Patient characteristics for module 2: evaluation of the REVEAL prognostic risk score calculator using a validation cohort from the FPHN registry
| Characteristics# | REVEAL development cohort¶ | FPHN validation cohort |
| Subjects n | 2716 | 1737 |
| Age years | 50.4±16.8 | 54.7±16.3 |
| Female:male ratio | 3.7:1 | 1.7:1 |
| Time from diagnosis months | 39.3±45.4 (2716) | 33.0±68.4 (1708) |
| Newly diagnosed PAH+ | 367 (13.5) | 818 (47.9) |
| Previously diagnosed PAH§ | 2349 (86.5) | 890 (52.1) |
| Group I PAH subgroups | ||
| Idiopathic PAH | 1262 (46.5) | 654 (37.7) |
| Familial PAH | 79 (2.9) | 61 (3.5) |
| Associated PAH | ||
| Congenital heart disease | 319 (11.8) | 151 (8.7) |
| Connective tissue disease | 648 (23.9) | 373 (21.5) |
| Portopulmonary hypertension | 138 (5.1) | 285 (16.4) |
| HIV infection | 51 (1.9) | 82 (4.7) |
| Drugs and toxins | 134 (4.9) | 122 (7.0) |
| Other | 85 (3.1) | 9 (0.5) |
| Functional class | ||
| I | 210 (8.5) | 93 (5.4) |
| II | 936 (37.8) | 535 (31.3) |
| III | 1194 (48.2) | 903 (52.7) |
| IV | 136 (5.5) | 181 (10.6) |
| Missing | 240 (8.8) | 25 (1.4) |
| 6MWD m | 370±127 (2173) | 356±130 (1496) |
| Haemodynamics | ||
| RAP mmHg | 8.6±5.3 (2451) | 7.7±5.2 (1421) |
| Mean PAP mmHg | 49.5±14.8 (2623) | 48.3±13.7 (1737) |
| PCWP mmHg | 9.6±4.0 (2598) | 8.4±3.6 (1737) |
| Cardiac index L·min−1·m−2 | 2.6±0.9 (2105) | 2.60.8 (1694) |
| PVR Wood units | 10.5±6.6 (2562) | 9.7±5.6 (1737) |
Data are presented as mean±sd, mean±sd (n) or n (%), unless otherwise stated. REVEAL: Registry to Evaluate Early and Long-term PAH Disease Management; FPHN: French Pulmonary Hypertension Network; PAH: pulmonary arterial hypertension; 6MWD: 6-min walking distance; RAP: right atrial pressure; PAP: pulmonary artery pressure; PCWP: pulmonary capillary wedge pressure; PVR: pulmonary vascular resistance. #: for REVEAL at the time of enrolment, for FPHN at time zero; ¶: data from Benza et al. [2]; +: right heart catheterisation (RHC) diagnosis ≤90 days prior to enrolment date (for REVEAL), or prior to time zero (for FPHN Registry); §: RHC diagnosis >90 days prior to enrolment date (for REVEAL), or prior to time zero (for FPHN Registry).