Table of ContentsView AllTable of ContentsUnderstanding PAHTreatment OptionsCombination Therapies for PAHFrequently Asked Questions
Table of ContentsView All
View All
Table of Contents
Understanding PAH
Treatment Options
Combination Therapies for PAH
Frequently Asked Questions
Pulmonary hypertension (PH)can be challenging for healthcare professionals to diagnose because of its vague symptoms, such asfatigueandshortness of breath.
These symptoms, which may be mild initially, can sometimes lead to a misdiagnosis, such asasthmaorheart failure.
In fact, a recent study suggests that board-certified physicians specializing in heart and lung conditions may correctly suspect PH less than 50% of the time.
Because PAH can be severe and often challenging to treat, it can lead to severe short- and long-term health challenges.
Read on to learn more about the medications available to treat PAH, including the drug classes and specific drugs that make up these categories.
Dobrila Vignjevic / Getty Images
PAH: Prevalence, Symptoms, and Health Risks
PH is defined as high lung blood pressure with various underlying causes.
In comparison, PAH is a rare and gradually worsening condition in which the blood pressure in the pulmonary arteries becomes too high.
These arteries carry blood from the right side of the heart through the lungs. When this pressure gets too high, the arteries in the lungs become more narrow. This limits blood flow, thus resulting in less oxygen in the blood.
If left untreated, PAH makes the right side of the heart work much harder, which can eventually weaken or damage the heart, leading toright heart failureand even death.
Right heart failure occurs when the heart is too weak to pump adequate blood supply to the lungs.
At-Risk Population(s)
PAH is rare, affecting 1 to 2 out of every 1 million people in the United States.
PAH most commonly affects females between ages 30 and 60, occurring three out of five times more frequently in females than males.
Roughly 1,000 new cases are diagnosed in the United States yearly, with a five-year survival rate of 61%.
Symptoms
The most common symptoms of PAH are shortness of breath andchest pain, especially when walking or engaging in other physical activity.
Other symptoms of PAH include:
Causes
There’s no cure for this condition, but many treatments are available to manage the symptoms and help maintain quality of life.
Pharmaceutical Treatment Options
The number oftreatment optionsfor PAH has grown over the past few decades.
The main classes of drugs prescribed to manage this condition include:
The following subsections will discuss different categories of PAH medications and the specific drugs that make up these categories.
Prostacyclin Analogs
Their mechanism of action includes stoppingplateletsfrom forming clots in your blood vessels and causing vasodilation of the pulmonary arteries.
When these blood vessels relax and widen, blood flows more efficiently, reducing the pressure.
Prostacyclin analogs are a standard long-term treatment for severe PAH.
Flolan (epoprostenol) was the first drug in this class. It is used in severe cases if other treatments aren’t working well enough.
Prostacyclin analogs come in several forms. They may be given as intravenous (IV, within a vein) infusions, subcutaneous (under the skin) injections, or inhalation. An oral tablet option is also available as Orenitram (treprostinil).
Potentially serious side effects includehypotension (low blood pressure)and an increased risk of bleeding. It’s important not to stop or reduce the dosage of prostacyclin analogs abruptly. Doing so may cause sudden worsening of PAH symptoms.
A similar and newer drug, Uptravi (selexipag), has a comparable mechanism of action as a prostacyclin receptor agonist.It also mimics the effects of the natural hormone prostacyclin.
Endothelin Receptor Antagonists (ERAs)
ERAs block endothelin-related effects on the smooth muscle cells lining your blood vessels.
This causes your blood vessels to relax and widen, which lowers pressure, reduces strain on the heart, eases PAH symptoms, and improves tolerance of physical activity.
ERAs are prescribed to help slow the progression of PAH.
ERAs carry serious risks, including aboxed warning, the most severe type of warning from the FDA, due to the risk of congenital disabilities.
Due to this risk, females withreproductive potentialare required to enroll in a particular program, take regular pregnancy tests, and useacceptable forms of birth control.
