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Am Fam Physician. 2024;110(2):183-191

This clinical content conforms to AAFP criteria for CME.

Author disclosure: No relevant financial relationships.

Pulmonary hypertension includes a diverse set of conditions defined by a mean pulmonary artery pressure greater than 20 mm Hg found during right heart catheterization that can lead to right-sided heart failure and death if untreated. The most common cause of pulmonary hypertension is left-sided heart failure, followed by chronic obstructive lung disease. Pulmonary hypertension presents as unexplained dyspnea on exertion and possible findings of right-sided heart failure. The diagnosis is commonly delayed because the symptoms are often attributed to underlying heart or lung disease. Echocardiography is the initial study of choice, and findings can suggest a low, intermediate, or high risk of pulmonary hypertension. Right heart catheterization is the standard of care for diagnosing and classifying pulmonary hypertension, and the results may inform treatment. Patients with pulmonary hypertension should be referred to a center specializing in treatment. Patients with pulmonary hypertension have a high risk of perioperative complications, and detailed specialty preoperative evaluation is recommended. Physicians should counsel patients of childbearing age with pulmonary hypertension to prevent pregnancy to avoid worsening the severity of pulmonary hypertension and fetal loss. Pulmonary hypertension is severe, chronic, progressive, and challenging to treat; therefore, family physicians should update the patient’s immunization status, screen for and address mental health conditions, and discuss goals of care and advance directives with patients.

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