Table 1 Patient characteristics
Etiology [33] | Number (%) |
|---|---|
1. PAH | 11 (28) |
 - 1.1 Idiopathic | 10 |
 - 1.2 Heritable | 1 |
1.4 Associated with (APAH): | 21 (53) |
 - 1.4.1 CTD | 20 |
  - Limited cutaneous systemic sclerosis | 13 |
  - Diffuse cutaneous systemic sclerosis | 2 |
  - Systemic lupus erythematosus | 3 |
  - Sjögren’s syndrome | 1 |
  - Mixed CTD | 1 |
 - 1.4.3 Portal Hypertension | 1 |
4 CTEPH | 8 (20) |
 - Proximal (operable) | 5 |
 - Distal (inoperable) | 3 |
Right Heart Catheterization (RHC) data (n = 29) | |
 Time interval between CMR and RHC (days) | 8 (IQR 12) |
 Mean pulmonary artery pressure (mPAP, mmHg) | 46 ± 13 |
 Pulmonary vascular resistance (PVR, dyn · s/cm5) | 523 (IQR 402–717) |
World Health Organization class (%) | |
 I | 1 (2.5) |
 II | 14 (35) |
 III | 22 (55) |
 IV | 3 (7.5) |
6-MWD (metres) | 372 (IQR 286–450) |
Vasodilator therapy | |
 Phosphodiesterase type 5 inhibitor | 28 (70) |
 Endothelin receptor antagonist | 22 (55) |
 Intravenous prostacyclin | 1 (3) |
 Treatment naive | 8 (20) |
 Oral monotherapy | 14 (35) |
 Dual combination oral therapy | 16 (40) |
 Triple combination therapy | 1 (3) |
Additional PH therapies | |
 Oral prostacyclin | 1 (3) |
 Inhaled prostacyclin | 1 (3) |
 Tyrosine-kinase inhibitor | 2 (5) |