Table 5 Genotype-phenotype correlation between PAH patients with and without a BMPR2 mutation
From: Genetic analyses in a cohort of 191 pulmonary arterial hypertension patients
Characteristic | PAH with a BMPR2 mutation | PAH without a BMPR2 mutation | P value |
|---|---|---|---|
Age at diagnosis, y | 27.2 ± 9.9 | 31.6 ± 10.5 | 0.0003 |
Female sex | 38 (67.9%) | 99 (76.7%) | Â |
NYHA I-II | 27 (48.2%) | 56 (43.4%) | Â |
NYHA III | 28 (50.0%) | 67 (51.9%) | Â |
NYHA IV | 1 (1.8%) | 6 (4.7%) | Â |
Hemodynamics at diagnosis | |||
RAP, mmHg | 5.1 ± 4.4 | 5.1 ± 4.2 | ns |
mPAP, mmHg | 60.2 ± 15.3 | 54.9 ± 14.7 | 0.0321 |
PVR, dyn*s*cm−5 | 1386.2 ± 641.4 | 1042.1 ± 417.0 | 0.0024 |
PAWP, mmHg | 6.9 ± 3.5 | 7.2 ± 3.5 | ns |
CI, L/min/m2 | 2.6 ± 0.9 | 3.0 ± 0.9 | 0.0017 |
SvO2, % | 64.8 ± 8.1 | 69.4 ± 6.6 | 0.0005 |
Peak VO2, ml/min/Kg | 11.7 ± 2.7 | 13.9 ± 3.6 | 0.0096 |
6-min walk distance, m | 396.8 ± 106.1 | 427.0 ± 97.7 | ns |
NT-proBNP, pg/ml | 1432.1 ± 1326.2 | 1327.4 ± 1283.9 | ns |