Abstract
Pulmonary arterial hypertension (PAH) covers a range of life-limiting illnesses that are characterized by increased mean pulmonary artery pressures that, if untreated, lead to right heart failure and death. This is due to remodeling of the small-to-medium sized pulmonary vessels, which obstruct blood flow. PAH can be further categorized into idiopathic PAH (without any identifiable cause, 6th World Symposium class 1.11) and heritable PAH (defined by mutations in specific genes, 6th World Symposium class 1.21), the most common affecting bone morphogenetic protein receptor type II (BMPR2).
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