Table 1.

Baseline characteristics for enrolled subjects by cohort.

Characteristic	Overall (N=36)	Exercise testing only (N=6)	37.5 mg daily (N=6)	37.5 mg twice (N=6)	87.5 mg daily (N=6)	125 mg daily (N=6)	87.5 mg twice (N=6)	P-value*
Age, year	15.8±1.3	16.2±1.2	15.5±1.0	16.2±0.8	15.0±0.9	16.5±1.5	15.5±1.8	0.319
Median (Interquartile Range)	16 (15, 17)	16 (15, 17)	16 (15, 16)	16 (16, 17)	15 (14, 16)	17 (16, 18)	15 (14, 17)	0.309
Range, Min - Max	14 – 18	15 – 18	14 – 17	15 – 17	14 – 16	14 – 18	14 – 18
Male	21 (58%)	4 (67%)	4 (67%)	3 (50%)	3 (50%)	4 (67%)	3 (50%)	1.000
Race								0.453
White/Caucasian	28 (78%)	5 (83%)	3 (50%)	4 (67%)	6 (100%)	4 (67%)	6 (100%)
Black/African American	3 (8%)	0 (0%)	2 (33%)	1 (17%)	0 (0%)	0 (0%)	0 (0%)
Other/Unknown	4 (11%)	1 (17%)	1 (17%)	1 (17%)	0 (0%)	1 (17%)	0 (0%)
Height, cm	165.7±10.1	163.6±5.6	161.0±12.9	164.9±10.9	168.6±7.8	171.3±7.8	164.7±14.2	0.585
Weight, kg	62.4±15.9	54.9±16.8	72.5±27.1	56.3±7.7	66.8±11.0	61.9±9.0	62.0±15.5	0.425
Body mass index, kg/m2	22.6±5.0	20.3±4.8	27.2±8.1	20.8±3.2	23.4±3.2	21.0±2.0	22.6±4.8	0.157
Right ventricle as dominant ventricle**	13 (36%)	1 (17%)	3 (50%)	3 (50%)	0 (0%)	5 (83%)	1 (17%)	0.032
Oxygen saturation	93.8±3.1	94.7±2.5	96.0±3.0	92.3±2.7	92.8±1.9	93.2±3.7	93.5±4.2	0.363
Ventricular function								0.677
Normal	30 (83%)	6 (100%)	4 (67%)	4 (67%)	5 (83%)	5 (83%)	6 (100%)
Mild dysfunction	6 (17%)	0 (0%)	2 (33%)	2 (33%)	1 (17%)	1 (17%)	0 (0%)
Presence of fenestration	15 (42%)	1 (17%)	1 (17%)	3 (50%)	4 (67%)	2 (33%)	4 (67%)	0.378

^*P-values for continuous variables were calculated by ANOVA for parametric analysis or Kruskal-Wallis test for non-parametric analysis.

P-values for categorical variables were calculated by Fisher’s exact test.

^**There are 13 subjects with dominant RV, 22 subjects with dominant LV, and one subject with co-dominant ventricles (37.5 mg twice daily cohort).