Haemodynamic, genetic and clinical characteristics of patients with pre-capillary and combined pulmonary hypertension
| Sex/age years | mPAP mmHg | PAWP mmHg | Cardiac index L·min−1·m−2 | PVR WU | Hb g·dL−1 | Mutation gene, exon, nucleotide, change | AVM | FEV1 L (% pred) | FVC L (% pred) | TLC L (% pred) | PaO2 mmHg | CTEPH | Classification subgroups |
| F/60 | 34 | 14 | 3.8 | 3.3 | 10.6 | ALK1, 8, c.1112, dup | P+H | 0.72 (31%) | 1.58 (56%) | 3.94 (80%) | 50.2 | • | 1.2+3+4 |
| F/67 | 36 | 10 | 4.1 | 3.8 | 10.7 | ENG, 4, c.446, G>A | H | 1.93 (94%) | 2.71 (105%) | 4.33 (91%) | 72.7 | • | 1.2+HS |
| F/46 | 29 | 9 | 3.2 | 3.1 | 14.8 | ALK1, 4, c.470, C>T | H | 1.54 (53%) | 2.01 (58%) | 4.92 (93%) | 93 | 1.2+3 | |
| F/82 | 48 | 9 | 3.1 | 6.8 | 13.8 | ALK1, 8, c.1112, dup | H | 1.80 (84%) | 2.80 (103%) | 5.20 (102%) | 99 | • | 1.2+4 |
| F/70 | 41 | 6 | 2.7 | 8.7 | 9.4 | ALK1, 8, c.1112, dup | H | 1.2 | |||||
| F/68 | 24 | 13 | 2.3 | 3.2 | 11.3 | ALK1, 3, c.102, C>A | P+H | 1.56 (92%) | 2.03 (93%) | 3.68 (85%) | 89.2 | 1.2 | |
| M/77 | 34 | 20 | 1.9 | 4.8 | 12.4 | ENG, 12, c.1522, C>T | 0.86 (43%) | 2.34 (86%) | 4.75 (88%) | 81 | • | 1.2+2+3+4 | |
| M/55 | 53 | 23 | 3.7 | 4.9 | 9.2 | ALK1, 8, c.1112, dup | P | 0.49 (17%) | 0.80 (21.5%) | 2.67 (45%) | 44.2 | 1.2+2+3 | |
| F/71 | 49 | 21 | 4.6 | 4.0 | ALK1, 8, c.1112, dup | P+H | 0.79 (37%) | 1.13 (44%) | 1.2+2+3+HS | ||||
| F/57 | 30 | 18 | 2.1 | 3.4 | 13.1 | ALK1, 7, c.956, G>A | 2.28 (97%) | 3.33 (117%) | 5.66 (117%) | 81 | 1.2+2 | ||
| F/80 | 31 | 18 | 2.6 | 3.1 | 10.9 | ALK1, 8, c.1112, dup | P | 1.2+2 | |||||
| F/65 | 45 | 20 | 2.9 | 4.3 | 8.9 | ALK1, 8, c.1112, dup | H | 2.61(101%) | 3.63 (115%) | 5.97 (111%) | 77.2 | 1.2+2 | |
| F/67 | 38 | 22 | 2.4 | 3.6 | ALK1, 8, c.1112, dup | H | 0.64 (29%) | 0.86 (33%) | 88.5 | 1.2+2+3 | |||
| F/63 | 53 | 28 | 4.3 | 3.1 | 9 | ALK1, 3, c.140, G>C | H | 1.2+1.4.3+2 +HS |
mPAP: mean pulmonary arterial pressure; PAWP: pulmonary artery wedge pressure; PVR : pulmonary vascular resistance; WU: Wood units; Hb: haemoglobin; AVM: arteriovenous malformation; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; TLC: total lung capacity; PaO2: arterial oxygen tension; CTEPH: chronic thromboembolic pulmonary hypertension; F: female; M: male; dup: duplication; H: hepatic; P: pulmonary; 1.2: heritable pulmonary hypertension; 1.4.3: portal hypertension; 2: left heart diseases; 3: lung diseases; 4: pulmonary artery obstruction; HS: hyperkinetic state.