TABLE 1.

Summary Characteristics of Patients With Group 1 PAH, Healthy Control Subjects, and Comparators

							P Value
	PAH (n = 186)		Healthy Control (n = 78)		Comparator (n = 32)		PAH vs Comparator	All 3 Groups
Demographics
Age at enrollment, y	186	52.6 ± 14.1a	78	48.2 ± 14.7a	32	60.1 ± 12.2b,c	0.005d	<0.001d
Male	186	53 (28.5)	78	24 (30.8)	32	6 (18.8)	0.25e	0.43e
Hispanic ethnicity	183	21 (11.5)	78	9 (11.5)	30	3 (10.0)	0.99g	0.97e
Race (Black, White, other)	182		78		31		0.19g	0.30g
Black or African American		22 (12.1)		9(11.5)		6 (19.4)
White		147 (80.8)		67 (85.9)		25 (80.6)
Other		13 (7.1)		2 (2.6)		0 (0.00)
Body mass index, kg/m2	186	30.3 ± 7.6	78	28.3 ± 5.9	32	28.9 ± 6.3	0.32d	0.090d
PH medicationsf	186	154 (82.8)a,b	78	0 (0.00)c	32	4 (12.5)c	<0.001e	<0.001e
Age at diagnosis of PH, y	186	47.1 ± 15.8
Years of PH at time of enrollment	186	3.8 (0.83–9.0)
NYHA functional class	185		0		31		0.19h	0.19h
I		23 (12.4)				2 (6.5)
II		76 (41.1)				20 (64.5)
III		77 (41.6)				9 (29.0)
IV		9 (4.9)				0 (0.00)
6MWD, m	171	382.2 ± 128.8b	78	524.7 ± 94.6a,c	32	375.9 ± 113.0b	0.80d	<0.001d
Etiology of PHi
Idiopathic pulmonary arterial hypertension	186	89 (47.8)			32	0 (0.00)	<0.001e
Connective tissue disease	186	44 (23.7)			32	25 (78.1)	<0.001e
Systemic sclerosis	44	21 (11.3)			32	12 (37.5)	<0.001g
Systemic lupus erythematosus	44	9 (4.8)			32	4 (12.5)	0.10g
Sjogren's syndrome	44	6 (3.2)			32	3 (9.4)	0.13g
Rheumatoid arthritis	44	4 (2.2)			32	5 (15.6)	0.004g
Mixed connective tissue disease	44	4 (2.2)			32	5 (15.6)	0.004g
Antisynthetase syndrome	44	0 (0.00)			32	1 (3.1)	0.15g
Congenital heart disease	186	17(9.1)			32	5 (15.6)	0.34g
Familial pulmonary arterial hypertension	186	16 (8.6)			32	0 (0.00)	0.14g
Portal hypertension	186	10 (5.4)			32	0 (0.00)	0.36g
Shunt repaired	186	7 (3.8)			32	2 (6.3)	0.62g
Shunt unrepaired	186	14 (7.5)			32	3 (9.4)	0.72g
Drug-induced pulmonary arterial hypertension	186	6 (3.2)			32	1 (3.1)	0.99g
Human immunodeficiency virus	186	4 (2.2)			32	0 (0.00)	0.99g
Pulmonary veno-occlusive disease	186	4 (2.2)			32	0 (0.00)	0.99g
Pulmonary capillary hemangiomatosis	186	3(1.6)			32	0 (0.00)	0.99g
Schistosomiasis	186	0 (0.00)			32	0 (0.00)
Other	186	0 (0.00)

Values are n, mean ± SD, n (column %), or median (Q1-Q3). Post hoc pairwise comparisons were performed using Bonferroni adjustment.

^aSignificantly different from comparator group.

^bSignificantly different from healthy control group.

^cSignificantly different from PAH group.

^dAnalysis of variance.

^ePearson chi-square test.

^fIncluded PH medications of endothelin receptor antagonists, phosphodiesterase 5 inhibitors, soluble guanylate cyclase stimulators, and calcium-channel blockers for PH (see the Supplemental Appendix for medication specifics).

^gFisher exact test.

^hKruskal-Wallis test.

ⁱParticipants may have had more than one noted etiology for PAH.

PAH = pulmonary arterial hypertension; PH = pulmonary hypertension; 6MWD = 6-minute walk distance.