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Table 1 PAH Biobank cohort demographic and hemodynamic data

From: Novel risk genes and mechanisms implicated by exome sequencing of 2572 individuals with pulmonary arterial hypertension

 

All

IPAH

APAH

FPAH

Other*

Total (%)

2572

1110 (43.2)

1239 (48.2)

101 (3.9)

122 (4.7)

Age of onset, n (%)

 Child (dx age < 19)

226 (8.8)

94 (8.5)

112 (9.0)

15 (14.9)

5 (4.1)

 Adult (dx age > =19)

2345 (91.2)

1015 (91.4)

1127 (91.0)

86 (85.1)

117 (95.9)

 Mean age

48 ± 19

48 ± 18

49 ± 19

37 ± 15

47 ± 15

Gender, n (%)

 Female

2023 (78.7)

868 (78.2)

996 (80.4)

69 (68.3)

90 (73.8)

 Male

549 (21.3)

242 (21.8)

243 (19.6)

32 (31.7)

32 (26.2)

 Female to male ratio

3.7:1

3.6:1

4.1:1

2.2:1

2.8:1

Ancestry, n (%)

 European

1852 (72)

809 (73.0)

855 (69.0)

89 (88.1)

99 (81.2)

 Hispanic

315 (12.3)

137 (12.3)

156 (12.6)

10 (9.9)

12 (9.8)

 African

292 (11.4)

117 (10.5)

168 (13.6)

1 (1)

6 (4.9)

 East Asian

70 (2.7)

25 (2.2)

41 (3.3)

0

4 (3.3)

 South Asian

28 (1.1)

12 (1.1)

15 (1.2)

1 (1)

0

 Others

15 (0.58)

10 (0.9)

4 (0.3)

0

1 (0.8)

Hemodynamic parameters

 MPAP (mmHg)

50 ± 14

52 ± 14

48 ± 14

58 ± 14

52 ± 13

 MPCW (mmHg)

10 ± 4

10 ± 4

10 ± 4

10 ± 4

11 ± 4

 CO, Fick (L/min)

4.5 ± 1.8

4.5 ± 1.7

4.6 ± 1.9

3.6 ± 1.0

4.2 ± 1.3

 PVR (Woods units)

10.7 ± 7.0

11.2 ± 7.0

10.0 ± 7.1

14.9 ± 6.3

11.0 ± 6.6

 MAP (mmHg)

90 ± 19

91 ± 20

90 ± 19

88 ± 16

94 ± 19

 MAP:MPAP

1.9 ± 0.7

1.9 ± 0.7

2.0 ± 0.7

1.6 ± 0.5

1.9 ± 0.5

  1. Abbreviations: MPAP mean pulmonary arterial pressure, MPCW mean pulmonary capillary wedge pressure, CO cardiac output by Frick’s method, PVR pulmonary vascular resistance, MAP mean arterial pressure
  2. *Other included 110 diet- and toxin-induced PAH, 11 non-familial pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis, and 1 persistent pulmonary hypertension of the newborn