Table III.

Secondary endpoints and outcome measures

•The composite end point of aortic dissection, aortic root surgery, or death⁎ •Annual rate of change in absolute aortic dimensions at the sinuses of Valsalva† •Annual rate of change in absolute aortic dimensions at the ascending aorta† •Annual rate of change in absolute aortic dimensions at other aortic sites†‡ •Annual rate of change in BSA-adjusted, normalized aortic dimensions, expressed as a z-score†‡ •Annual rate of change in proportional aortic root size‡ •Annual rate of change in absolute and BSA-adjusted dimensions of the pulmonary artery •The incidence of moderate to severe aortic valve regurgitation •The incidence of moderate to severe mitral valve regurgitation •The incidence of aortic valve–sparing aortic root surgery and combined aortic valve and aortic root replacement •Annual rate of change of measures of left ventricular cavity size, wall thickness, and systolic function§ •Annual rate of change of brachial systolic, diastolic, and mean arterial pressure and pulse pressure •Rate of change of age-adjusted measures of arterial stiffness/elasticity •Rate of change of levels of circulating biomarkers of vascular function (including TGFβ where available) •Annual rate of change of markers of somatic growth and disproportion║ •Tolerability and side effects of therapy, frequency, and nature of adverse drug reactions and quality-of-life indices and the proportion of treatment failures, discontinuations, and/or patient drop-outs.

^⁎And of each of these components separately.

^†Sensitivity analysis will also estimate the effects of different imaging methods (systole vs diastole; inner-edge to inner-edge vs leading-edge to leading-edge method, echo versus magnetic resonance imaging).

^‡At the aortic annulus, sinuses of Valsalva, sinotubular junction, ascending aorta, aortic arch, and descending aorta.

^§End-diastolic dimension, end-diastolic volume, end-systolic dimension, end-systolic volume, left ventricular wall thickness (septum), left ventricular wall thickness (posterior wall), left ventricular mass, left ventricular mass/volume ratio, fractional shortening, and ejection fraction, each indexed to body size and normalized where appropriate.

^║Height, weight, body surface area, body mass index z-scores indexed to age, and markers of skeletal disproportion (arm span-to-height ratio and upper-segment-to-lower-segment ratio) with age at enrollment and height at enrollment as covariates.