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A 2020 expert consensus survey has provided recommendations for treatment using oral prostacyclin analogs.


  • Dyspnea, fatigue, chest pain, and syncope on exertion.

  • Narrow splitting of second heart sound with loud pulmonary component; findings of right ventricular hypertrophy and heart failure in advanced disease.

  • Electrocardiographic evidence of right ventricular strain or hypertrophy and right atrial enlargement.

  • Enlarged central pulmonary arteries on chest radiograph.

  • Elevated right ventricular systolic pressure, right ventricular dilation or dysfunction on two-dimensional echocardiography with Doppler flow studies.


Pulmonary hypertension is a complex problem characterized by pathologic elevation in pulmonary arterial pressure. Normal pulmonary artery systolic pressure at rest is 15–30 mm Hg, with a mean pressure between 10 mm Hg and 18 mm Hg. The pulmonary circulation is a low-pressure, low-resistance system due to its large cross-sectional area, and it can accommodate significant increase in blood flow during exercise. The primary pathologic mechanism in pulmonary hypertension is an increase in pulmonary vascular resistance that leads to an increase in the pulmonary systolic pressure. Pulmonary hypertension is defined by a mean pulmonary arterial pressure of 20 mm Hg or more on a resting cardiac catheterization.

The WHO/New York Heart Association (NYHA) functional class currently classifies pulmonary hypertension based on similarities in pathologic mechanisms and includes the following five groups.

Group 1 (pulmonary arterial hypertension [PAH]): This group gathers diseases that localize directly to the pulmonary arteries leading to structural changes, smooth muscle hypertrophy, and endothelial dysfunction. This group includes idiopathic (formerly primary) PAH; heritable PAH; drug- and toxin-induced PAH; PAH associated with HIV infection, portal hypertension, connective tissue disorders (most commonly scleroderma), congenital heart disease, schistosomiasis; and PAH with features of veno-occlusive disease and pulmonary capillary hemangiomatosis. PAH is defined on a resting cardiac catheterization by a mean pulmonary arterial pressure of 20 mm Hg or more with a pulmonary capillary wedge pressure of 15 mm Hg or less and a pulmonary vascular resistance of 3 Wood units (WU) or more.

Group 2 (pulmonary venous hypertension due to left heart disease): This group includes left ventricular systolic or diastolic dysfunction and valvular heart disease.

Group 3 (pulmonary hypertension due to lung disease or hypoxemia): This group is caused by advanced obstructive and restrictive lung disease, including COPD, interstitial lung disease, pulmonary fibrosis as well as other causes of chronic hypoxemia, such as sleep-disordered breathing, alveolar hypoventilation syndromes, and high-altitude exposure.

Group 4 (pulmonary hypertension due to pulmonary obstruction): This group primarily includes chronic thromboembolic pulmonary hypertension but also includes other causes of pulmonary obstructions, such as sarcoma, metastatic malignancies, and congenital pulmonary artery stenosis.

Group 5 (pulmonary hypertension secondary to unclear or multifactorial mechanisms): These patients have pulmonary hypertension secondary to hematologic disorders (eg, chronic hemolytic anemia, sickle cell anemia, myeloproliferative disorders, splenectomy), systemic disorders (eg, ...

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