Pulmonary Arterial Hypertension - NORD (National Organization for Rare Disorders) (2023)

Pulmonary Arterial Hypertension

NORD gratefully acknowledges Anna R. Hemnes, MD, Vanderbilt University Medical Center, Department of Medicine, for assistance in the preparation of this report.

Synonyms of Pulmonary Arterial Hypertension

  • FPAH
  • HPAH
  • idiopathic pulmonary arterial hypertension
  • IPAH
  • PAH
  • precapillary pulmonary hypertension
  • primary obliterative pulmonary vascular disease
  • primary pulmonary hypertension
  • associated pulmonary arterial hypertension
  • APAH

Subdivisions of Pulmonary Arterial Hypertension

  • heritable pulmonary arterial hypertension (HPAH)
  • idiopathic pulmonary arterial hypertension (IPAH)
  • associated pulmonary arterial hypertension (APAH)

General Discussion

Summary

Pulmonary arterial hypertension (PAH) is a rare, progressive disorder characterized by high blood pressure (hypertension) in the arteries of the lungs (pulmonary artery) for no apparent reason. The pulmonary arteries are the blood vessels that carry blood from the right side of the heart through the lungs. Symptoms of PAH include shortness of breath (dyspnea) especially during exercise, chest pain, and fainting episodes. The exact cause of PAH is unknown and although treatable, there is no known cure for the disease. PAH usually affects women between the ages of 30-60. Individuals with PAH may go years without a diagnosis, either because their symptoms are mild, nonspecific, or only present during demanding exercise. However, it is important to treat PAH because without treatment, high blood pressure in the lungs causes the right heart to work much harder, and over time, this heart muscle may weaken or fail. The progressive nature of this disease means that an individual may experience only mild symptoms at first, but will eventually require treatment and medical care to maintain a reasonable quality of life.

Approximately 15-20% of patients with PAH have heritable forms of PAH. People with heritable PAH have either: (1) an autosomal dominant genetic condition associated with mutations in theBMPR2gene or other recently identified genes now associated with HPAH or other forms of PAH or associated conditions such as pulmonary capillary hemangiomatosis or pulmonary veno-occlusive disease, or (2) are members of a family in which PAH is known to occur as primary disease.

Introduction

The first reported case of PAH occurred in 1891, when the German doctor E. Romberg published a description of a patient who, at autopsy, showed thickening of the pulmonary artery but no heart or lung disease that might have caused the condition. In 1951, 3 cases were reported by Dr. D.T. Dresdale in the U.S. and the illness was originally called primary pulmonary hypertension, which is a term no longer used but broadly refers to a form of pulmonary arterial hypertension (PAH). PAH has been linked to diet drugs such as Fen-Phen, Pondimin and Redux. These drugs were taken off the market in 1997, although cases related to drugs and toxins, such as methamphetamines do still appear. PAH is also associated with a number of other medical diseases such as cirrhosis, congenital heart disease and connective tissue diseases like scleroderma. About 30% of patients with PAH have an associated connective tissue disease.

NORD Video: Pulmonary Arterial Hypertension (Spanish)

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NORD Video: Pulmonary Arterial Hypertension

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Signs & Symptoms

PAH symptoms are those that are usually due to not having enough oxygen in the blood or inability of the heart to pump enough blood to meet the demands of the body. In most cases, the initial symptom is severe shortness of breath following exertion. Additional symptoms include excessive fatigue, weakness, chest pain, dizzy spells, and fainting episodes.

Affected individuals may also have a cough, sometimes with blood (hemoptysis), an enlarged heart and liver, low blood pressure (hypotension), and hoarseness due to compression of a nerve in the chest by an enlarged pulmonary artery.

Some affected individuals may experience puffiness or swelling of the face, ankles, abdomen and feet due to abnormal accumulation of fluid (edema) within fascial tissues.

Individuals with advanced stages of PAH may have abnormal bluish discoloration of the skin due to low levels of circulating oxygen in the blood (cyanosis). In addition, in severe cases of PAH, the right chamber (ventricle) of the heart is abnormally enlarged (hypertrophy), resulting in diminished functioning of the right portion of the heart and, potentially, right heart failure. Some patients with PAH are diagnosed with more advanced disease when they are no longer able to continue with their normal activities. At this time, the disease may have progressed to a point where the patient is completely bedridden from shortness of breath or other symptoms.

