Treatment | PH outcomes | Clinical outcomes | |||||
Cardiopulmonary haemodynamic | RV function | Symptoms | Functional capacity | HRQoL | Hospitalisation | Survival | |
Oxygen (n=4) | |||||||
LTOT (n=8) | + | NA | NA | NA | NA | NA | + |
NOT (n=2) | +/− | NA | NA | NA | NA | NA | 0 |
CCBs (n=4) | |||||||
Nifedipine (n=3) | 0 | NA | + | NA | NA | NA | 0 |
Felodipine (n=1) | + | NA | NA | 0 | NA | NA | NA |
PH-targeted therapy (n=9) | |||||||
PDE type 5 inhibitors | |||||||
Sildenafil (n=5) | + | NA | +/− | +/− | +/− | NA | NA |
Tadalafil (n=1) | + | NA | 0 | 0 | 0 | NA | NA |
ERA | |||||||
Bosentan (n=2) | +/− | NA | + | +/− | + | NA | NA |
Ambrisentan (n=1) | NA | + | +/− | 0 | NA | NA | NA |
Statins (n=6) | |||||||
Atorvastatin (n=4) | + | 0 | NA | 0 | NA | NA | NA |
Rosuvastatin (n=1) | + | 0 | 0 | + | 0 | NA | NA |
Pravastatin (n=1) | + | NA | + | + | NA | NA | NA |
RV: right ventricular; HRQoL: health-related quality of life; LTOT: long-term oxygen therapy; NOT: nocturnal oxygen therapy; CCB: calcium channel blocker; PDE: phosphodiesterase; ERA: endothelin receptor antagonist. Clinically relevant effects: +: significant; +/−: uncertain; 0: none; NA: not assessed.