Table 1. Demographic and clinical characteristics of BMPR2 carriers and non-carriers.
| BMPR2 mutation | ||||
|---|---|---|---|---|
| Total (n = 73) | Carrier (n = 16) | Non-carrier (n = 57) |
P-value | |
| Demography | ||||
| Men, n (%) | 19 (26) | 4 (25) | 15 (26) | 0.857 |
| Age (y), mean±SD | 42.2±19.7 | 27.2±11.1 | 46.6±19.6 | 0.022* |
| Functional and hemodynamic status | ||||
| NYHA functional class ≥II, n (%) | 52 (71) | 10 (63) | 42 (74) | 0.270 |
| mPAP, mmHg | 53.3±16.5 | 63.7±17.3 | 50.5±15.2 | <0.001* |
| PAWP mmHg | 15.8±12.3 | 6.8±2.2 | 10.8±3.5 | 0.020* |
| PVR, mmHg/L/min | 10.2±8.9 | 11.5±9.6 | 9.9 ±8.8 | 0.722 |
| Cardiac output, L/min | 4.2±2.2 | 3.7±1.2 | 4.3±2.4 | 0.092 |
| PAH targeted therapy | ||||
| Prostaglandin I2, n (%) | 20 (27) | 2 (13) | 18 (32) | 0.391 |
| PDE5i, n (%) | 14 (19) | 3 (19) | 11 (19) | 0.854 |
| ERA, n (%) | 38 (52) | 10 (63) | 28 (49) | 0.649 |
| TKI, n (%) | 2 (3) | 0 (0) | 2 (4) | 0.587 |
| Follow-up data | ||||
| Median follow-up years (IQR)† | 1.5 (1.0–2.2) | 1.0 (1.0–2.1) | 1.3 (0.9–2.3) | N/A |
| Death, n (%) | 4 (5) | 0 (0) | 4 (7) | N/A |
Abbreviations: BMPR2, bone morphogenetic protein receptor type 2; SD, standard deviation; NYHA, New York Heart Association; mPAP, mean pulmonary arterial pressure; PAWP, pulmonary artery wedge pressure; PVR, pulmonary vascular resistance; PDE5i, phosphodiesterase-5 inhibitors; ERA, Endothelin receptor antagonists; TKI, tyrosine kinase inhibitors. IQR, interquartile range
*P < 0.05
#Mann-Whitney test
† Median follow-up information was gathered among survivors only