Advanced therapies for the management of adults with pulmonary arterial hypertension due to congenital heart disease: a systematic review.

Pulmonary arterial hypertension (PAH) secondary to congenital heart disease (CHD) is the third most common cause of PAH, and it is becoming increasingly common as improvements in the management of CHD have led to increased life expectancy for these patients. The medical management of PAH due to CHD (PAH-CHD) is largely the same as what has been used for the treatment of idiopathic PAH, though the body of literature supporting this management decision is very small. There are currently few studies available which specifically focus on the treatment of PAH-CHD. The purpose of this literature review is to compare the results of those studies that assessed the response to medical therapy among adults with PAH-CHD; studies were excluded if they focused on paediatric patients, did not include an assessment of 6 min walking distance or specifically assessed combination therapies. This review found that riociguat, bosentan, epoprostenol and sildenafil were all capable of improving functional capacity and haemodynamic parameters in patients with PAH-CHD, but whether this corresponds to an increase in mortality remains to be seen. Limitations of this review include the small sample size and variable duration of the included studies, which makes drawing direct comparisons between studies and the study drugs difficult. The lack of large, randomised double-blind clinical trials comparing different drugs head to head highlights an area that is ripe for ongoing medical research, the results of which may help shape future treatment algorithms tailored specifically for adults with PAH-CHD.