Table 1. Candidate Predictor Definitions.
Candidate Predictor Variable | Definition | Coding |
---|---|---|
NYHA/Ross functional class | NYHA functional classification20/modified Ross heart failure classification for children13 at baseline evaluation | Binary (NYHA/Ross 1 = 0, NYHA/Ross ≥2 = 1) |
Unexplained syncope | Defined as a transient loss of consciousness with no identifiable cause at or before first evaluation1,8,14,15 | Binary (no = 0, yes = 1) |
Nonsustained ventricular tachycardia | ≥3 Consecutive ventricular beats at a rate of ≥120 beats/min lasting <30 s on ambulatory ECG monitoring (minimum duration 24 h) at or before first evaluation16,21 | Binary (no = 0, yes = 1) |
Maximal wall thickness z score | Defined as the number of SDs from the population mean22; the 2-D measurement of maximal wall thickness (millimeters) is at baseline evaluationa | Continuous (z score) |
Left atrial diameter z score | Defined as the number of SDs from the population mean23 the 2-D measurement of maximal left-atrial diameter (millimeters) is at baseline evaluationb | Continuous (z score) |
Maximal LV outflow tract gradient | The maximum LV outflow tract gradient at rest or with Valsalva provocation using continuous wave Doppler from the apical 3- or 5-chamber views17c | Continuous (mm Hg) |
Abbreviations: 2-D, 2 dimensional; ECG, electrocardiogram; LV, left ventricular; NYHA, New York Heart Association.
Maximal wall thickness is the greatest thickness as measured by 2-D echocardiography in the parasternal short-axis views of the left ventricle in 4 places at the level of the mitral valve and papillary muscles (anterior and posterior septum, lateral and posterior wall) and in 2 places at the apical level (anterior and posterior septum).5
Left atrial diameter is determined by M mode or 2-D echocardiography in the parasternal long-axis plane.
Peak outflow tract gradient is determined using the modified Bernoulli equation: gradient = 4V2, where V is the peak aortic outflow velocity.