(Video) Pat Shares Her Pulmonary Hypertension Story

Causes

The exact cause of PAH is unknown. Researchers believe that injury to the layer of cells that line the small blood vessels of the lung, perhaps then causing or in concert with changes in the smooth muscle cells in the vessel wall, initiates blood vessel disease. This injury, which occurs for unknown reasons, results in the contraction of smooth muscle and therefore narrows the vessel. Researchers also think that some people who develop PAH have blood vessels that are particularly sensitive to certain internal or external factors and constrict, or narrow, when exposed to these factors.

Approximately 15-20% of patients with PAH have heritable PAH. Heritable PAH is an autosomal dominant genetic condition caused by changes (mutations) in theBMPR2gene most commonly, though recently other genes and pathways have been identified. In approximately 20% of families with PAH, we do not yet know the underlying gene mutations.

Dominant genetic disorders occur when only a single copy of an abnormal gene is necessary for the appearance of the disease. The abnormal gene can be inherited from either parent, or can be the result of a new mutation in the affected individual. Approximately 80% of individuals who have a mutated (abnormal)BMPR2gene will not develop PAH, so other genes or environmental triggers must be necessary for PAH to develop. The risk of passing the abnormal gene from parent to child is 50% for each pregnancy and the risk is the same for males and females.

Of note, there now exist several publications associating PAH with mutations in other genes in a small number of patients (e.g.CAV1,KCNK3, etc), although many of these genes are closely linked toBMPR2in terms of biologic signaling (SMAD9, ALK1, endoglin). Recently, pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis have been linked to mutations in the geneEIF2AK4. The function of this gene is still being studied.

In August 1996, the Food and Drug Administration (FDA) evaluated data from a report of the International Primary Pulmonary Hypertension Study (IPPHS). The study examined the relationship between appetite-suppressant drugs (dexfenfluramine [Redux] and fenfluramine [Pondimin] and what was then called primary pulmonary hypertension. Findings indicated that the risk of primary pulmonary hypertension (now called a type of PAH) in individuals using appetite-suppressant drugs for three months or longer is about nine times higher than the risk for non-users. The final IPPHS report estimated that the risk of this disorder is about 23 times higher in individuals who use appetite-suppressants for three months or longer. These drugs were taken off the market in 1997, though other diet drugs have been associated with PAH such as benfluorex that was used in Europe until 2009.

Along with the increased risk of diet pills, other exposures have been associated with the development of PAH. These include methamphetamines and dasatanib. In addition, a very small percentage of people with HIV develop PAH. In terms of other exposures which may contribute to the development of PAH, few are validated in rigorous studies. However, female sex hormones are an area of great interest for several reasons, including: (1) the higher risk of PAH among females and (2) the association of pregnancy with the development of PAH (may be more common in the peripartum period).

Several conditions have been associated with PAH such as liver disease (cirrhosis), congenital heart disease and connective tissue diseases such as scleroderma. The mechanisms through which these conditions might cause PAH are presently being studied and are unknown.

Scleroderma is a chronic systemic autoimmune disease (primarily of the skin) characterized by fibrosis (or hardening), vascular alterations, and auto-antibodies. One of the serious complications of this rare disease is PAH, which can occur in up to a third of scleroderma patients. Almost everyone with scleroderma experiences Raynaud’s phenomenon, or cold sensitivity in the fingers and toes, as well, however it is important to note that many patients with PAH have Raynaud’s phenomenon and do not have scleroderma. (For more information on this disorder, choose “scleroderma” as your search term in the Rare Disease Database.)

Persistent pulmonary hypertension of the newborn (PPHN) is a kind of PAH that occurs when a newborn’s circulatory system does not adapt to breathing outside the womb. It occurs most often in full-term or post-term babies who had a difficult birth. Newborns that have PPHN have rapid respiration (tachypnea) and abnormal bluish discoloration of the skin due to low levels of circulating oxygen in the blood (cyanosis). The disorder is believed to be caused by insufficient oxygen in the blood flowing to the lungs just before, during or after birth (perinatal hypoxemia), although its precise cause is unknown.

Affected Populations

PAH occurs 3-5 times more frequently in females than in males. It tends to affect females between the ages of 30 and 60. New cases are estimated to occur in one to two individuals per million each year in the U.S. The incidence is estimated to be similar in Europe. Approximately 500-1000 new cases of PAH are diagnosed each year in the U.S. There is no ethnic or racial group that is known to have a higher frequency of patients with PAH. An exception to this is an apparent paucity of cases of HPAH among subjects of African ancestry, although this may relate to reporting bias and has not been rigorously studied.

