Table 1

Repeatability in all patients with PAH (ICC), and treatment effect size for patients with PAH initiating or escalating PAH therapy

All PAHPatients with PAH initiating or escalating therapy
NICCNVisit 1Visit 2Change
(Visit 1–visit 2)
95% CICohen’s d
MeanSDMeanSDMean differenceSDSEMLowerUpper
Walk test
6MWT distance (m)390.98724325.63156.30361.50166.29−35.8879.0616.14−69.26−2.490.22
Blood tests
Log NT-ProBNP320.772242.760.462.670.410.090.320.07−0.050.220.20
MRI metrics
SA with threshold
RVEDM (g)400.97026117.8045.7299.4043.9618.4030.906.065.9230.880.41
RVESM (g)400.98026106.6839.7394.6142.0812.0626.795.251.2422.880.29
RVEDV (mL)400.96926145.7139.12146.0355.87−0.3229.135.71−12.0811.450.01
RVESV (mL)400.9832693.9334.6681.2841.4012.6522.024.323.7621.550.33
RVEF (%)400.8832636.5611.4845.6911.12−9.1210.452.05−13.35−4.900.81
RVSV (mL)400.8642651.7817.3064.7523.92−12.9723.274.56−22.37−3.570.62
RVCO (L/min)400.886263.951.454.481.55−0.531.540.30−1.150.090.35
Systolic septal angle (o)400.85227163.3316.45156.8114.006.5211.282.172.0610.980.43
Diastolic septal angle (o)400.89727153.1114.73145.4810.447.6310.151.953.6111.650.60
Q flow
Net flow volume (mL)410.8932658.0530.1869.4931.30−11.4434.836.83−25.512.620.37
Forward flow volume (mL)410.8602660.3727.5872.3329.15−11.9631.976.27−24.880.950.42
Backward flow volume (mL)410.817262.326.762.845.74−0.525.851.15−2.881.850.08
Regurgitant fraction (%)410.731266.2819.585.4211.520.8718.773.68−6.718.450.05
Average flow velocity (cm/s)410.909267.313.608.253.69−0.943.690.72−2.430.550.26
Peak flow velocity (cm/s)410.5822652.9716.3767.6822.71−14.7119.353.79−22.53−6.900.74
Diastolic vessel area (mm2)410.93326981.10257.92961.84242.9619.26104.5220.5−22.9661.480.08
Systolic vessel area (mm2)410.953261077.57279.961083.62266.78−6.05101.0819.82−46.8834.770.02
Pulmonary arterial pulsatility (%)410.776269.964.8713.005.12−3.043.620.71−4.50−1.580.61
DCE imaging
Pulmonary transit time (s)360.728216.761.816.121.880.641.600.35−0.091.370.35
FWHM (s)320.906187.893.146.202.401.682.190.520.602.770.60
  • Data are shown for all patients with PAH initiating or escalating PAH therapy.

  • DCE, dynamic contrast-enhanced imaging; FWHM, full width at half maximum; ICC, intraclass correlation coefficient; Log10NT-ProBNP, log to base 10 N-terminal pro-brain natriuretic peptide; 6MWT, six min walk test; PAH, pulmonary arterial hypertension; RVCO, right ventricular cardiac output; RVEDM, right ventricle end-diastolic mass; RVEDV, right ventricle end-systolic volume; RVEF, right ventricle end-systolic volume; RVESM, right ventricle end-systolic mass; RVSV, right ventricle stroke volume; SA, short axis.