Clinics in chest medicine
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The pathology of pulmonary hypertension includes numerous abnormalities of the intima, media, and adventitia of the pulmonary vascular tree. A recently completed systematic analysis revealed high variability of morphologic measurements within a given pulmonary arterial hypertension (PAH) lung and among all PAH lungs, as well as distinct pathologic subphenotypes, and included a subset of lungs lacking intimal and medial remodeling. There was correlation between perivascular inflammation, remodeling, and hemodynamics. This article summarizes the pathologic features of the normal and abnormal pulmonary circulation, and correlations with animal models.
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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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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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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.