aortic insufficiency and may have lower risk for aortic stenosis. The results are limited by the small sample size of patients with CoA and further study is recommended.