ERAs also pose risks of severeliver injuryor liver failure, such as in the case of Opsumit (macitentan), Tracleer (bosentan), and Letairis (ambrisentan).
The table below summarizes critical details about three ERAs:
Phosphodiesterase-5 (PDE-5) Inhibitors
Phosphodiesterase-5 (PDE-5) inhibitors enhance the effects of nitric oxide in the body.
In other words, PDE-5 inhibitors cause blood vessels to open wider, improving blood flow and decreasing pressure. These medications are also used forerectile dysfunction.
Your body produces nitric oxide to relax blood vessels. Nitric oxide attaches to receptors on blood vessel walls, triggering the production of cyclic GMP (cGMP). cGMP signals your blood vessel muscles to relax, widening the vessels.
PDE-5 inhibitors should not be taken withnitratesor riociguat due to the risk of dangerously low blood pressure. PDE-5 inhibitors may also causesevere side effectssuch as vision changes andhearing loss.
The table below highlights some of the crucial details about a few drugs in this class that are approved for treating PAH:
Soluble Guanylate Cyclase (sGC) Stimulators
SGC is a substance produced in the body that causes the blood vessels in the lungs to dilate (become wider). This process is similar to the PDE5 inhibitors, so these two medicine classes aren’t taken together.
Adempas (riociguat) is the only drug in its class of soluble guanylate cyclase (sGC) stimulators.
The drug is FDA-approved to treat PAH, more specifically, to improve tolerance for activity, reduce symptom severity, and delay clinical worsening.
Note that sGC stimulators carry a boxed warning due to toxic effects on a developing fetus and are therefore unsafe to take during pregnancy.
Like PDE-5 inhibitors, riociguat stimulates increased cGMP production, leading to relaxation and widening blood vessels, thus lowering pressure.
Additionally, riociguat also offers protection againstfibrosis (scar tissue)in the lungs.
Irritation in the lungs can lead to fibrosis and inflammation. However, riociguat can combat this and aid in diminishingpulmonary fibrosis.
The table below summarizes other vital details about Adempas:
Calcium Channel Blockers (CCBs)
CCBs are a group of medications that treat conditions affecting the heart and blood vessels, including hypertension.
Other PAH CCBs include Procardia XL (nifedipine) and Cardizem CD (diltiazem).
Common side effects of CCBs include dizziness, constipation, flushing, headache, and swelling in the ankles or feet. Some individuals may also experience heart palpitations.
However, these drugs aren’t specifically FDA-approved for this purpose.
Unfortunately, CCBs only seem helpful for a small percentage of people with PAH.
Activin Signaling Inhibitors
Winrevair (sotatercept) is the first activin signaling inhibitor therapy approved to treat PAH. By regulating PAH cell growth, Winrevair increases exercise capacity and decreases the risk of PAH worsening in adults.
In some cases, healthcare providers might recommend combining more than one type of medication for PAH.
The goal of combination therapy is to target multiple pathways involved in PAH at the same time. Typically, combination therapy is prescribed for people with severe PAH.
Combination therapies for PAH often include:
The choice between these two combination regimens depends on the patient’s needs.
Your healthcare team will determine the proper treatment approach based on your PAH severity and how well you’ve tolerated previous treatments.
Summary
Various treatment options are available for pulmonary arterial hypertension (PAH).
Medications like prostacyclin analogs, endothelin receptor antagonists, and activin signaling inhibitors help relax blood vessels in the lungs, making it easier for the heart to pump blood and improve your tolerance of physical activity.
PDE-5 inhibitors, soluble guanylate cyclase (sGC) stimulators, and calcium channel blockers are commonly used to manage symptoms.
Oxygen therapycan also be used to increase oxygen levels in the blood. In some cases, surgeries might be an option.
Treatment choices depend on the severity of your PAH (based on the WHO classification system). A healthcare provider can discuss the best treatment plan for you.