A rare form of pulmonary hypertension affects individuals who are at high altitude levels (e.g., mountain climbing). It is not recommended for people with PAH or a family history of PAH to live at high altitudes.

Related Disorders

Pulmonary hypertension is a general term that means high blood pressure that occurs only in the arteries in the lungs may affect the right side of the heart. The term PAH refers to a specific cause of pulmonary hypertension, but there are other causes of pulmonary hypertension as well. There are four other main causes of pulmonary hypertension:

  1. pulmonary hypertension due to left heart disease
  2. pulmonary hypertension due to lung disease
  3. pulmonary hypertension due to blood clots in the lung
  4. miscellaneous causes (such as kidney disease, sarcoidosis and others)

Comparisons may be useful for a differential diagnosis:

Most advanced stages of cardiac conditions affecting the left heart can cause pulmonary hypertension through back up of fluid into the lung blood vessels. These cardiac diseases include ischemic heart disease, valvular disease, and cardiomyopathy. They can be ruled out as the cause of pulmonary hypertension by careful history, and using electrocardiography, echocardiography, and cardiac catheterization. This kind of pulmonary hypertension is sometimes called pulmonary venous hypertension. Pulmonary venous hypertension can lead to accumulation of blood in the lung which puts the affected individual at a higher risk for developing pleural effusions and pulmonary edema. Like PAH, pulmonary venous hypertension can cause high blood pressure in the pulmonary artery, difficulty breathing, fatigue, and cough.

Lung disease is a leading cause of secondary pulmonary hypertension. Most lung diseases that lead to pulmonary hypertension are detected by abnormal lung sounds on physical examination, pulmonary function testing, and/or high resolution computed tomographic lung imaging. Interstitial pneumonia is a type of chronic lung disease that is associated with pulmonary hypertension. It involves the spaces and tissues in the lining of the lungs with abnormal increases in these tissues. Major symptoms may include shortness of breath on exertion, coughing and loss of appetite. The symptoms may vary from mild to severe according to the extent of involvement. An affected individual usually has no fever, and there is usually no overproduction of mucous.

Blood clots may obstruct blood flow into the pulmonary arteries and can cause pulmonary hypertension, so called chronic thromboembolic pulmonary hypertension. These are usually detected by history and specialized lung imaging. This cause of pulmonary hypertension is important to test for because it can be cured in some patients with surgery.

Cor pulmonale is a term that denotes enlargement of the right ventricle of the heart that occurs as a result of severe lung disease and associated pulmonary hypertension. It is used as a term for pulmonary heart disease that affects both the heart and lungs. A common cause of cor pulmonale is massive lung clotting from chronic thromboembolic pulmonary hypertension that results in increased pressure in the right ventricle of the heart, usually resulting in heart failure. Additional causes include severe forms of chronic bronchitis and emphysema, as well as extensive loss of lung tissue from surgery or injury. Symptoms usually include difficulty breathing, fainting spells upon exertion, and substernal angina pain in the chest.

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Diagnosis

It can often be hard to detect PAH in a routine clinical examination, even if the disease has progressed. Symptoms of PAH are not unique and may be confused with many other diseases that cause a lack of oxygen in the blood. The diagnosis of PAH is also one of exclusion, meaning that PAH is only diagnosed when other causes of pulmonary hypertension have been ruled out and there seems to be no known cause of the hypertension. The tests that are commonly performed to diagnose PAH and rule out other diseases are echocardiography, blood tests, pulmonary function tests, X-rays of the chest, lung blood flow scans, electrocardiography (ECG), and the “6-minute walk test”, which measures how far an individual can walk in that time period. Ultimately, the majority of subjects undergo confirmation by cardiac catheterization with and without vasodilator testing.

Heritable PAH is confirmed if two or more family members have PAH or if aBMPR2gene mutation or mutation in another gene known to cause PAH is identified in the affected person. Molecular genetic testing for mutations in theBMPR2gene is available, but should only be performed in concert with genetic counseling.

Clinical Testing and Work-Up

Patients suspected to have PAH should be referred to a referral center specializing in PAH diagnosis and treatment. The Pulmonary Hypertension Association website can provide contact information for these centers.

Genetic counseling is recommended for affected individuals and their families.

Standard Therapies

Treatment

Several medications have been approved by the US Food and Drug Administration (FDA) for the treatment of PAH. These medications can be broadly broken down into four categories described below.

