Table 2.
Clinical characteristics and catecholamine measurements of PH phenotypes A–D. Medians (IQR) or n (%) are reported.
| A | B | C | D | ||
|---|---|---|---|---|---|
| (n = 13) | (n = 15) | (n = 3) | (n = 4) | ||
| PH classification | |||||
| IPAH | Yes n (%) | 3 (23.1%) | 6 (40.0%) | 2 (66.7%) | 1 (25.0%) |
| CTD-aPAH | Yes n (%) | 6 (46.2%) | 4 (26.7%) | 0 (0%) | 2 (50.0%) |
| CTEPH | Yes n (%) | 4 (30.8%) | 5 (33.3%) | 1 (33.3%) | 1 (25.0%) |
| Demography | |||||
| Age | Years | 56.0 [47.0, 61.0] | 54.0 [41.5, 71.0] | 63.0 [53.5, 64.0] | 67.0 [56.0, 74.3] |
| Sex | Male n (%) | 6 (46.2%) | 7 (46.7%) | 3 (100%) | 2 (50.0%) |
| Echocardiography | |||||
| TR | mmHg | 53.5 [49.3, 68.8] | 57.0 [44.3, 73.8] | 57.0 [43.5, 63.5] | 62.0 [59.3, 64.5] |
| Right heart catheterisation | |||||
| mPAP | mmHg | 50.0 [47.3, 60.5] | 40.0 [30.0, 47.8] | 60.0 [56.5, 62.5] | 49.5 [45.8, 52.5] |
| CI | L/min/m2 | 2.35 [2.15, 2.68] | 3.20 [1.60, 3.60] | 2.10 [2.03, 2.45] | 2.90 [2.30, 3.23] |
| PCWP | mmHg | 11.0 [9.50, 15.0] | 10.0 [8.75, 14.0] | 12.0 [11.0, 12.0] | 13.0 [11.8, 14.0] |
| PVR | Wood units | 10.0 [6.35, 12.8] | 5.40 [4.13, 10.5] | 12.0 [10.0, 12.5] | 7.95 [6.25, 12.0] |
| Laboratory parameter | |||||
| NT-proBNP | % max ULN | 208 [98.9, 517] | 141 [87.2, 265] | 809 [637, 864] | 1980 [1710, 2210] |
| ELISA measurements | |||||
| Epinephrine | pg/mL | 15.3 [6.54, 27.2] | 10.9 [6.15, 25.0] | 16.7 [15.1, 22.1] | 26.1 [19.3, 45.9] |
| Norepinephrine | pg/mL | 358 [170, 477] | 328 [159, 428] | 496 [260, 864] | 644 [352, 964] |
| Outcome | |||||
| Walking distance | Yes n (%) | 426 [394, 504] | 552 [475, 614] | 410 [325, 481] | 443 [379, 498] |
| WHO functional class | |||||
| I | Yes n (%) | 1 (7.7%) | 2 (13.3%) | 0 (0%) | 0 (0%) |
| II | Yes n (%) | 7 (53.8%) | 9 (60.0%) | 0 (0%) | 1 (25.0%) |
| III | Yes n (%) | 5 (38.5%) | 4 (26.7%) | 3 (100%) | 2 (50.0%) |
| IV | Yes n (%) | 0 (0%) | 0 (0%) | 0 (0%) | 1 (25.0%) |
CI, cardiac index; CTD-aPAH, connective tissue disease associated PAH; CTEPH, chronic thromboembolic pulmonary hypertension; IPAH, idiopathic pulmonary arterial hypertension; mPAP, mean pulmonary arterial pressure; NT-proBNP, N-terminal (NT)-pro brain natriuretic peptide (BNP) ; PCWP, pulmonary capillary wedge pressure; PVR, pulmonary vascular resistance; TR, tricuspid regurgitation; ULN: upper limit of normal.