Risk Factors for Aortopathy in Bicuspid Aortic Valve: Insights from a Large Echocardiographic Cohort

Bicuspid aortic valve (BAV) is the most common congenital heart defect and is frequently associated with thoracic aortopathy. Risk stratification remains challenging, as predictors of aortopathy are incompletely understood, and prior studies have been limited by modest sample sizes or incomplete echocardiographic characterization.

We retrospectively analyzed 1602 patients diagnosed with BAV. Aortopathy was defined as dilation of the sinus of Valsalva or ascending aorta using guideline-recommended thresholds. Clinical and echocardiographic predictors included age, sex, body surface area, hypertension, diabetes mellitus, medications (including beta blockers, calcium channel blockers, angiotensin converting enzyme inhibitors (ACEi), angiotensin receptor blockers (ARB) and statins, BAV morphology (Sievers type I-III), presence of raphe, valve calcification severity, aortic regurgitation (AR), and stenosis (AS) severity and coarctation. Missing data were addressed by multiple imputations. Univariable and multivariable logistic regression identified independent predictors of aortopathy.

Aortopathy was present in 64.7% of patients. On multivariable analysis, older age (odds ratio [OR] 1.011 per year, p=0.005), male sex (OR 0.76 for female vs male, p=0.03), BAV type I (reference; type II OR 0.6, p=0.01; type III OR 0.57, p=0.04), valve calcification severity (OR 2.31 per grade, p<0.001), and AR severity (OR 1.20 per grade, p=0.002) were independently associated with aortopathy. AS severity (OR 0.87 per grade, p=0.02) was inversely associated with aortopathy. Raphe, coarctation, hypertension, diabetes mellitus, consuming beta-blockers, calcium channel blockers, ACEi/ARB, and statins were not significant predictors. The model demonstrated good discrimination (AUC 0.79, 95% CI 0.76- 0.81), excellent calibration, and a Brier score of 0.18, indicating good overall predictive accuracy.

In a large echocardiographic cohort, age, sex, BAV type, valve calcification, AS, and AR severity were independently associated with aortopathy. These readily available variables refine risk stratification in BAV. Prospective, multicenter validation is warranted to incorporate these predictors into clinical practice.