Longitudinal Doppler Assessment of Congenital Aortic Stenosis
It Is About Time!
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Congenital valvar aortic stenosis (AS) is most often caused by a bicuspid (bicommissural) or unicuspid aortic valve. Bicuspid aortic valve, found in up to 2% of the population,1 is the most common congenital cardiac malformation, and because the severity of stenosis tends to progress over time, congenital AS is relatively common. Beyond the acute presentation of the ill neonate with critical AS, most children are asymptomatic, and symptoms, such as fatigue, exertional dyspnea, angina, and syncope, are uncommon and typically appear late, when AS is already severe.2 Nonetheless, the complications of AS are potentially serious, including left ventricular (LV) hypertrophy, ischemia and dysfunction, syncope, arrhythmia, and sudden death. Because AS is progressive before and after intervention, the frequency of follow-up and timing of intervention are important deliberations.
See Article by Kuebler et al
Current indications for intervention continue to rest on the second natural history study of congenital heart defects, which used catheter measured peak-to-peak gradients.3 However, this study is now 25 years old, and through technological improvements over the years, echocardiography has become the central modality used to assess AS and to inform on the potential need for intervention. Indeed, catheterization is now uncommonly performed for hemodynamic evaluation alone. Thus, despite the well-recognized differences between echocardiographic and catheter assessment of AS gradients,4,5 management decisions and the decision to proceed with catheterization are now based to a large degree on echocardiography, within the clinical context.
In children, the central echo parameters that determine the need for intervention are LV function and Doppler evaluation of the maximal and mean instantaneous gradient across the aortic valve. Because LV dysfunction is uncommon in children outside of neonatal AS, …