Clinics in chest medicine
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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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Painstaking research led to the discovery of gene mutations responsible for heritable forms of pulmonary arterial hypertension (PAH). Mutations in the gene BMPR2, which codes for a cell surface receptor (BMPRII), cause the approximately 80% of heritable cases of PAH. ⋯ Other family members of many patients diagnosed with idiopathic PAH may be diagnosed with PAH. Genetic counseling and testing should be offered to patients diagnosed with heritable or idiopathic PAH.
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The relationship between pulmonary hypertension and left heart disease is complex. When initial assessment suggests the presence of pulmonary hypertension, it is critical to determine its precise nature: pulmonary arterial hypertension versus pulmonary hypertension owing to left heart disease. ⋯ Treatment requires optimal therapy of the underlying left heart disease. It is uncertain whether therapies for pulmonary arterial hypertension have any role in treating pulmonary hypertension owing to left heart disease, and there are safety concerns with these agents in this population.
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Clinics in chest medicine · Dec 2013
ReviewParenteral and inhaled prostanoid therapy in the treatment of pulmonary arterial hypertension.
Since continuous IV epoprostenol was approved in the U. S., parenteral prostanoid therapy has remained the gold standard for the treatment of patients with advanced pulmonary arterial hypertension (PAH). ⋯ The varied routes of administration allow for the incremental use of this class of agents in advanced PAH, and if PAH progresses. Prostanoids will remain a major component of PAH therapy for the foreseeable future.
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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.