Table 1

Characteristics of subjects

Age, yearsSex, F/MBMPR2 mutation, +/−Mean PAP, mm HgCardiac output, L/minPVR, wood unitRVSP, mm HgERA, +/−Prostacyclin, +/−Sildenafil, +/−
IPAH 43 ± 3 20/4 3/21 52 ± 3 5.5 ± 0.5 9.6 ± 1.8 70 ± 5 14/24 21/3 8/16 
FPAH 41 ± 4 10/3 4/9 58 ± 7 3.4 ± 0.3 14.6 ± 3.0 66 ± 7 7/13 6/7 7/6 
APAH 56 ± 3 11/4 0/11 50 ± 5 5.3 ± 0.6 8 ± 2 77 ± 8 9/15 7/15 7/8 
Controls 35 ± 1 46/16         
P < .01 .8 .06 .6 < .01 .1 .6 .6 .2 .4 
Age, yearsSex, F/MBMPR2 mutation, +/−Mean PAP, mm HgCardiac output, L/minPVR, wood unitRVSP, mm HgERA, +/−Prostacyclin, +/−Sildenafil, +/−
IPAH 43 ± 3 20/4 3/21 52 ± 3 5.5 ± 0.5 9.6 ± 1.8 70 ± 5 14/24 21/3 8/16 
FPAH 41 ± 4 10/3 4/9 58 ± 7 3.4 ± 0.3 14.6 ± 3.0 66 ± 7 7/13 6/7 7/6 
APAH 56 ± 3 11/4 0/11 50 ± 5 5.3 ± 0.6 8 ± 2 77 ± 8 9/15 7/15 7/8 
Controls 35 ± 1 46/16         
P < .01 .8 .06 .6 < .01 .1 .6 .6 .2 .4 

BMPR2 indicates bone morphogenetic protein receptor; F/M, female/male; PAP, pulmonary arterial pressure; PVR, pulmonary vascular resistance; RVSP, right ventricular systolic pressure; ERA, endothelin receptor antagonist; IPAH, idiopathic pulmonary arterial hypertension; FPAH, familial pulmonary arterial hypertension; and APAH, associated pulmonary arterial hypertension.

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