Male patients | Female patients | |||||
---|---|---|---|---|---|---|
BMPR2 mutation non-carriers n = 79 | BMPR2 mutation carriers n = 34 | p | BMPR2 mutation non-carriers n = 188 | BMPR2 mutation carriers n = 81 | p | |
Age at diagnosis, yrs (mean ± SD) | 47.8 ± 17.1 | 34.6 ± 16.8 | < 0.0001 | 47.4 ± 15.8 | 36.4 ± 14.9 | < 0.0001 |
NYHA | ||||||
I-II | 21 (26.6%) | 6 (17.6%) | < 0.01 | 27 (14.4%) | 11 (13.6%) | 0.90 |
III | 57 (72.2%) | 21 (61.8%) | 135 (71.8%) | 55 (67.9%) | ||
IV | 1 (1.3%) | 6 (17.6%) | 20 (10.6%) | 10 (12.3%) | ||
Six-minute walk test distance, m | 394 ± 96 | 377 ± 98 | 0.49 | 328 ± 119 | 343 ± 102 | 0.35 |
mPAP, mmHg | 57 ± 14 | 63 ± 13 | 0.03 | 56 ± 14 | 62 ± 13 | 0.0007 |
RAP, mmHg | 8 ± 5 | 8 ± 6 | 0.81 | 8 ± 5 | 8 ± 5 | 0.70 |
PCWP, mmHg | 9 ± 3 | 8 ± 3 | 0.39 | 8 ± 3 | 8 ± 3 | 0.84 |
CI, L/min/m² | 2.71 ± 0.83 | 2.17 ± 0.60 | 0.0003 | 2.45 ± 0.66 | 2.07 ± 0.62 | < 0.0001 |
PVRi, mmHg/L/min/m² | 19.6 ± 9.3 | 21.9 ± 13.5 | 0.29 | 20.5 ± 8.5 | 25.1 ± 11.1 | 0.0015 |
SvO 2, % | 67 ± 8 | 59 ± 9 | 0.0015 | 61 ± 10 | 59 ± 10 | 0.09 |
Acute vasodilator responders, % | 9 (11.4%) | 1 (2.9%) | 0.20 | 27 (14.4%) | 1 (1.2%) | < 0.01 |