Pulmonary Hypertension and Vascular Lung Diseases

Pulmonary hypertension (PH) and vascular lung diseases are serious conditions characterized by elevated blood pressure within the pulmonary arteries, leading to right heart strain, progressive dyspnea, and reduced exercise tolerance. These disorders may arise idiopathically, genetically, or secondary to underlying diseases such as left heart disease, chronic lung diseases, chronic thromboembolic events, or connective tissue disorders. Pulmonary vascular remodeling, endothelial dysfunction, and impaired vasodilation contribute to disease progression, making early recognition and intervention crucial to improving outcomes.

Diagnosis involves a combination of clinical assessment, echocardiography, right heart catheterization, pulmonary function tests, and imaging studies such as CT pulmonary angiography or ventilation-perfusion scans. Management strategies include targeted pharmacologic therapies, such as endothelin receptor antagonists, phosphodiesterase-5 inhibitors, prostacyclin analogs, and soluble guanylate cyclase stimulators, which aim to reduce pulmonary vascular resistance and improve cardiac output. Supportive measures, including oxygen therapy, diuretics, anticoagulation, and lifestyle modifications, complement medical therapy. In select cases, surgical interventions such as pulmonary thromboendarterectomy or lung transplantation may be indicated.

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