Pulmonary Hypertension in Portugal: First Data from a Nationwide Registry

Table 2

Clinical and hemodynamic data stratified by pulmonary arterial hypertension subgroup.

Subgroup	𝑁 (%)	Female (%)	Age (years)	WHO I/II (%)	6MWT (meters)	RAP (mmHg)	mPAP (mmHg)	CO (L·min^−1)	PCW (mmHg)	PVR (WU)
Idiopathic	17 (37.0)	70.6	37.5 ± 12.9	31.3	405 ± 121	11 ± 6	53 ± 15	4.2 ± 1.5	10.7 ± 3.3	11.7 ± 5.6
CTD	12 (26.1)	75.0	56.8 ± 12.4	27.3	275 ± 127	6 ± 6	39 ± 11	4.9 ± 1.8	7.6 ± 3.2	8.7 ± 7
CHD	10 (21.7)	50.0	37.7 ± 15	22.2	351 ± 171	6 ± 5	60 ± 27	4.6 ± 2.7	10.7 ± 3.7	13.9 ± 8.9
PortPulm	5 (10.9)	60.0	51.2 ± 18.3	33.3	n/d	7 ± 5	51 ± 11	4 ± 1.2	8.6 ± 2.9	11.1 ± 3.3
Total	𝟒𝟒(𝟏𝟎𝟎.𝟎)^∗	65.2	43.4 ± 16.4	29.3	371 ± 140	8 ± 6	51 ± 18	4.5 ± 1.8	9.5 ± 3.5	11.4 ± 6.5

CTD: connective tissue disease; CHD: congenital heart disease; PortPulm: portopulmonary. WHO: World Health Organization; 6MWT: six-minute walking test distance; RAP: right atrial pressure; mPAPA: mean pulmonary artery pressure; CO: cardiac output; PCWP: pulmonary capillary wedge pressure; PVR: pulmonary vascular resistance; WU: wood units.
*Heritable PAH (𝑛=1) and other etiologies PAH (𝑛=1) were not reported as there was one case of each in the cohort.