Topics in Lung Disease: Pulmonary Hypertension

Pulmonary hypertension is a complex progressive disorder characterized by elevated pulmonary artery pressure. Diagnosis requires early clinical suspicion based on symptoms such as exertional dyspnea, fatigue, syncope, and chest discomfort, with echocardiography as a first-line diagnostic study. Diagnosis is confirmed using right heart catheterization, with a mean pulmonary artery pressure of 20 mm Hg or more and pulmonary vascular resistance greater than 2 Wood units. Strategies for primary and secondary prevention include regular physical activity, tobacco cessation, weight management, immunizations, and family planning to minimize risks associated with pregnancy. Prevention and evidence-based control of hypertension, diabetes, and dyslipidemia are essential. Occupational and environmental exposures, including poor indoor and outdoor air quality, should be addressed. Advances in pharmacotherapy for pulmonary arterial hypertension that target the endothelin, prostacyclin, and nitric oxide pathways and activin receptor inhibition have improved outcomes. Endothelin receptor antagonists, prostacyclin analogues, and phosphodiesterase 5 inhibitors are key treatment options. Combination therapy is recommended for patients with moderate to severe disease, whereas parenteral prostanoids are indicated for advanced pulmonary arterial hypertension. Right-sided heart failure, a major complication, is managed with fluid manage-ment and diuretics, with lung transplant evaluation and palliative shunt procedures considered for refractory cases. Addressing nutrition and iron deficiency are important supportive measures.