Table 1.
Demographics and clinical measurements at diagnosis
| All patients |
BMPR2 mutation status |
||||
|---|---|---|---|---|---|
| Non-carriers (N=1102) | Carriers (N=448) | p value | |||
| Age at diagnosis (N=1447), years | 40·1 (17·2) | 42·0 (17·8) | 35·4 (14·8) | <0·0001 | |
| Male sex | 440/1545 (28%) | 302/1097 (28%) | 138/448 (31%) | 0·20 | |
| Family history of PAH | 202/1376 (15%) | .. | 202/402 (50%) | .. | |
| Body-mass index (N=1206), kg/m2 | 24·9 (9·1) | 24·9 (10·6) | 24·9 (5·9) | 0·99 | |
| 6-min walk distance (N=1072), m | 378 (124) | 374 (128) | 388 (113) | 0·088 | |
| NYHA functional class | 0·38 | ||||
| I–II | 423/1426 (30%) | 313/1031 (30%) | 110/394 (28%) | ||
| III | 896/1426 (63%) | 647/1031 (63%) | 249/394 (63%) | ||
| IV | 107/1426 (8%) | 72/1031 (7%) | 35/394 (9%) | ||
| Mean pulmonary artery pressure (N=1503), mm Hg | 57·6 (15·0) | 56·4 (15·3) | 60·5 (13·8) | <0·0001 | |
| Pulmonary vascular resistance (N=1300), Wood units | 14·0 (8·4) | 12·9 (8·3) | 16·6 (8·3) | <0·0001 | |
| Right atrial pressure (N=1253), mm Hg | 8·2 (5·5) | 8·0 (5·7) | 8·6 (5·2) | 0·065 | |
| Cardiac output (N=1202), L/min | 3·98 (1·44) | 4·20 (1·50) | 3·50 (1·17) | <0·0001 | |
| Cardiac index (N=1358), L/min per m2 | 2·40 (0·88) | 2·51 (0·92) | 2·11 (0·69) | <0·0001 | |
| Vasodilator responder | 157/1287 (12%) | 147/907 (16%) | 10/380 (3%) | <0·0001 | |
Data are n/N (%) or mean (SD), unless otherwise stated. PAH=pulmonary arterial hypertension. NYHA=New York Heart Association.