Table 2.
Pulmonary hypertension disease severity and treatment.
| Characteristic | n | IPAH | n | POPH | p |
|---|---|---|---|---|---|
| Incident cases (diagnosed within 6 months) | 344 | 166 (48.3) | 57 | 31 (54.4) | 0.39 |
| Prevalent cases (diagnosed more than 6 months prior to registry entry) | 344 | 178 (51.7) | 57 | 26 (45.6) | 0.39 |
| Treated more than 6 months | 344 | 94 (27.3) | 57 | 12 (21.1) | 0.32 |
| Treated <6 months | 344 | 84 (24.4) | 57 | 14 (24.6) | 0.98 |
| World Health Organization functional class | 315 | 53 | 0.84 | ||
| 1 | 31 (9.8) | 2 (3.8) | |||
| 2 | 109 (34.6) | 24 (45.3) | |||
| 3 | 157 (49.8) | 26 (49.1) | |||
| 4 | 18 (5.7) | 1 (1.9) | |||
| Quality of life | 340 | 55 | |||
| SF-36 physical component summary | 33.6 ± 6.6 | 34.9 ± 5.9 | 0.2 | ||
| SF-36 mental component summary | 49.0 ± 8.3 | 48.7 ± 9.0 | 0.82 | ||
| EMPHASIS-10 score | 24.1 ± 12.4 | 24.2 ± 11.2 | 0.96 | ||
| Laboratory data | |||||
| Creatinine | 332 | 1.0 ± 0.5 | 55 | 1.0 ± 0.7 | 0.93 |
| BNP | 198 | 329.5 ± 684.6 | 29 | 231.8 ± 362.1 | 0.24 |
| N-terminal pro BNP (NTproBNP) | 147 | 1811.7 ± 3597.8 | 22 | 2001.6 ± 3463.0 | 0.82 |
| 6-min walk distance, m | 287 | 344.6 ± 141.8 | 52 | 351.2 ± 82.0 | 0.64 |
| Hemodynamics | |||||
| Right atrial pressure, mmHg | 323 | 10.1 ± 5.9 | 56 | 10.3 ± 5.0 | 0.87 |
| Pulmonary artery systolic pressure | 332 | 81.0 ± 21.0 | 56 | 82.2 ± 15.7 | 0.61 |
| Pulmonary artery diastolic pressure | 332 | 33.7 ± 11.7 | 56 | 33.1 ± 8.5 | 0.62 |
| Mean pulmonary arterial pressure, mmHg | 327 | 51.2 ± 14.1 | 56 | 50.9 ± 10.4 | 0.84 |
| Pulmonary artery wedge pressure, mmHg | 320 | 11.6 ± 6.0 | 54 | 11.4 ± 5.0 | 0.82 |
| Cardiac output, L/min | 311 | 4.23 ± 1.37 | 56 | 5.50 ± 2.24 | <0.001 |
| Cardiac index, L/min/m2 | 306 | 2.20 ± 0.67 | 54 | 2.84 ± 1.22 | <0.001 |
| Pulmonary vascular resistance, Wood units | 295 | 10.5 ± 5.5 | 54 | 8.2 ± 3.8 | <0.001 |
| Stroke volume, mL | 218 | 55.3 ± 21.0 | 44 | 79.3 ± 27.5 | <0.001 |
| Stroke volume index, ml/m2 | 213 | 28.9 ± 10.2 | 42 | 40.5 ± 13.9 | <0.001 |
| Pulmonary artery compliance, mL/mmHg | 218 | 1.33 ± 0.94 | 44 | 1.78 ± 0.91 | 0.004 |
| Risk stratification at enrollment | |||||
| REVEAL 2.0 score | 344 | 7.2 ± 2.7 | 57 | 9.8 ± 2.7 | <0.001 |
| REVEAL 2.0 score (without POPH diagnosis) | 344 | 7.2 ± 2.7 | 57 | 6.8 ± 2.7 | 0.24 |
| French Registry # low-risk criteria (non-invasive) | 344 | 57 | 0.81 | ||
| 0 | 140 (40.7) | 21 (36.8) | |||
| 1 | 127 (36.9) | 22 (38.6) | |||
| 2 | 56 (16.3) | 12 (21.1) | |||
| 3 | 21 (6.1) | 2 (3.5) | |||
| French registry # low-risk criteria (invasive) | 344 | 57 | 0.14 | ||
| 0 | 101 (29.4) | 13 (22.8) | |||
| 1 | 121 (35.2) | 16 (28.1) | |||
