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Pulmonary Hypertension (PH): Treatments

Pulmonary hypertension, or PH, is increased pressure in the pulmonary arteries. These arteries carry blood from your heart to your lungs to pick up oxygen.

PubMed Health Glossary
(Source: NIH - National Heart, Lung, and Blood Institute)

Treatments for Pulmonary Hypertension (PH)

Pulmonary hypertension (PH) has no cure. However, treatment may help relieve symptoms and slow the progress of the disease.

PH is treated with medicines, procedures, and other therapies. Treatment will depend on what type of PH you have and its severity. (For more information, go to "Types of Pulmonary Hypertension.")

Group 1 Pulmonary Arterial Hypertension

Group 1 pulmonary arterial hypertension (PAH) includes PH that's inherited, that has no known cause, or that's caused by certain drugs or conditions. Treatments for group 1 PAH include medicines and medical procedures.

Medicines

Your doctor may prescribe medicines to relax the blood vessels in your lungs and reduce excess cell growth in the blood vessels. As the blood vessels relax, more blood can flow through them.

Your doctor may prescribe medicines that are taken by mouth, inhaled, or injected....Read more about Pulmonary Hypertension: Treatments
NIH - National Heart, Lung, and Blood Institute

What works? Research summarized

Evidence reviews

Prostacyclin for pulmonary hypertension in adults

Prostacyclin may benefit patients with pulmonary hypertension (raised blood pressure in the lungs) in the short term but studies longer in duration are required. Pulmonary hypertension occurs when blood is pumped through arteries in the lungs at an increased pressure. The condition can lead to heart failure and death. Once the diagnosis is made, life expectancy ranges from a few months to a few years. Most current treatments apart from lung transplantation do not improve survival. Over an 8‐12 week period prostacyclin improved exercise capacity and some measures of blood flow when given intravenously or via injection to patients with pulmonary hypertension. However, with intravenous administration there can be serious side effects as the drug has to be given continuously via a pump into a catheter placed into a central vein. It is not clear how long the drug continues to confer benefit without serious side effects. Prostacyclin can also be given by mouth, under the skin or through an inhaler. These forms of administration may be safer than intravenous prostacyclin and there is evidence that these may be effective in the short term.

Drug Therapy for Chronic Thromboembolic Pulmonary Hypertension: A Review of the Comparative Clinical Effectiveness [Internet]

Riociguat, a soluble guanylate cyclase stimulator (sGC), is currently the only drug with Health Canada approved indication for the management of inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or persistent or recurrent CTEPH after surgical treatment in adults 18 years or older with progressive pulmonary hypertension (PH) of World Health Organization (WHO) functional class II or III. The WHO functional class ranges from I to IV, with higher numbers indicating greater functional limitations. Since histopathology studies suggest that CTEPH and primary pulmonary artery hypertension (PAH) share common pathways in their pathophysiology and manifest similar small-vessel changes, vasoactive therapies with evidence of efficacy in PAH are commonly prescribed off-label for CTEPH patients who require medical intervention. In Canada, specific therapies for PAH include sGC stimulators, endothelin receptor antagonists (ERA), phosphodiesterase-5 inhibitors (PDE-5), and prostacyclin analogues. The objective of this report is to review current evidence of comparative efficacy and safety of monotherapy or combination therapy of medical interventions for patients with CTEPH.

Riociguat (Adempas): Management of Inoperable Chronic Thromboembolic Pulmonary Hypertension [Internet]

Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease subtype of pulmonary hypertension. It is a progressive disease characterized by the presence of non-resolving or recurrent thrombi distributed within the pulmonary arteries. This can obstruct or occlude the luminal space, eventually leading to increased pulmonary vascular resistance (PVR), pulmonary hypertension, and right-sided heart failure. Although the exact etiology of CTEPH remains poorly understood, it may arise following an initial episode of acute pulmonary embolism (PE); however, up to 60% of CTEPH patients have not had any antecedent episode of acute PE. The epidemiology of CTEPH is likewise not well established. Some surveillance data estimate CTEPH to occur in 0.1% to 0.5% of patients surviving an initial episode of acute PE; however, the true incidence of CTEPH is likely to be higher, owing to an unknown number of undetected cases either presenting occultly or latently in the setting of acute PE or through non-venous thromboembolism etiologies.

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Summaries for consumers

Prostacyclin for pulmonary hypertension in adults

Prostacyclin may benefit patients with pulmonary hypertension (raised blood pressure in the lungs) in the short term but studies longer in duration are required. Pulmonary hypertension occurs when blood is pumped through arteries in the lungs at an increased pressure. The condition can lead to heart failure and death. Once the diagnosis is made, life expectancy ranges from a few months to a few years. Most current treatments apart from lung transplantation do not improve survival. Over an 8‐12 week period prostacyclin improved exercise capacity and some measures of blood flow when given intravenously or via injection to patients with pulmonary hypertension. However, with intravenous administration there can be serious side effects as the drug has to be given continuously via a pump into a catheter placed into a central vein. It is not clear how long the drug continues to confer benefit without serious side effects. Prostacyclin can also be given by mouth, under the skin or through an inhaler. These forms of administration may be safer than intravenous prostacyclin and there is evidence that these may be effective in the short term.

Terms to know

Arteries
A blood vessel that carries blood from the heart to tissues and organs in the body.
Heart
The hollow, muscular organ that maintains the circulation of the blood.
Lungs
One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.
Oxygen
A colorless, odorless gas. It is needed for animal and plant life. Oxygen that is breathed in enters the blood from the lungs and travels to the tissues.
Pulmonary
Having to do with the lungs.
Pulmonary Artery
The pulmonary artery and its branches deliver blood rich in carbon dioxide (and lacking in oxygen) to the capillaries that surround the air sacs.
Pulmonary Circulation
The circulation of the blood through the lungs.

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Other terms to know: See all 7
Arteries, Heart, Lungs

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