Overview
Pulmonary hypertension is a rare disease in which the blood vessels in the lungs become stiff and narrow. The pressure in these vessels goes up because the right side of the heart has to work harder to pump blood. Over time, this can lead to right heart failure. Because the lungs are responsible for adding oxygen to the pumped blood, in pulmonary hypertension, less oxygen can enter the blood. Without enough oxygen, symptoms like shortness of breath, tiredness, fainting, chest pain, or “brain fog” are likely. These symptoms are also common in other lung diseases like asthma or chronic obstructive pulmonary disease (COPD), so a diagnosis of pulmonary hypertension takes time. To officially diagnose pulmonary hypertension, a doctor will perform a heart procedure called a right heart catheterization (RHC). During a RHC, the pressures in the blood vessels in the lungs are measured. If those pressures are high, treatment should be started. Currently, no medication can cure pulmonary hypertension, but many options are available to control symptoms and improve quality of life.
Drug Therapies
-
Adcirca (tadalafil)
Adcirca (tadalafil) is an inhibitor of the enzyme phosphodiesterase type-5 approved to treat certain types of pulmonary hypertension. By relaxing muscles and the vasculature in the lungs, it increases blood flow and can help improve exercise tolerance.Manufacturer:United TherapeuticsRoute of Administration:OralSite of Care:OutpatientEnrollment Forms:Synergen Prescription FormApproved Indication:
treatment of pulmonary arterial hypertension (WHO Group 1) to improve exercise ability
Conditions:
- Pulmonary Arterial Hypertension
-
Adempas (riociguat)
Adempas (riociguat) is a soluble guanylate cyclose stimulator approved to treat adults with WHO group 1 pulmonary arterial hypertension or WHO group 4 chronic thromboembolic pulmonary hypertension (CTEPH). Adempas is the only medication FDA-approved for the treatment of CTEPH.Approved Indication:
- persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) (WHO group 4) after surgical treatment or inoperable CTEPH to improve exercise capacity and functional class
- pulmonary arterial hypertension (WHO group 1) to improve exercise capacity, improve functional class, and delay clinical worsening
Conditions:
- Chronic Thromboembolic Pulmonary Hypertension
- Pulmonary Arterial Hypertension
-
Alyq (tadalafil)
Alyq (tadalafil) is the authorized generic for Adcirca. Like Adcirca, Alyq is an inhibitor of the enzyme phosphodiesterase type-5 approved to treat certain types of pulmonary hypertension. By relaxing muscles and the vasculature in the lungs, it increases blood flow and can help improve exercise tolerance.Approved Indication:
treatment of pulmonary arterial hypertension (WHO Group 1) to improve exercise ability
Conditions:
- Pulmonary Arterial Hypertension
-
ambrisentan
Ambrisentan is an endothelin receptor antagonist approved to treat adults with WHO group 1 pulmonary arterial hypertension. By increasing pulmonary vasodilation, it can improve exercise tolerance and quality of life.Manufacturer:GileadRoute of Administration:OralSite of Care:OutpatientWebsite:Enrollment Forms:Synergen Prescription FormApproved Indication:
To improve exercise ability and delay clinical worsening in combination with tadalafil to reduce the risks of disease progression and hospitalization for worsening WHO group 1 pulmonary arterial hypertension
Conditions:
- Pulmonary Arterial Hypertension
-
bosentan
Ambrisentan is an endothelin receptor antagonist approved to treat adults and children 3 years and older with WHO group 1 pulmonary arterial hypertension. By increasing pulmonary vasodilation, it can improve exercise tolerance and quality of life.Manufacturer:MiscRoute of Administration:OralSite of Care:OutpatientWebsite:phone:1-866-359-2612Approved Indication:
- treatment of pulmonary arterial hypertension (WHO group 1) in adults to improve exercise ability and to decrease clinical worsening
- treatment of pulmonary arterial hypertension (WHO group 1) in pediatric patients aged 3 years and older with idiopathic or congenital PAH to improve pulmonary vascular resistance which is expected to result in an improvement in exercise ability
Conditions:
- Pulmonary Arterial Hypertension
-
Opsumit (macitentan)
Opsumit (macitentan) is an endothelin receptor antagonist approved to treat adults with WHO group 1 pulmonary arterial hypertension. By increasing pulmonary vasodilation, it can improve exercise tolerance and quality of life.Manufacturer:Johnson & JohnsonRoute of Administration:OralSite of Care:OutpatientWebsite:phone:866-228-3546Approved Indication:
treatment of pulmonary arterial hypertension (PAH, WHO group 1) in adults to reduce the risks of disease progression and hospitalization
Conditions:
- Pulmonary Arterial Hypertension
-
Opsynvi (macitentan/tadalafil)