Regular checkups and monitoring are vital for people living with PAH to ensure optimal management and a better quality of life.
Research is ongoing to develop new treatments for PAH. A new drug called elafin is being investigated for treating PAH and seems to prevent inflammation and blood vessel damage.
Likewise, the Pediatric Pulmonary Hypertension Network continues investigating PAH treatment options for children.
Frequently Asked QuestionsWhen you breathe, your lungs take in oxygen, which gets carried throughout your body in your blood. However, trouble breathing is a common symptom of PAH.Oxygen therapy boosts the amount of oxygen in your blood, making it easier to breathe, and helps your body deliver oxygen-rich blood throughout your tissues and organs.PAH increases the risk oflung blood clots, which can be life-threatening.Anticoagulants, such asJantoven (warfarin), can reduce this risk in people with PAH.Also known as blood thinners, these drugs help preventblood clotsfrom forming in the blood vessels.Besides medications, some people may have surgeries to manage PAH. Receiving alung transplantis an option for severe PAH.In addition, supportive lifestyle habits include following ahealthy diet, watching yoursalt intake, and remaining asphysically activeas possible.
When you breathe, your lungs take in oxygen, which gets carried throughout your body in your blood. However, trouble breathing is a common symptom of PAH.Oxygen therapy boosts the amount of oxygen in your blood, making it easier to breathe, and helps your body deliver oxygen-rich blood throughout your tissues and organs.
When you breathe, your lungs take in oxygen, which gets carried throughout your body in your blood. However, trouble breathing is a common symptom of PAH.
Oxygen therapy boosts the amount of oxygen in your blood, making it easier to breathe, and helps your body deliver oxygen-rich blood throughout your tissues and organs.
PAH increases the risk oflung blood clots, which can be life-threatening.Anticoagulants, such asJantoven (warfarin), can reduce this risk in people with PAH.Also known as blood thinners, these drugs help preventblood clotsfrom forming in the blood vessels.
PAH increases the risk oflung blood clots, which can be life-threatening.Anticoagulants, such asJantoven (warfarin), can reduce this risk in people with PAH.
Also known as blood thinners, these drugs help preventblood clotsfrom forming in the blood vessels.
Besides medications, some people may have surgeries to manage PAH. Receiving alung transplantis an option for severe PAH.In addition, supportive lifestyle habits include following ahealthy diet, watching yoursalt intake, and remaining asphysically activeas possible.
Besides medications, some people may have surgeries to manage PAH. Receiving alung transplantis an option for severe PAH.
In addition, supportive lifestyle habits include following ahealthy diet, watching yoursalt intake, and remaining asphysically activeas possible.