Prostaglandins

The orphan drug Flolan (epoprostenol sodium for injection or prostacycline) has been approved as a standard long-term treatment of individuals with severe PAH. It was the first drug approved specifically for patients with pulmonary hypertension. This drug is used in individuals who do not respond to other types of therapy and in patients with very severe disease. This drug is administered by intravenous infusion through a permanent ambulatory in-dwelling central venous catheter. Since this drug requires continuous infusion, it must not be withdrawn suddenly (including sudden reduction of dosage). Flolan, which is a version of a natural hormone called prostacyclin that dilates constricted blood vessels, is manufactured by GlaxoSmithKline.

Recently, a room temperature stable form of epoprostenol had been developed. This is known and Veletri and is made by Actelion Pharmaceuticals US, Inc.

The FDA has approved the orphan drug treprostinil (Remodulin) in subcutaneous and intravenous forms and Tyvaso, an inhaled form of treprostinil, and oral form (Orenitram) for the treatment of PAH. The drug is made by United Therapeutics Corp.

In 2004, the FDA approved iloprost (Ventavis) for the treatment of PAH. The treatment is inhaled through the mouth with the assistance of a special nebulizer, dilating the arteries and preventing the formation of blood clots. Ventavis is marketed in the U.S. by Actelion Pharmaceuticals US, Inc.

Endothelin Receptor Antagonists

The orphan drug bosentan (Tracleer) has been approved by the FDA for treatment of PAH. The drug allows affected individuals to exert themselves physically with less shortness of breath. It should be carefully monitored while in use. Tracleer is manufactured by Actelion Pharmaceuticals US, Inc.

The FDA approved the orphan drug Ambrisentan (Letairis) for treatment of PAH in June 2007. It is used primarily to make exercise and breathing easier.

The FDA approved the orphan drug Macitentan (Opsumit) for treatment of PAH in October 2013. In clinical trials this drug was shown to delay disease progression. It works through similar mechanisms as bosentan and ambrisentan. Macitentan is manufactured by Actelion Pharmaceuticals US, Inc.

Because of the risk of birth defects with this entire class of medication, these drugs are available only through a special restricted distribution program and require monthly pregnancy testing for women capable of becoming pregnant.

Phosphodiesterase Type 5 Inhibitors

Revatio (sildenafil), a phosphodiesterase type 5 (PDE5) inhibitor is also used to treat PAH. In clinical studies it increased the distance people walked and decreased pressure in the pulmonary artery. It contains the same ingredient as Viagra (sildenafil citrate). This medication is manufactured by Pfizer Pharmaceuticals.

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Tadalafil (Adcirca) is a once-daily phosphodiesterase type 5 (PDE-5) inhibitor, shown to improve the patient’s ability to exercise. Adcirca contains the same ingredient (tadalafil) as Cialis.This medication is from United Therapeutics.

Other Mechanisms

The FDA has approved the drug riociguat (Adempas) for the treatment of PAH. Riociguat works on the same pathway as the phosphodiesterase type 5 inhibitors. This medication is made by Bayer.

In 2016, selexipag(Uptravi) was approved for adults and acts by relaxing muscles in the walls of blood vessels. Uptravi is manufactured by Actelion Pharmaceuticals US, Inc.

Supportive Therapies

Drugs that cause widening of blood vessels (vasodilators) and lessen blood pressure may also be used to treat PAH. In some PAH cases, calcium channel blockers (e,g, nifedipine and diltiazem) are used as vasodilators. Unfortunately, only a small minority of patients appear to respond with improvement to the use of calcium channel blockers. Other vasodilator drugs have been used including phentolamine, phenoxybenzamine and prazosin. The effectiveness of vasodilator therapy varies from case to case.

Other treatments such as anticoagulants, diuretics, and oxygen may be used to treat PAH as supportive therapies. Anticoagulants, such as warfarin, are drugs that prevent blood clots from forming. There are equivocal data on whether these drugs are useful in PAH patients and there are significant bleeding risks associated with them. Diuretics are used to treat fluid retention and swelling (edema) often associated with the condition.

To continue with daily activities some individuals may need to carry portable oxygen when they go out. Often light exercise such as walking is still possible for PAH patients and can be useful to maintain muscle strength and conditioning.

In severe cases of PAH, a heart-lung, single lung or double lung transplant may be recommended. In patients with lung transplants, both the structure and function of the right ventricle markedly improve. Lung transplant is itself a difficult process and results in new challenges for patients who undergo this procedure. Complications of transplantation include rejection of the transplanted organ and infection. Patients take medications for life to reduce their immune system’s ability to reject their transplanted organ.

