Table 1 Baseline characteristics of the study cohort
Study cohort (n = 15) | ||
|---|---|---|
Mean ± standard deviation or n and % | ||
Sex (male/female) | 3/12 | |
Age, years | 59.9 ± 13.7 | |
Height, cm | 169.5 ± 6.9 | |
Weight, kg | 92.3 ± 31.3 | |
WHO Functional class | ||
III | 10 | (66.7%) |
IV | 5 | (33.3%) |
Diagnosis | ||
Idiopathic pulmonary arterial hypertension (PAH) | 10 | (66.6%) |
Heritable PAH | 1 | (6.7%) |
Connective tissue disease associated PAH | 3 | (20.0%) |
Portopulmonary hypertension | 1 | (6.7%) |
Time since initial diagnosis, months | 45.4 ± 23.7 | |
Concomitant disease | ||
Cardiac | ||
Systemic arterial hypertension | (66.7%) | |
Coronary artery disease | (20.0%) | |
Atrial fibrillation | (13.3%) | |
Pulmonary | ||
History of pulmonary embolism | (26.7%) | |
Obstructive sleep apnea | (20.0%) | |
Chronic obstructive pulmonary disease | (6.7%) | |
Other | ||
Systemic sclerosis | (20.0%) | |
Hypothyreosis | (13.3%) | |
History of stroke | (13.3%) | |
Chronic renal insufficiency | (13.3%) | |
Hepatic cirrhosis | (6.7%) | |
History of splenectomy | (6.7%) | |
Diabetes mellitus | (6.7%) | |
PAH-targeted medication | ||
Endothelin receptor antagonists | ||
Bosentan | 1 | (6.7%) |
Ambrisentan | 4 | (26.7%) |
Macitentan | 9 | (60.0%) |
Phosphodiesterase-5 inhibitors/ soluble guanylate cyclase stimulator | ||
Sildenafil | 4 | (26.7%) |
Tadalafil | 5 | (33.3%) |
Riociguat | 6 | (40.0%) |
Prostanoids before starting Epoprostenol | ||
Treprostinil s.c | 1 | (6.7%) |
Iloprost inhalative | 2 | (13.3%) |
Iloprost i.v. | 1 | (6.7%) |