Left Ventricular Diastolic Function in Children and Young Adults With Congenital Aortic Valve Disease

Young patients with congenital aortic valve disease are at risk of left ventricular (LV) diastolic dysfunction (DD). We evaluated LV remodeling and the prevalence of, and risk factors for, DD in patients with aortic stenosis (AS), pure aortic regurgitation (AR), and AS+AR. Patients aged 8 to 39 years with congenital AS (n = 103), AR (n = 36), or AS+AR (n = 107) were identified. Cross-sectional assessment of the LV remodeling pattern and diastolic function was performed. A diastolic function score (DFS; range 0 to 4) was assigned to each patient, with 1 point for an abnormal value in each of 4 categories: mitral inflow (E/A and E-wave deceleration time), tissue Doppler E′, E/E′, and left atrial volume. Patients with a DFS of ≥2 were compared to those with a DFS <2. Concentric hypertrophy was the most common remodeling pattern in those with AS (51%), mixed/physiologic hypertrophy in those with AS+AR (48%) and eccentric hypertrophy in those with AR (49%) predominated. In the entire cohort, 91 patients (37%) had a DFS of ≥2. Patients with AS or AS+AR had greater DFS than those with pure AR (p <0.001). On multivariate analysis, a greater LV mass z-score and previous aortic valve balloon dilation were associated with a DFS of ≥2. In patients with catheterization data (n = 65), E/E′ correlated with LV end-diastolic pressure. Those with a DFS of ≥2 had a greater LV end-diastolic pressure and mean pulmonary artery pressure than those with a DFS <2. In conclusion, DD is common in young patients with AS and AS+AR but not in those with pure AR. A greater LV mass and previous aortic valve dilation were associated with DD.