Key result
PAH treatment expands beyond epoprostenol to include inhaled therapies, oral agents, and combination regimens.
Why the study?
Significant advances in PAH treatment, revisions to disease classification, and emerging long-term and combination therapy data over the preceding 10 years prompted a synthesis of the current evidence for diagnosis, prognosis, and treatment.
This review summarizes a decade of advances in the classification, diagnosis, prognosis, and treatment of pulmonary arterial hypertension, including prostacyclins, oral therapies, and combination regimens.
Improved PAH prognosis with expanded therapies; leaves open optimal sequencing and combinations for trials.
Significant advances in the treatment of pulmonary arterial hypertension (PAH) have occurred over the last 10 years, starting with the approval of epoprostenol in 1998. Subsequently, multiple additional medications have received approval, including a subcutaneous prostacyclin, an inhaled prostacyclin, and oral medications in 2 separate classes. Over this same period, the classification of pulmonary hypertension has been revised with changes including the substitution of the term idiopathic for primary PAH and an expanded list of conditions felt to be associated with the development of PAH. Long-term follow-up studies have provided better information on prognosis and expected outcomes with treatment, with particularly valuable data on reassessment of prognosis after treatment with epoprostenol. Combination therapy is more frequently being used, and limited data on novel therapies such as stem cell transplantation have been published. The purpose of this review is to describe the current state of evidence for the diagnosis, prognosis, and treatment of the patient with PAH.
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Chin et al. (2008) conducted a review in Pulmonary arterial hypertension. Epoprostenol, subcutaneous and inhaled prostacyclins, oral medications, combination therapy, and stem cell transplantation was evaluated. Advances in pulmonary arterial hypertension treatment since epoprostenol approval in 1998 include subcutaneous and inhaled prostacyclins, two classes of oral medications, and combination therapy.
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