38 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.National Institutes of Health.What is pulmonary hypertension?.Taichman DB, Ornelas J, Chung L, Klinger JR, Lewis S, Mandel J, Palevsky HI, Rich S, Sood N, Rosenzweig EB, Trow TK, Yung R, Elliott CG, Badesch DB.Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report.Chest. 2014;146(2):449-475. doi:10.1378/chest.14-0793Guillevin L, Armstrong I, Aldrighetti R, et al.Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives.Eur Respir Rev. 2013;22(130):535-542. doi:10.1183/09059180.00005713George MG, Schieb L, Ayala C, Talwalkar A, Levant S.Pulmonary hypertension surveillance: United States, 2001 to 2010.Chest. 2014;146(2):476–95. doi:10.1378/chest.14-0527National Organization for Rare Disorders.Pulmonary arterial hypertension.American Lung Association.Pulmonary arterial hypertension symptoms and diagnosis.Barnes H, Yeoh HL, Fothergill T, Burns A, Humbert M, Williams T.Prostacyclin for pulmonary arterial hypertension.Cochrane Database Syst Rev.2019;5(5):CD012785. doi: 10.1002/14651858.CD012785.pub2Ruan CH, Dixon RA, Willerson JT, Ruan KH.Prostacyclin therapy for pulmonary arterial hypertension.Tex Heart Inst J.2010;37(4):391-399.DailyMed.Label: Flolan- epoprostenol sodium injection, powder, lyophilized, for solution diluent- water solution.Kingman M, Archer-Chicko C, Bartlett M, Beckmann J, Hohsfield R, Lombardi S.Management of prostacyclin side effects in adult patients with pulmonary arterial hypertension.Pulm Circ. 2017;7(3):598-608. doi:10.1177/2045893217719250DailyMed.Label: Orenitram- treprostinil tablet, extended-release Orenitram- treprostinil kit.DailyMed.Label: Uptravi- selexipag tablet, coated Uptravi titration pack- selexipag kit Uptravi- selexipag injection, powder, for solution.DailyMed.Label: Ventavis- iloprost solution.DailyMed.Label: Remodulin- treprostinil injection, solution sterile diluent for Remodulin- water injection, solution.LiverTox.Clinical and research information on drug-induced liver injury: endothelin receptor antagonists.Liu C, Chen J, Gao Y, Deng B, Liu K.Endothelin receptor antagonists for pulmonary arterial hypertension.Cochrane Database Syst Rev. 2013;2013(2):CD004434. doi:10.1002/14651858.CD004434.pub5DailyMed.Label: Opsumit- macitentan tablet, film coated.DailyMed.Label: Tracleer- bosentan tablet, film coated Tracleer- bosentan tablet, for suspension.DailyMed.Label: Letairis- ambrisentan tablet, film coated.Huang SA, Lie JD.Phosphodiesterase-5 (PDE5) inhibitors in the management of erectile dysfunction.P T. 2013;38(7):407-19.Unegbu C, Noje C, Coulson JD, Segal JB, Romer L.Pulmonary hypertension therapy and a systematic review of efficacy and safety of PDE-5 inhibitors.Pediatrics. 2017;139(3):e20161450. doi:10.1542/peds.2016-1450DailyMed.Label: Revatio- sildenafil citrate tablet, film coated Revatio- sildenafil citrate injection, solution Revatio- sildenafil citrate powder, for suspension.DailyMed.Label: Adcirca- tadalafil tablet.DailyMed.Label: Tadliq- tadalafil suspension.Watanabe H.Treatment selection in pulmonary arterial hypertension: phosphodiesterase type 5 inhibitors versus soluble guanylate cyclase stimulator.EurCardiol. 2018;13(1):35-37. doi:10.15420/ecr.2017:22:2DailyMed.Label: Adempas- riociguat tablet, film coated.Khaybullina D, Patel A, Zerilli T.Riociguat (adempas): a novel agent for the treatment of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.P T. 2014;39(11):749-758.British Heart Foundation.Drug cabinet: calcium channel blockers.DailyMed.Label: Norvasc- amlodipine besylate tablet.DailyMed.Label: Procardia XL- nifedipine tablet, film coated, extended-release.DailyMed.Label: Cardizem CD- diltiazem hydrochloride capsule, coated, extended release.Medarov BI, Judson MA.The role of calcium channel blockers for the treatment of pulmonary arterial hypertension: how much do we actually know and how could they be positioned today?.Respir Med. 2015;109(5):557-564. doi:10.1016/j.rmed.2015.01.004Food and Drug Administration.Opsynvi label.Sommer N, Ghofrani HA, Pak O, et al.Current and future treatments of pulmonary arterial hypertension.Br J Pharmacol. 