Opsynvi (macitentan/tadalafil) is a one-pill combination of two pulmonary hypertension medications – a phosphodiesterase type 5 inhibitor and an endothelin receptor antagonist – given to relax pulmonary blood vessels and increase exercise tolerance.Manufacturer:Johnson & JohnsonRoute of Administration:OralSite of Care:OutpatientWebsite:phone:866-228-3546Enrollment Forms:Patient Support FormApproved Indication:
treatment of pulmonary arterial hypertension (PAH, WHO Group I) in adult patients of WHO functional class (FC) II-III
Conditions:
- Pulmonary Arterial Hypertension
-
Orenitram (treprostinil)
Orenitram (treprostinil) is the oral dosage form of the prostacyclin mimic treprostinil given to patients with certain forms of pulmonary hypertension to increase exercise tolerance.Manufacturer:United TherapeuticsRoute of Administration:OralSite of Care:OutpatientWebsite:phone:844-864-8437Approved Indication:
treatment of pulmonary arterial hypertension (PAH, WHO group 1) to delay disease progression and to improve exercise capacity
Conditions:
- Pulmonary Arterial Hypertension
-
Revatio (sildenafil)
Revatio (sildenafil) is an inhibitor of the enzyme phosphodiesterase type-5 approved to treat certain types of pulmonary hypertension. By relaxing muscles and the vasculature in the lungs, it increases blood flow and can help improve exercise tolerance.Manufacturer:MiscRoute of Administration:OralSite of Care:OutpatientWebsite:Enrollment Forms:Synergen Prescription FormApproved Indication:
- to improve exercise ability and delay clinical worsening adult patients with pulmonary arterial hypertension (PAH WHO group 1)
- to improve exercise ability and, in pediatric patients too young to perform standardized exercise testing, pulmonary hemodynamics thought to underlie improvements in exercise in pediatric patients 1 to 17 years old with pulmonary arterial hypertension (PAH WHO group 1)
Conditions:
- Pulmonary Arterial Hypertension
-
Tadliq (tadalafil)
Tadliq (tadalafil) is the oral suspension preparation of tadalafil. As an inhibitor of the enzyme phosphodiesterase type-5, Tadliq is approved to treat certain types of pulmonary hypertension. By relaxing muscles and the vasculature in the lungs, it increases blood flow and can help improve exercise tolerance.Manufacturer:CMP PharmaRoute of Administration:OralSite of Care:OutpatientWebsite:Approved Indication:
treatment of pulmonary arterial hypertension (WHO Group 1) to improve exercise ability
Conditions:
- Pulmonary Arterial Hypertension
-
Tyvaso (treprostinil inhalation solution & powder)
Tyvaso (treprostinil) is the inhaled dosage form of the prostacyclin mimic treprostinil given to patients with certain forms of pulmonary hypertension to increase exercise tolerance. Tyvaso can be inhaled through a nebulizer or a dry powder inhaler.Approved Indication:
- pulmonary arterial hypertension (WHO group 1) to improve exercise ability
- pulmonary hypertension associated with interstitial lung disease (PH-ILD, WHO group 3) to improve exercise ability
Conditions:
- Pulmonary Arterial Hypertension (PAH)
- Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD)
-
Uptravi (selexipag)
Uptravi (selexipag) is not a prostacyclin, but it activates prostacyclin receptor(s) to relax pulmonary blood vessels and help keep patients with pulmonary hypertension out of the hospital.Manufacturer:Johnson & JohnsonRoute of Administration:OralSite of Care:OutpatientWebsite:phone:866-228-3546Approved Indication:
treatment of pulmonary arterial hypertension (PAH, WHO group 1) to delay disease progression and reduce the risk of hospitalization for PAH
Conditions:
- Pulmonary Arterial Hypertension
-
Winrevair (sotatercept)
Winrevair (sotatercept) is an injectable activin signaling inhibitor approved to increase exercise capacity and reduce the risk of decompensation in patients with certain types of pulmonary hypertension. In clinical study, Winrevair was added to baseline therapies for pulmonary hypertension.Manufacturer:MerckRoute of Administration:SubcutaneousSite of Care:Outpatientphone:888-637-2502Approved Indication:
treatment of adults with pulmonary arterial hypertension (PAH, WHO group 1) to increase exercise capacity, improve WHO functional class, and reduce the risk of clinical worsening events
Conditions:
- Pulmonary Arterial Hypertension
-
Yutrepia (treprostinil)
Yutrepia (treprostinil) is an inhaled dosage form of the prostacyclin mimic treprostinil given to patients with certain forms of pulmonary hypertension to increase exercise tolerance. Yutrepia is inhaled through a capsule-based inhaler.Manufacturer:LiquidiaSite of Care:OutpatientWebsite:Approved Indication:
- pulmonary arterial hypertension (WHO group 1) to improve exercise ability
- pulmonary hypertension associated with interstitial lung disease (PH-ILD, WHO group 3) to improve exercise ability
Conditions:
- Pulmonary Arterial Hypertension (PAH)
- Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD)
Resources
Understanding a diagnosis can feel overwhelming. These resources link to well-established organizations, for in-depth education, research, and support.
Stay Connected
Get the latest news and updates