Absence of influence of gender and BMPR2 mutation type on clinical phenotypes of pulmonary arterial hypertension

Table 2 Baseline hemodynamic characteristics of BMPR2 mutation carriers according to mutation types.

	*BMPR2* missense mutation carriers n = 32	*BMPR2* truncating mutation carriers n = 51	*BMPR2* large rearrangement or splice defect mutation carriers n = 32
Age at diagnosis, *yrs* (mean ± SD)	35.8 ± 16.9	37.3 ± 14.0	33.5 ± 16.3
NYHA
I-II	5 (15.6%)	5 (9.8%)	7 (21.8%)
III	21 (65.6%)	37 (72.5%)	18 (56.3%)
IV	3 (9.4%)	7 (13.7%)	6 (18.8%)
Six-minute walk test distance, m	382 ± 107	351 ± 91	325 ± 109
mPAP, *mmHg*	63 ± 14	62 ± 12	62 ± 13
RAP, *mmHg*	6 ± 5*	9 ± 5	8 ± 5
PCWP, *mmHg*	8 ± 3	7 ± 2	8 ± 4
CI, *L/min/m²*	2.31 ± 0.68^¶	1.94 ± 0.41	2.16 ± 0.75
PVRi, *mmHg/L/min/m²*	24.9 ± 10.8	25.0 ± 10.5	22.5 ± 14.9
SvO _2, %	60 ± 9	57 ± 10	60 ± 9
Acute vasodilator responders, %	1(3.1%)	1 (1.9%)	0.9

*p = 0.02 compared to BMPR2 truncating mutation carriers
^¶p = 0.01 compared to BMPR2 truncating mutation carriers
BMPR2: bone morphogenetic protein receptor type 2, PAH: pulmonary arterial hypertension, NYHA = New York Heart Association, mPAP = mean pulmonary artery pressure, RAP = right atrial pressure, PCWP = pulmonary capillary wedge pressure, CI = cardiac index, PVRi = indexed pulmonary vascular resistance, SvO2 = mixed venous oxygen saturation, NO = nitric oxide.
Results are expressed as mean ± SD

ISSN: 1465-993X