2021;178(1):6-30. doi:10.1111/bph.15016Farber HW, Badesch DB, Benza RL, et al.Use of supplemental oxygen in patients with pulmonary arterial hypertension in REVEAL.J Heart Lung Transplant. 2018;37(8):948-955. doi:10.1016/j.healun.2018.03.010National Heart, Lung, and Blood Institute.Pulmonary hypertension research.Said K.Anticoagulation in pulmonary arterial hypertension: contemporary data from COMPERA registry.Glob Cardiol Sci Pract.2014;2014(2):48-52. doi:10.5339/gcsp.2014.25George MP, Champion HC, Pilewski JM.Lung transplantation for pulmonary hypertension.Pulm Circ. 2011;1(2):182-91. doi:10.4103/2045-8932.83455
38 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.National Institutes of Health.What is pulmonary hypertension?.Taichman DB, Ornelas J, Chung L, Klinger JR, Lewis S, Mandel J, Palevsky HI, Rich S, Sood N, Rosenzweig EB, Trow TK, Yung R, Elliott CG, Badesch DB.Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report.Chest. 2014;146(2):449-475. doi:10.1378/chest.14-0793Guillevin L, Armstrong I, Aldrighetti R, et al.Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives.Eur Respir Rev. 2013;22(130):535-542. doi:10.1183/09059180.00005713George MG, Schieb L, Ayala C, Talwalkar A, Levant S.Pulmonary hypertension surveillance: United States, 2001 to 2010.Chest. 2014;146(2):476–95. doi:10.1378/chest.14-0527National Organization for Rare Disorders.Pulmonary arterial hypertension.American Lung Association.Pulmonary arterial hypertension symptoms and diagnosis.Barnes H, Yeoh HL, Fothergill T, Burns A, Humbert M, Williams T.Prostacyclin for pulmonary arterial hypertension.Cochrane Database Syst Rev.2019;5(5):CD012785. doi: 10.1002/14651858.CD012785.pub2Ruan CH, Dixon RA, Willerson JT, Ruan KH.Prostacyclin therapy for pulmonary arterial hypertension.Tex Heart Inst J.2010;37(4):391-399.DailyMed.Label: Flolan- epoprostenol sodium injection, powder, lyophilized, for solution diluent- water solution.Kingman M, Archer-Chicko C, Bartlett M, Beckmann J, Hohsfield R, Lombardi S.Management of prostacyclin side effects in adult patients with pulmonary arterial hypertension.Pulm Circ. 2017;7(3):598-608. doi:10.1177/2045893217719250DailyMed.Label: Orenitram- treprostinil tablet, extended-release Orenitram- treprostinil kit.DailyMed.Label: Uptravi- selexipag tablet, coated Uptravi titration pack- selexipag kit Uptravi- selexipag injection, powder, for solution.DailyMed.Label: Ventavis- iloprost solution.DailyMed.Label: Remodulin- treprostinil injection, solution sterile diluent for Remodulin- water injection, solution.LiverTox.Clinical and research information on drug-induced liver injury: endothelin receptor antagonists.Liu C, Chen J, Gao Y, Deng B, Liu K.Endothelin receptor antagonists for pulmonary arterial hypertension.Cochrane Database Syst Rev. 2013;2013(2):CD004434. doi:10.1002/14651858.CD004434.pub5DailyMed.Label: Opsumit- macitentan tablet, film coated.DailyMed.Label: Tracleer- bosentan tablet, film coated Tracleer- bosentan tablet, for suspension.DailyMed.Label: Letairis- ambrisentan tablet, film coated.Huang SA, Lie JD.Phosphodiesterase-5 (PDE5) inhibitors in the management of erectile dysfunction.P T. 2013;38(7):407-19.Unegbu C, Noje C, Coulson JD, Segal JB, Romer L.Pulmonary hypertension therapy and a systematic review of efficacy and safety of PDE-5 inhibitors.Pediatrics. 