Clinics in chest medicine
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Clinics in chest medicine · Dec 2013
ReviewStandard nonspecific therapies in the management of pulmonary arterial hypertension.
Recent advances in pulmonary arterial hypertension (PAH) research have created a new era of PAH-specific therapies. Although these therapeutics have revolutionized PAH therapy, their innovation was predated by supportive but nonspecific medical therapies adapted from their use in more common cardiopulmonary diseases. ⋯ Expert opinion continues to support the use of adjunct therapies based on current pathologic understandings of PAH combined with some evidence extrapolated from small studies. This article discusses why these therapies continue to play an important role in the treatment of patients with PAH.
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Clinics in chest medicine · Dec 2013
Review Historical ArticleEpidemiology of pulmonary arterial hypertension.
Changes in the epidemiology of pulmonary arterial hypertension (PAH) have resulted from changes in classification schemes and an increased emphasis on diagnosis because of the availability of effective therapies. The terms primary pulmonary hypertension and secondary pulmonary hypertension are considered inappropriate, confusing, and should not be used. Recent registries of patients with PAH have provided improved data regarding prognosis in the era of advanced therapies.
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Clinics in chest medicine · Dec 2013
ReviewOral therapies for pulmonary arterial hypertension: endothelin receptor antagonists and phosphodiesterase-5 inhibitors.
The development of orally active pulmonary vasodilators has been a major breakthrough in the treatment of pulmonary arterial hypertension (PAH). Orally active medications greatly enhanced patient access to PAH treatment and increased an interest in the diagnosis and treatment of this disease that still continues. Four different orally active drugs are currently available for the treatment of PAH and several more are undergoing evaluation. This article discusses the mechanisms by which endothelin receptor antagonists and phosphodiesterase-5 inhibitors mitigate pulmonary hypertensive responses, and reviews the most recent data concerning their efficacy and limitations in the treatment of PAH.
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Clinics in chest medicine · Dec 2013
ReviewNovel medical therapies for pulmonary arterial hypertension.
Available targeted therapies for pulmonary arterial hypertension are capable only of slowing progression of the disease and a cure remains elusive. However with the improved understanding of the pulmonary vascular remodeling that characterizes the disease, there is optimism that the disconnect between preclinical and clinical studies may be bridged with some of the newer therapies that are now at different stages of clinical evaluation. This article examines the evidence behind these new candidate treatments that may become part of the arsenal available for clinicians managing this devastating disease.
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Despite major advances in understanding the mechanisms of disease and development of specific drug therapy, pulmonary arterial hypertension (PAH) remains a progressive, fatal disease. At present there are 3 classes of drug therapy for PAH: prostaglandins, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors. ⋯ This review presents a detailed account of the published data on the use of combination therapy in PAH. There are few randomized, placebo-controlled trial data to strongly support efficacy of most combination therapy, particularly oral combination therapy.