Table 1. Short-term effects of medical treatment in chronic post-embolic pulmonary hypertension.
Treatment | Administration | First author [Ref.] | Study | Duration months | Patients n | NYHA-FC | 6MWD m | Effect m | PVR dyn·s−1·cm−5 | Effect % |
Epoprostenol | i.v. | Cabrol [6] | 3 | 23 | III–IV | 280±112 | +66 | 29±7#,¶ | -21 | |
Treprostinil | Subcutaneously | Skoro-Sajer [7] | 6 or 19 | 25 | III–IV | 260±111 | +59 | 924±347 | -13 | |
Iloprost | Inhaled | Olschewski [8] | RCT | 3 | 57 | III–IV | NA | ns | NA | ns |
Sildenafil | By mouth | Ghofrani [9] | 6 | 12 | ns | 312±30 | +54 | 1935±228+ | -30 | |
Sildenafil | By mouth | Reichenberger [10] | 3 | 104 | II–IV | 310±11 | +51 | 863±38 | -12 | |
Sildenafil | By mouth | Suntharalingam [11] | RCT | 3 | 19 | II–III | 339±58 | +18 (ns) | 734±363 | -27 |
Bosentan | By mouth | Hoeper [12] | 3 | 19 | II–IV | 340±102 | +73 | 914±329 | -33 | |
Bosentan | By mouth | Hughes [13] | 3 | 20 | II–IV | 262±106 | +45 | 1165±392# | -21 | |
Bosentan | By mouth | Bonderman [14] | 6 | 16 | II–IV | 299±131 | +92 | 712±213 | NA | |
Bosentan | By mouth | Seyfarth [15] | 6 | 12 | III | 319±85 | +72 | 1008±428 | NA | |
Bosentan | By mouth | Jaïs [16] | RCT | 4 | 157 | II–III | 342±84 | +2 (ns) | 783 | -24 |
Riociguat | By mouth | Ghofrani [17] | OL | 3 | 41 | II–III | 390 (330–441) | +55 | 691 (533–844) | -29 |
Riociguat | By mouth | Ghofrani [18] | RCT | 4 | 261 | II–III | 349 | +46 | 785 | -31 |
Data are presented as mean±sd or median (interquartile range), unless otherwise stated. NYHA-FC: New York Heart Association functional class; 6MWD: 6-min walking distance; PVR: pulmonary vascular resistance; RCT: randomised controlled trial; OL: open label; NA: not available; ns: not significant. #: total PVR; ¶: in Woods Units·m2; +: in dyn·s−1·cm−5·m−2.