Pulmonary arterial hypertension is a disease defined by the mean pulmonary arterial pressure same or greater than 25 mmHg. Pulmonary arterial hypertension belongs to the first group of pulmonary hypertension by distribution of world health organisation. According to world health organisation, Pulmonary arterial hypertension is divided into idiophatic, heritable pulmonary arterial hypertension, pulmonary arterial hypertension associated with drugs and toxins and pulmonary arterial hypertension with other systemic diseases. The guidelines recommend specific treatment of pulmonary arterial hypertension with medicine groups such as endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, prostacyclin analogues, soluble guanylate cyclase stimulators and prostacyclin receptor agonists.
Our purpose was to systematically review the latest evidence for the efficacy of drugs and drug groups for the treatment of pulmonary arterial hypertension and to present results for drug efficacy and drug effects on mortality for ambrisentan, bosentan, macitentan, sildenafil, tadalafil, beraprost, epoprostenol, iloprost, treprostinil, riociguate and selexipag.
We included 11 active substances in the search set. Studies were included if they meet exclusion and inclusion criteria. The included studies were systematically reviewed and performed meta-analysis with Review Manager 5.3. The results were reported as the efficacy of the active substances, with the mean diffrences of six minutes walk distance and the impact on mortality with the odds ratio for all studies, for studies with no associated treatment, for studies with conventional background therapy and for studies with specific background therapy.
Epoprostenol has been found to be the most effective and has the greatest impact on mortality for all studies and for studies with conventional background therapy. with specific background therapy, iloprost is the most effective and riociguate has the greatest impact on mortality. The results of our meta-analysis are comparable to the results of previously published meta-analyzes.