Table 1.

Patient Demographics and Baseline Clinical Measurements (N = 78)

Median Age, years	9.3 (5.2 – 14.2)
Female	42 (53.9%)
IPAH	26 (33.3%)
Associated Pulmonary Arterial Hypertension
Congenital Heart Disease	41 (52.5%)
Repaired	Repaired/Unrepaired
Ventricular Septal Defect	10/3
Atrial Septal Defect	6/2
Atrioventricular Septal Defect	6/2
Patent Ductus Arteriosus	2/1
Transposition of the Great Arteries	2/0
Double Outlet Right Ventricle	1/0
Total Anomalous Pulmonary Venous Return	2/0
Tetralogy of Fallot	1/0
“Absent Pulmonary Artery”	0/3
Portopulmonary Hypertension	2 (2.5%)
Connective Tissue Disease	3 (3.8%)
Sickle Cell Disease	1 (1.2%)
Bronchopulmonary Dyslpasia	5 (6.4%)
Baseline Cath measurements - median (IQR)
Right Atrial Pressure, mm Hg	6 (4 – 8) N = 77
Pulmonary Artery Pressure, mm Hg	38 (29 – 59) N = 79
Pulmonary Capillary Wedge Pressure, mm Hg	9 (7 – 10) N = 73
Cardiac Index, L/min × m2	3.5 (2.8 – 4.4) N = 63
Pulmonary Vascular Resistance Index (PVR), Wood units × m2	6.5 (4.7 – 14.7) N = 74
Systemic Arterial Pressure, mm Hg	64 (58 – 70) N = 78
Systemic Vascular Resistance Index (SVR), Wood units × m2	14.8 (11.2 – 19.2) N = 72
PVR/SVR	0.5 (0.4 – 0.8) N = 72
Baseline Echo measurements - median (IQR)
Right Ventricular Diastolic Dimension, cm	2.7 (2.1 – 3.2) N = 47
Left Ventricular Diastolic Dimension, cm	3.6 (3.0 – 4.2) N = 70
Fractional Shortening of the left ventricle, %	40.0 (35.5 – 46.5) N = 69
Tricuspid Regurgitation Velocity, m/s	4.2 (3.5 – 4.8) N = 64
Tricuspid Regurgitation Pressure Gradient, mm Hg	70.1 (49.2 – 91.9) N = 64
No. caths per patient (range)	0 – 8
No. patients with 1 or no cath	41
No. patients with 2 or more caths	37
No. echos per patient (range)	0 – 27
No. patients with 1 or no echo	7
No. patients with 2 or more echos	71

Values are given as the median (IQR) or No. (%), unless otherwise indicated.