Advances in medical therapy for pulmonary arterial hypertension.

ABSTRACT

PURPOSE OF REVIEW The purpose of this review is to demonstrate advances in the medical treatment of pulmonary arterial hypertension (PAH). Reviewed will be the evidence that favors the use of risk assessment in the treatment of PAH. Optimization of combination therapy depending on the risk or worsening will be reviewed. Finally, recent advances in new treatment strategies will be mentioned. RECENT FINDINGS: The use of therapies in sequence or in combination for the treatment of PAH has been shown to decrease morbidity and mortality. Tailoring these treatment strategies to a risk of worsening has been shown to decrease mortality and time to clinical worsening because of PAH. In addition, there have been several advances in the development of other medications separate from the three known pathogenic pathways in PAH. SUMMARY: In the last 15 years, 12 specific therapies have been approved for PAH. These therapies target three separate pathogenic pathways [the endothelin (ET), nitric oxide (NO) and prostacyclin (PGI2)]. As a result, treatment guidelines have tailored the treatment of PAH with these medications either as single drug therapy or in combination. Recently, other treatment pathways have been explored as new strategies for the treatment of PAH.