Pregnancy is not advised for patients with PAH because it puts an extra load on the heart. Estrogen-containing oral contraceptives are generally not recommended, but other types of birth control may be used.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. funding, and some supported by private industry, are posted on this government website.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email:[emailprotected]

Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/for-patients-and-families/information-resources/news-patient-recruitment/

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/

Familial Pulmonary Arterial Hypertension (FPAH) Study

The major goals of the study are to understand the gene(s) that causes the disease, attempt to develop new treatments, and provide information to patients and physicians. Current studies are varied and include: 1) Estrogen study to determine if estrogen effects explain why women get this disease more frequently than men. For this study, urine samples are needed and a health history questionnaire must be completed by study participants– patients and family members, males and females. 2) Why do some family members with a mutation in the BMPR2 gene never develop disease? Are other genes involved in controlling who gets FPAH and who is protected? This study requires blood samples and possibly a small skin biopsy (no stitches required) from patients and family members to provide the materials needed to evaluate other possible genetic influences on disease development.

For more information contact:

Kelly Fox, Coordinator
Vanderbilt University Medical Center
1161 21st Ave. S., T-1218 MCN
Nashville, TN 37232-2650
1-800-288-0378
FAX 1-615-343-7587
[emailprotected]

For further information regarding Pulmonary Arterial Hypertension:

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Anna R. Hemnes, MD
Vanderbilt University Medical Center,
Division of Allergy, Pulmonary and Critical Care Medicine
T1218 Medical Center North
1161 21st Avenue South
Vanderbilt University School of Medicine
Nashville, TN 37232
Phone: 615-322-3412
Fax: 615-343-7448
[emailprotected]

Resources

RareConnect offers a safe patient-hosted online community for patients and caregivers affected by this rare disease. For more information, visitwww.rareconnect.org.

NORD Member Organizations

  • Pulmonary Hypertension Association

Other Organizations

  • American Heart Association
    • 7272 Greenville Avenue
    • Dallas, TX 75231
    • Phone: (214) 784-7212
    • Toll-free: (800) 242-8721
    • Email: [emailprotected]
    • Website: http://www.heart.org
  • American Lung Association
    • 55 W. Wacker Drive
    • Suite 1150
    • Chicago, IL 60601 USA
    • Toll-free: (800) 548-8252
    • Email: [emailprotected]
    • Website: https://www.lung.org/
  • Genetic and Rare Diseases (GARD) Information Center
  • iSEEK Pulmonary Hypertension Hope Center
    • No. 9501A, Guobin Plaza,
    • NO.11 Fuchengmenwai Street
    • Xicheng District, Beijing, P. R. China
    • Email: [emailprotected]
    • Website: http://www.iseek.org.cn/
  • NIH/National Heart, Lung and Blood Institute
  • PHA EUROPE, European Pulmonary Hypertension Association
  • Scleroderma Research Foundation
    • 220 Montgomery Street
    • Suite 1411
    • San Francisco, CA 94104 USA
    • Phone: (415) 834-9444
    • Toll-free: (800) 441-2873
    • Email: [emailprotected]
    • Website: http://www.srfcure.org/home

References

TEXTBOOKS
McGoon MD. Primary Pulmonary Hypertension. In: The NORD Guide to Rare Disorders, Philadelphia: Lippincott, Williams and Wilkins, 2003:678.

JOURNAL ARTICLES
Taichman D OJ, Chung L, Klinger J, Lewis S, Mandel J, Palevsky H, Rich S, Sood N, Trow T, Yung R, Elliott C, Badesch D. Pharmacological therapy for pulmonary arterial hypertension in adults: Chest guideline. Chest. 2014;146(2):449-475. 2014;146(2):449-475.

Ghofrani HA, D’Armini AM, Grimminger F, Hoeper MM, Jansa P, Kim NH, Mayer E, Simonneau G, Wilkins MR, Fritsch A, Neuser D, Weimann G, Wang C, Group C-S. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013;369:319-329.

McLaughlin VV, Gaine SP, Howard LS, Leuchte HH, Mathier MA, Mehta S, Palazzini M, Park MH, Tapson VF, Sitbon O. Treatment goals of pulmonary hypertension. J Am Coll Cardiol. 2013;62:D73-81.

Pulido et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. NEJM 2013;369:809-818.

Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A, Gomez Sanchez MA, Krishna Kumar R, Landzberg M, Machado RF, Olschewski H, Robbins IM, Souza R. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62:D34-41.