2017;139(3):e20161450. doi:10.1542/peds.2016-1450DailyMed.Label: Revatio- sildenafil citrate tablet, film coated Revatio- sildenafil citrate injection, solution Revatio- sildenafil citrate powder, for suspension.DailyMed.Label: Adcirca- tadalafil tablet.DailyMed.Label: Tadliq- tadalafil suspension.Watanabe H.Treatment selection in pulmonary arterial hypertension: phosphodiesterase type 5 inhibitors versus soluble guanylate cyclase stimulator.EurCardiol. 2018;13(1):35-37. doi:10.15420/ecr.2017:22:2DailyMed.Label: Adempas- riociguat tablet, film coated.Khaybullina D, Patel A, Zerilli T.Riociguat (adempas): a novel agent for the treatment of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.P T. 2014;39(11):749-758.British Heart Foundation.Drug cabinet: calcium channel blockers.DailyMed.Label: Norvasc- amlodipine besylate tablet.DailyMed.Label: Procardia XL- nifedipine tablet, film coated, extended-release.DailyMed.Label: Cardizem CD- diltiazem hydrochloride capsule, coated, extended release.Medarov BI, Judson MA.The role of calcium channel blockers for the treatment of pulmonary arterial hypertension: how much do we actually know and how could they be positioned today?.Respir Med. 2015;109(5):557-564. doi:10.1016/j.rmed.2015.01.004Food and Drug Administration.Opsynvi label.Sommer N, Ghofrani HA, Pak O, et al.Current and future treatments of pulmonary arterial hypertension.Br J Pharmacol. 2021;178(1):6-30. doi:10.1111/bph.15016Farber HW, Badesch DB, Benza RL, et al.Use of supplemental oxygen in patients with pulmonary arterial hypertension in REVEAL.J Heart Lung Transplant. 2018;37(8):948-955. doi:10.1016/j.healun.2018.03.010National Heart, Lung, and Blood Institute.Pulmonary hypertension research.Said K.Anticoagulation in pulmonary arterial hypertension: contemporary data from COMPERA registry.Glob Cardiol Sci Pract.2014;2014(2):48-52. doi:10.5339/gcsp.2014.25George MP, Champion HC, Pilewski JM.Lung transplantation for pulmonary hypertension.Pulm Circ. 2011;1(2):182-91. doi:10.4103/2045-8932.83455
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
National Institutes of Health.What is pulmonary hypertension?.Taichman DB, Ornelas J, Chung L, Klinger JR, Lewis S, Mandel J, Palevsky HI, Rich S, Sood N, Rosenzweig EB, Trow TK, Yung R, Elliott CG, Badesch DB.Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report.Chest. 2014;146(2):449-475. doi:10.1378/chest.14-0793Guillevin L, Armstrong I, Aldrighetti R, et al.Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives.Eur Respir Rev. 2013;22(130):535-542. doi:10.1183/09059180.00005713George MG, Schieb L, Ayala C, Talwalkar A, Levant S.Pulmonary hypertension surveillance: United States, 2001 to 2010.Chest. 2014;146(2):476–95. doi:10.1378/chest.14-0527National Organization for Rare Disorders.Pulmonary arterial hypertension.American Lung Association.Pulmonary arterial hypertension symptoms and diagnosis.Barnes H, Yeoh HL, Fothergill T, Burns A, Humbert M, Williams T.Prostacyclin for pulmonary arterial hypertension.Cochrane Database Syst Rev.2019;5(5):CD012785. doi: 10.1002/14651858.CD012785.pub2Ruan CH, Dixon RA, Willerson JT, Ruan KH.Prostacyclin therapy for pulmonary arterial hypertension.Tex Heart Inst J.2010;37(4):391-399.DailyMed.Label: Flolan- epoprostenol sodium injection, powder, lyophilized, for solution diluent- water solution.Kingman M, Archer-Chicko C, Bartlett