Pulmonary arterial hypertension is a progressive disorder characterized by elevated pressure in the pulmonary arteries, leading to right heart strain, reduced exercise capacity, and eventually right heart failure if untreated. The condition can occur as an isolated disease or be associated with connective tissue disorders, congenital heart disease, chronic lung diseases, or exposure to certain drugs and toxins. Diagnosis of pulmonary arterial hypertension relies on a combination of echocardiography, right heart catheterization, imaging studies, and laboratory evaluation to confirm elevated pressures and rule out secondary causes. Management strategies are multifaceted, focusing on reducing pulmonary vascular resistance, improving functional status, and preventing disease progression. Pharmacologic therapies include vasodilators such as endothelin receptor antagonists, phosphodiesterase-5 inhibitors, prostacyclin analogs, and soluble guanylate cyclase stimulators, often used in combination for better outcomes. Non-pharmacological approaches, such as supervised exercise training, oxygen therapy, anticoagulation when indicated, and patient education, complement medical therapy. Pulmonary arterial hypertension care also emphasizes regular monitoring to assess treatment response and adjust therapy as needed. Multidisciplinary collaboration between pulmonologists, cardiologists, and specialized care teams is vital to ensure comprehensive management. Advances in research are exploring novel pathways and targeted therapies aimed at improving survival and quality of life. By combining timely diagnosis, evidence-based treatment, and supportive care, pulmonary arterial hypertension management helps reduce complications, enhance functional ability, and improve long-term outcomes for affected individuals.
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