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Pulmonary hypertension 

Pulmonary hypertension
Pulmonary hypertension

James Tonkin

, Kate Ryan

, and Brendan Madden


July 29, 2020: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.

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date: 28 November 2021

Pulmonary hypertension is characterized by a mean pulmonary artery pressure greater than 25mmHg at rest. Pulmonary hypertension is divided into groups 1 to 5, according to the underlying cause. Group 1 diseases are characterized by plexogenic pulmonary arteriopathy, which occurs through nitric oxide, endothelin, and prostacyclin pathways. Investigations for diagnosis include echocardiography, computed tomography pulmonary angiography, and right heart catheterization. Treatment options for group 1 pulmonary hypertension include phosphodiesterase-5 inhibitors, endothelin receptor antagonists, prostacyclin analogues, and novel therapies such as selexipag and riociguat. However, chronic thromboembolic pulmonary hypertension is often treated surgically with pulmonary thromboendarterectomy.

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