M, Beckmann J, Hohsfield R, Lombardi S.Management of prostacyclin side effects in adult patients with pulmonary arterial hypertension.Pulm Circ. 2017;7(3):598-608. doi:10.1177/2045893217719250DailyMed.Label: Orenitram- treprostinil tablet, extended-release Orenitram- treprostinil kit.DailyMed.Label: Uptravi- selexipag tablet, coated Uptravi titration pack- selexipag kit Uptravi- selexipag injection, powder, for solution.DailyMed.Label: Ventavis- iloprost solution.DailyMed.Label: Remodulin- treprostinil injection, solution sterile diluent for Remodulin- water injection, solution.LiverTox.Clinical and research information on drug-induced liver injury: endothelin receptor antagonists.Liu C, Chen J, Gao Y, Deng B, Liu K.Endothelin receptor antagonists for pulmonary arterial hypertension.Cochrane Database Syst Rev. 2013;2013(2):CD004434. doi:10.1002/14651858.CD004434.pub5DailyMed.Label: Opsumit- macitentan tablet, film coated.DailyMed.Label: Tracleer- bosentan tablet, film coated Tracleer- bosentan tablet, for suspension.DailyMed.Label: Letairis- ambrisentan tablet, film coated.Huang SA, Lie JD.Phosphodiesterase-5 (PDE5) inhibitors in the management of erectile dysfunction.P T. 2013;38(7):407-19.Unegbu C, Noje C, Coulson JD, Segal JB, Romer L.Pulmonary hypertension therapy and a systematic review of efficacy and safety of PDE-5 inhibitors.Pediatrics. 2017;139(3):e20161450. doi:10.1542/peds.2016-1450DailyMed.Label: Revatio- sildenafil citrate tablet, film coated Revatio- sildenafil citrate injection, solution Revatio- sildenafil citrate powder, for suspension.DailyMed.Label: Adcirca- tadalafil tablet.DailyMed.Label: Tadliq- tadalafil suspension.Watanabe H.Treatment selection in pulmonary arterial hypertension: phosphodiesterase type 5 inhibitors versus soluble guanylate cyclase stimulator.EurCardiol. 2018;13(1):35-37. doi:10.15420/ecr.2017:22:2DailyMed.Label: Adempas- riociguat tablet, film coated.Khaybullina D, Patel A, Zerilli T.Riociguat (adempas): a novel agent for the treatment of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.P T. 2014;39(11):749-758.British Heart Foundation.Drug cabinet: calcium channel blockers.DailyMed.Label: Norvasc- amlodipine besylate tablet.DailyMed.Label: Procardia XL- nifedipine tablet, film coated, extended-release.DailyMed.Label: Cardizem CD- diltiazem hydrochloride capsule, coated, extended release.Medarov BI, Judson MA.The role of calcium channel blockers for the treatment of pulmonary arterial hypertension: how much do we actually know and how could they be positioned today?.Respir Med. 2015;109(5):557-564. doi:10.1016/j.rmed.2015.01.004Food and Drug Administration.Opsynvi label.Sommer N, Ghofrani HA, Pak O, et al.Current and future treatments of pulmonary arterial hypertension.Br J Pharmacol. 2021;178(1):6-30. doi:10.1111/bph.15016Farber HW, Badesch DB, Benza RL, et al.Use of supplemental oxygen in patients with pulmonary arterial hypertension in REVEAL.J Heart Lung Transplant. 2018;37(8):948-955. doi:10.1016/j.healun.2018.03.010National Heart, Lung, and Blood Institute.Pulmonary hypertension research.Said K.Anticoagulation in pulmonary arterial hypertension: contemporary data from COMPERA registry.Glob Cardiol Sci Pract.2014;2014(2):48-52. doi:10.5339/gcsp.2014.25George MP, Champion HC, Pilewski JM.Lung transplantation for pulmonary hypertension.Pulm Circ. 2011;1(2):182-91. doi:10.4103/2045-8932.83455
National Institutes of Health.What is pulmonary hypertension?.
