| First author [ref.] | Patients | Therapy | NYHA/WHO FC at initiation of therapy I/II/II/IV % | Treatment length | Positive parameters versus placebo |
| Prospective, multicentre studies | |||||
| Echocardiography | |||||
| Galie [55] | IPAH or PAH-CTD (n=85); bosentan (n=56) or placebo (n=29) | Bosentan | 0/0/84/16 | 16 weeks | Lower increase in right ventricular area and less worsening of right ventricular per cent area compared with placebo Treatment improved right:left ventricular diastolic areas ratio, increased right ventricular ejection time, stroke volume and cardiac index, and improved Doppler right ventricular index Trend towards a reduction of maximal TRV |
| Hinderliter [36] | Severe IPAH (n=81; n=41 treated with prostacyclin) | Epoprostenol | 0/0/74/26 | 12 weeks | Beneficial effects on right ventricular size, curvature of the interventricular septum and maximal TRV No significant effects of treatment on right ventricular per cent change in area or pericardial effusion size |
| MRI | |||||
| Wilkins [56] | PAH (n=42) | Simvastatin add-on therapy | 0/0/52/48 | 6–12 months | Significant decrease in right ventricular mass over the first 6-month period; no significant changes in RVEF or Tei index, or right ventricular systolic pressure as estimated by echocardiography At 12 months, the decrease in right ventricular mass seen on active treatment appeared to have reversed in those who continued on simvastatin |
NYHA/WHO FC: New York Heart Association/World Health Organization functional class; MRI: magnetic resonance imaging; PAH: pulmonary arterial hypertension; IPAH: idiopathic PAH; PAH-CTD: PAH associated with connective tissue disease; TRV: tricuspid regurgitant velocity; RVEF: right ventricular ejection fraction.