Table 1. Patients' characteristics (n = 21).
| PAH Type | |
| IPAH | n = 9 |
| PAH-Her | n = 1 |
| PAH-CTD | n = 9 |
| PAH-CHD* | n = 2 |
| Sex (F/M) | (17/4) |
| Age (years) | 54 (15) |
| BMI (kg·m−2) | 27 (5) |
| WHO Functional Class (II/III) | (15/6) |
| Pulmonary Hemodynamics ** | |
| RAP (mmHg) | 6.6 (3.5) |
| mPAP (mmHg) | 42 (12) |
| PCWP (mmHg) | 11 (3) |
| CI (l/min·m2) | 3.0 (0.6) |
| PVR (WU·m2) | 6.6 (3.1) |
| 6MWD (m) | 447 (96) |
| 6MWD (% predicted) | 83 (17) |
Values are n or mean (SD).
* Includes two patients with Eisenmenger physiology related to persistent arterial canal and ventricular septal defect.
** Right heart catheterization performed <6 months was used to describe hemodynamic severity.
Table legend: PAH, Pulmonary arterial hypertension; IPAH, Idiopathic PAH; PAH-Her, Heritable PAH; PAH-CTD, PAH associated with connective tissue disease; PAH-CHD, PAH associated with congenital heat disease; BMI, Body mass index; WHO, World Health Organization; RAP, Right atrial pressure; mPAP, Mean pulmonary artery pressure; PCWP, Pre-capillary wedge pressure; CI, Cardiac index; PVRi, Pulmonary vascular resistance index; 6MWD, six-minute walk distance.