Taichman DB, Ornelas J, Chung L, Klinger JR, Lewis S, Mandel J, Palevsky HI, Rich S, Sood N, Rosenzweig EB, Trow TK, Yung R, Elliott CG, Badesch DB.Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report.Chest. 2014;146(2):449-475. doi:10.1378/chest.14-0793
Guillevin L, Armstrong I, Aldrighetti R, et al.Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives.Eur Respir Rev. 2013;22(130):535-542. doi:10.1183/09059180.00005713
George MG, Schieb L, Ayala C, Talwalkar A, Levant S.Pulmonary hypertension surveillance: United States, 2001 to 2010.Chest. 2014;146(2):476–95. doi:10.1378/chest.14-0527
National Organization for Rare Disorders.Pulmonary arterial hypertension.
American Lung Association.Pulmonary arterial hypertension symptoms and diagnosis.
Barnes H, Yeoh HL, Fothergill T, Burns A, Humbert M, Williams T.Prostacyclin for pulmonary arterial hypertension.Cochrane Database Syst Rev.2019;5(5):CD012785. doi: 10.1002/14651858.CD012785.pub2
Ruan CH, Dixon RA, Willerson JT, Ruan KH.Prostacyclin therapy for pulmonary arterial hypertension.Tex Heart Inst J.2010;37(4):391-399.
DailyMed.Label: Flolan- epoprostenol sodium injection, powder, lyophilized, for solution diluent- water solution.
Kingman M, Archer-Chicko C, Bartlett M, Beckmann J, Hohsfield R, Lombardi S.Management of prostacyclin side effects in adult patients with pulmonary arterial hypertension.Pulm Circ. 2017;7(3):598-608. doi:10.1177/2045893217719250
DailyMed.Label: Orenitram- treprostinil tablet, extended-release Orenitram- treprostinil kit.
DailyMed.Label: Uptravi- selexipag tablet, coated Uptravi titration pack- selexipag kit Uptravi- selexipag injection, powder, for solution.
DailyMed.Label: Ventavis- iloprost solution.
DailyMed.Label: Remodulin- treprostinil injection, solution sterile diluent for Remodulin- water injection, solution.
LiverTox.Clinical and research information on drug-induced liver injury: endothelin receptor antagonists.
Liu C, Chen J, Gao Y, Deng B, Liu K.Endothelin receptor antagonists for pulmonary arterial hypertension.Cochrane Database Syst Rev. 2013;2013(2):CD004434. doi:10.1002/14651858.CD004434.pub5
DailyMed.Label: Opsumit- macitentan tablet, film coated.
DailyMed.Label: Tracleer- bosentan tablet, film coated Tracleer- bosentan tablet, for suspension.
DailyMed.Label: Letairis- ambrisentan tablet, film coated.
Huang SA, Lie JD.Phosphodiesterase-5 (PDE5) inhibitors in the management of erectile dysfunction.P T. 2013;38(7):407-19.
Unegbu C, Noje C, Coulson JD, Segal JB, Romer L.Pulmonary hypertension therapy and a systematic review of efficacy and safety of PDE-5 inhibitors.Pediatrics. 2017;139(3):e20161450. doi:10.1542/peds.2016-1450
DailyMed.Label: Revatio- sildenafil citrate tablet, film coated Revatio- sildenafil citrate injection, solution Revatio- sildenafil citrate powder, for suspension.
DailyMed.Label: Adcirca- tadalafil tablet.
DailyMed.Label: Tadliq- tadalafil suspension.
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DailyMed.Label: Adempas- riociguat tablet, film coated.
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British Heart Foundation.Drug cabinet: calcium channel blockers.
DailyMed.Label: Norvasc- amlodipine besylate tablet.
DailyMed.Label: Procardia XL- nifedipine tablet, film coated, extended-release.
DailyMed.Label: Cardizem CD- diltiazem hydrochloride capsule, coated, extended release.
Medarov BI, Judson MA.The role of calcium channel blockers for the treatment of pulmonary arterial hypertension: how much do we actually know and how could they be positioned today?.Respir Med. 2015;109(5):557-564. doi:10.1016/j.rmed.2015.01.004
Food and Drug Administration.Opsynvi label.
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