Table 1.
Baseline patient characteristics
| Patient Characteristics | All PAH (n=112) |
CTD-PAH (n=27) |
IPAH (n=45) |
Tox-PAH (n=20) |
CHD-PAH (n=20) |
|---|---|---|---|---|---|
| Age (year) | 51.6 (14.4) | 58.6 (11.5) | 53.4 (14.4) | 47.8 (8.8) | 41.8 (16.9) |
| Female sex | 82% (92) | 89% (24) | 76% (34) | 90% (18) | 84% (16) |
| BMI (kg/m2) | 29.1 (7.3) | 27.6 (6.5) | 30.8 (7.7) | 32.1 (6.8) | 24.1 (4.8) |
| NYHA Functional Class | |||||
| I/II | 66% (57) | 65% (13) | 71% (25) | 44% (7) | 75% (12) |
| III | 22% (25) | 30% (6) | 20% (7) | 50% (8) | 25% (4) |
| IV | 6% (5) | 5% (1) | 9% (3) | 6% (1) | 0% (0) |
| 6MWD (meters) | 366 (107) | 334 (105) | 387 (119) | 358 (84) | 372 (102) |
| Deceased | 17% (19) | 33% (9) | 9% (4) | 15% (3) | 15% (3) |
| PAH Therapy | |||||
| Monotherapy | 32% (36) | 41% (11) | 27% (12) | 35% (7) | 30% (6) |
| Dual Therapy | 35% (39) | 33% (9) | 38% (18) | 40% (8) | 25% (5) |
| Triple Therapy | 19% (21) | 19% (5) | 24% (11) | 10% (2) | 15% (3) |
| No Therapy | 14% (16) | 7% (2) | 11% (5) | 15% (3) | 30% (6) |
| Right Heart Catheterization | All PAH (n=95) |
CTD-PAH (n=25) |
IPAH (n=36) |
Tox-PAH (n=18) |
CHD-PAH (n=16) |
| RAP (mmHg) | 8.9 (5.8) | 7.9 (5.4) | 9.9 (5.7) | 10.1 (5.9) | 6.7 (6.2) |
| mPAP (mmHg) | 47.1 (12.7) | 42.8 (10.8) | 49.5 (11.4) | 51.0 (11.2) | 44.1 (17.3) |
| PCWP (mmHg) | 10.4 (3.4) | 9.6 (3.5) | 11.2 (3.4) | 11.2 (2.7) | 9.1 (3.8) |
| CI (L/min/m2) | 2.6 (0.8) | 2.3 (0.6) | 2.6 (0.7) | 2.3 (0.7) | 3.3 (0.6) |
| PVR (Wood units) | 8.8 (4.8) | 9.5 (5.3) | 8.6 (4.4) | 10.0 (5.0) | 6.6 (4.3) |
| Echocardiography | All PAH (n=88) |
CTD-PAH (n=16) |
IPAH (n=39) |
Tox-PAH (n=18) |
CHD-PAH (n=13) |
| RV Diameter (mm) | 46 (9) | 42 (8) | 47 (10) | 47 (7) | 45 (1) |
| TAPSE (mm) | 21 (6) | 20 (4) | 22 (6) | 19 (6) | 20 (6) |
Data are presented as mean (standard deviation) or % (n). Functional class was available for 88 patients (79%) and 6MWD data for 98 (87.5%). For the 95 patients undergoing right heart catheterization, right atrial pressure data was available for 86 patients (91%), wedge pressure for 84 (88%), cardiac index for 73 (77%) and pulmonary vascular resistance for 70 (74%). Of the 88 patients with an echocardiogram, RV diameter and TAPSE were not measurable in 2 patients. PAH: pulmonary arterial hypertension; IPAH: idiopathic PAH; CTD-PAH: connective tissue disease-associated PAH; Tox-PAH: toxin-associated PAH; CHD-PAH: congenital heart disease-associated PAH; BMI: body mass index; NYHA: New York Heart Association; 6MWD: 6-minute walk distance; RAP: right atrial pressure; mPAP: mean pulmonary artery pressure; PCWP: pulmonary capillary wedge pressure; CI: cardiac index; PVR: pulmonary vascular resistance; RV: right ventricle; TAPSE: tricuspid annular plane systolic excursion.