| 2 | 75 (21.8) | 17 (29.8) | |||
| 3 | 38 (11.1) | 11 (19.3) | |||
| 4 | 9 (2.6) | 0 (0.0) | |||
| PAH therapy at enrollment | |||||
| Number of PAH medications | 341 | 1.7 ± 0.9 | 56 | 1.4 ± 0.8 | 0.01 |
| Combination therapy | 341 | 212 (62.2) | 56 | 26 (46.4) | 0.03 |
| Phosphodiesterase type 5 inhibitor | 343 | 240 (70.0) | 56 | 43 (76.8) | 0.3 |
| Riociguat | 343 | 12 (3.5) | 56 | 1 (1.8) | 1 |
| Endothelin receptor antagonist | 341 | 189 (55.4)a | 56 | 16 (28.6) | <0.001 |
| Bosentan | 7 (2.1) | 0 (0.0) | 0.6 | ||
| Ambrisentan | 98 (28.7) | 11 (19.6) | 0.16 | ||
| Macitentan | 85 (24.9) | 5 (8.9) | 0.008 | ||
| Parenteral prostacyclin | 344 | 86 (25.0) | 57 | 10 (17.5) | 0.22 |
| Inhaled prostacyclin | 341 | 20 (5.9) | 56 | 2 (3.6) | 0.75 |
| Selexipag | 341 | 17 (5.0) | 56 | 4 (7.1) | 0.52 |
| Oral treprostinil | 341 | 7 (2.1) | 56 | 0 (0.0) | 0.6 |
| First follow-up visit | |||||
| Number of outpatient visits with PH doctor or nurse in clinic since enrollment | 221 | 1.9 ± 1.6 | 34 | 2.3 ± 1.5 | 0.26 |
| PAH therapy at first follow-up visit | |||||
| Number of PH medications | 234 | 1.0 ± 0.8 | 38 | 1.8 ± 0.7 | 0.49 |
| Combination therapy | 234 | 170 (72.7) | 38 | 25 (65.8) | 0.38 |
| Phosphodiesterase type 5 inhibitor | 235 | 173 (73.6) | 40 | 36 (90.0) | 0.03 |
| Riociguat | 234 | 19 (8.1) | 38 | 0 (0.0) | 0.08 |
| Endothelin receptor antagonist | 234 | 147 (62.8) | 38 | 19 (50.0) | 0.13 |
| Parenteral prostacyclin | 234 | 59 (25.2) | 39 | 8 (20.5) | 0.53 |
| Inhaled prostacyclin | 234 | 15 (6.4) | 38 | 2 (5.3) | 1 |
| Selexipag | 234 | 21 (9.0) | 38 | 3 (7.9) | 1 |
| Oral treprostinil | 234 | 8 (3.4) | 38 | 0 (0.0) | 0.61 |
| Health-care utilization | |||||
| ED visit within last 6 months | 343 | 179 (52.2) | 56 | 39 (69.6) | 0.02 |
| Number of ED visits within last 6 months | 343 | 0.9 ± 1.2 | 56 | 1.7 ± 2.1 | 0.009 |
| Hospitalized within last 6 months | 343 | 169 (49.3) | 56 | 33 (58.9) | 0.18 |
| Number of hospitalizations within last 6 months | 343 | 0.8 ± 1.1 | 56 | 1.5 ± 2.1 | 0.02 |
| Number of nights in hospital within last 6 months | 343 | 6.3 ± 11.5 | 56 | 10.1 ± 12.2 | 0.02 |
BNP: B-type natriuretic peptide; ED: emergency department; NTproBNP: N-terminal pro B-type natriuretic peptide; PAH: pulmonary arterial hypertension; POPH: portopulmonary hypertension; SF-36: Short Form 36; IPAH: idiopathic pulmonary arterial hypertension. Statistically significant p-values (<0.05) are denoted in bold.
aNumber of IPAH patients treated with an endothelin receptor antagonist does not equal number of patients treated with individual endothelin receptor antagonists since one patient was recorded as being on both ambrisentan and macitentan at time of enrollment.