Pulmonary arterial hypertension: Current therapeutic strategies

The treatment of pulmonary arterial hypertension - once a lethal condition - has evolved considerably over the past few years as the number of therapeutic options available to treat this disease has increased. In this Review we attempt to summarize the current knowledge of the pathogenesis of pulmonary hypertension, in relation to the therapies presently available and those that could become available in the near future. The use of prostacyclin and its analogs, calcium-channel blockers, endothelin-receptor antagonists and phosphodiesterase type 5 inhibitors is reviewed. Newer concepts, such as the use of combination therapy, and the potential for long-term disease amelioration and improvement of outcomes, are also discussed. The role of supportive care and medications not specific to pulmonary hypertension is also examined. In addition, we review the novel emerging therapies, such as imatinib, fasudil, simvastatin, ghrelin and vasoactive intestinal peptide, which hold therapeutic potential for disease modification as well as treatment of symptoms.