TABLE 5.
Comparison of clinical and haemodynamic characteristics at diagnosis of pulmonary arterial hypertension (PAH) patients identified in DELPHI-2 and the index cases in their family
| Index cases | DELPHI-2 PAH patients | p-value | |
| Subjects | 31 | 5 | |
| Clinical characteristics | |||
| Female | 22 (71) | 4 (80) | 0.90 |
| Age years | 38 (5–63); (26–47) | 50 (26–78); (41–74) | 0.06 |
| NYHA FC | |||
| I | 0 | 3 | <0.001 |
| II | 4 | 2 | |
| III | 18 | 0 | |
| IV | 7 | 0 | |
| 6MWD m | 356 (0–530); (304–400) | 420 (397–533); (408–512) | 0.02 |
| Haemodynamic characteristics | |||
| mPAP mmHg | 58 (45–95); (51.5–72.3) | 29 (25–50); (25.8–44.8) | <0.001 |
| Cardiac output L·min−1 | 3.46 (1.85–5.9); (2.65.9–4.13) | 4.05 (3.27–7.27); (3.77–5.25) | 0.14 |
| Cardiac index L·min−1·m−2 | 2.10 (1.14–3.7); (1.61–2.42) | 2.36 (1.94–4.38); (2.22–3.20) | 0.14 |
| PAWP mmHg | 8 (1–19); (5–9) | 7 (5–8); (6.5–8) | 0.6 |
| PVR WU | 15.4 (7.6–31.8); (12.1–22.1) | 7.3 (2.5–9.2); (4.0–9.2) | 0.001 |
Data are presented as n, n (%) or median (minimum–maximum range); (interquartile range), unless otherwise stated. NYHA FC: New York Heart Association Functional Class; 6MWD: 6-min walk distance; mPAP: mean pulmonary arterial pressure; PAWP: pulmonary arterial wedge pressure; PVR: pulmonary vascular resistance.