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

  • Narrow splitting of second heart sound with loud pulmonary component; findings of RVH and HF in advanced disease.

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

  • Enlarged central pulmonary arteries on CXR.

  • 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 less than 20 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 World Symposium on Pulmonary Hypertension (WSPH) updated clinical classification includes five groups that are based on etiology and mechanism.

Group 1 (pulmonary arterial hypertension [PAH]): This group comprises diseases to the pulmonary arteries that lead to structural changes, smooth muscle hypertrophy, and endothelial dysfunction. It 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, and 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 or more.

Group 2 (pulmonary venous hypertension due to left heart disease): This group includes LV 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 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, sarcoidosis, vasculitis, pulmonary Langerhans cell histiocytosis, neurofibromatosis type 1), metabolic disorders (eg, glycogen storage disease, Gaucher disease, thyroid disease), and miscellaneous causes (eg, ESKD with or without hemodialysis, fibrosing mediastinitis).


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