This review provides a clinical overview of the diagnosis and management of pulmonary arterial hypertension, highlighting the role of targeted therapies in improving survival and quality of life.
Supports targeted PAH therapies in practice; leaves open optimal sequencing and novel agent trials.
Pulmonary arterial hypertension (PAH) is a rare disease characterized by increased pulmonary vascular resistance due to endothelial dysfunction and cellular proliferation. A high index of suspicion and a thorough clinical evaluation are required to secure a diagnosis of PAH and stratify risk prior to initiation of specific therapies. Disease management includes education, moderate exercise, warfarin, oxygen, and calcium channel blockers. Targeted therapies with endothelin receptor antagonist, phosphodiesterase-5 inhibitors, and prostacyclins have improved the survival and quality of life of patients with PAH. Key Words—pulmonary arterial hypertension, PAH, pulmonary hypertension
No takes yet. Share an insight, caveat, or question.
Dan Schuller (2013) studied this question.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: