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Pulmonary hypertension 

Pulmonary hypertension

Pulmonary hypertension

Nicholas W. Morrell



Chapter reviewed – minor alterations made.

Updated on 29 Oct 2015. The previous version of this content can be found here.
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date: 28 April 2017

Symptoms of unexplained exertional breathlessness or symptoms out of proportion to coexistent heart or lung disease should alert the clinician to the possibility of pulmonary hypertension, and the condition should be actively sought in patients with known associated conditions, such as scleroderma, hypoxic lung disease, liver disease, or congenital heart disease. Heterozygous germ-line mutations in the gene encoding the bone morphogenetic protein type II receptor (BMPR2) are found in over 70% of families with pulmonary arterial hypertension (PAH).

Pulmonary hypertension is defined as a mean pulmonary arterial pressure greater than 25 mmHg at rest, or 30 mmHg on exercise, and may be due to increased pulmonary vascular resistance (e.g. PAH), increased transpulmonary blood flow (e.g. congenital heart disease), or increased pulmonary venous pressures (e.g. mitral stenosis). Exercise tolerance and survival in pulmonary hypertension is ultimately related to indices of right heart function, such as cardiac output.

Investigation—echocardiography is a good screening tool for the presence of pulmonary hypertension, but right heart catheterization is needed to confirm the diagnosis and guide treatment. CT pulmonary angiography and high-resolution CT are important to exclude underlying parenchymal lung disease and chronic thomboembolic pulmonary hypertension. In idiopathic PAH a vasodilator study should be undertaken at the time of right heart catheterization to detect the few (5–10%) patients who will have good long-term survival on calcium channel blockers.

Management—treatments for PAH include prostanoids, endothelin receptor antagonists, and phosphodiesterase inhibitors, which improve symptoms of breathlessness, exercise tolerance, quality of life, and probably survival. Chronic thromboembolic pulmonary hypertension (CTEPH) is an important diagnosis to make because selected patients with predominantly proximal disease can be cured by pulmonary endarterectomy.

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