Galie N, Brundage BH, Ghofrani HA, Oudiz RJ, Simonneau G, Safdar Z, Shapiro S, White RJ, Chan M, Beardsworth A, Frumkin L, Barst RJ. Tadalafil therapy for pulmonary arterial hypertension. Circulation. 2009;119:2894-2903.

McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol 2009;53:1573-619.

Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009;54:S43-54.

INTERNET
Austin ED, Phillips JA III, Loyd JE. Heritable Pulmonary Arterial Hypertension Overview. 2002 Jul 18 [Updated 2020 Dec 23]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2021.Available from: https://www.ncbi.nlm.nih.gov/books/NBK1485 Accessed March 1, 2021.

McKusick VA, ed. Online Mendelian Inheritance in Man (OMIM). Baltimore. MD: The Johns Hopkins University; Pulmonary Hypertension, Primary. Entry No: 178600; Last Update: 06/04/2019.http://omim.org/entry/178600Accessed March 1, 2021.

Oudiz, RJ. Idiopathic Pulmonary Arterial Hypertension. Medscape Updated: Updated:Jul 8, 2020.http://emedicine.medscape.com/article/301450-overviewAccessed March 1, 2021.

Years Published

1990, 1992, 1995, 1996, 1997, 1998, 1999, 2001, 2002, 2004, 2011, 2015, 2018, 2021

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FAQs

Is pulmonary hypertension considered a rare disease? ›

PAH is a rare disease affecting 15 to 50 cases per million. Its prevalence is higher in certain groups of patients suffering from other diseases. For instance, in patients with sickle cell disease, the prevalence is around 2% to 3.75%. PAH affects people of all ages, races, and ethnic backgrounds.

What autoimmune diseases cause pulmonary hypertension? ›

PAH is a common complication of autoimmune diseases, such as systemic sclerosis (SSc), mixed connective tissue disease and systemic lupus erythematosus 11, 12. SSc is characterised by excessive collagen deposition in the dermis and internal organs, vascular hyper-reactivity and obliterative microvascular phenomena 13.

What is the survival rate for pulmonary hypertension? ›

For patients treated by experts in PAH, the average survival now exceeds 7 years and may be closer to 10 years.

What is the most common cause of pulmonary arterial hypertension? ›

Problems with the left side of the heart are thought to be one of the most common causes of pulmonary hypertension. These include mitral valve problems, left ventricle problems and aortic valve conditions.

What is the best treatment for pulmonary hypertension? ›

Treatments include: anticoagulant medicines – such as warfarin to help prevent blood clots. diuretics (water tablets) – to remove excess fluid from the body caused by heart failure. oxygen treatment – this involves inhaling air that contains a higher concentration of oxygen than normal.

What is the best medication for pulmonary hypertension? ›

A commonly prescribed vasodilator for pulmonary hypertension is epoprostenol (Flolan, Veletri). This drug continuously flows through an IV attached to a small pump, which is worn in a pack on the belt or shoulder.

Is pulmonary hypertension considered autoimmune disease? ›

Pulmonary arterial hypertension (PAH) is a progressive life-threatening disease. The notion that autoimmunity is associated with PAH is widely recognized by the observations that patients with connective tissue diseases or virus infections are more susceptible to PAH.

Can Rheumatoid arthritis cause pulmonary hypertension? ›

Rheumatoid arthritis associated pulmonary hypertension can be attributed to interstitial lung disease, vascular disease, and chronic thromboembolic disease. Chronic inflammation reduces patient's functional capacity and conceals early symptoms of cardiovascular disease and pulmonary hypertension.

Is hypertension an inflammatory disease? ›

Approximately 90% of hypertension cases are classified as essential hypertension, where the precise cause is unknown. Hypertension is associated with inflammation; however, whether inflammation is a cause or effect of hypertension is not well understood.

What should I avoid if I have pulmonary hypertension? ›

Avoiding caffeine and other stimulants, such as alcohol, is necessary for regulating your blood pressure. Try tea and coffee substitutes like chicory if you like a hot beverage in the morning.

Can pulmonary hypertension go away? ›

Pulmonary hypertension cannot be cured, but treatment can reduce the symptoms and help you manage your condition. Pulmonary hypertension usually gets worse over time. Left untreated, it may cause heart failure, which can be fatal, so it's important treatment is started as soon as possible.

How do you sleep with pulmonary hypertension? ›

11 Tips to Make Sleeping Better With Pulmonary Hypertension
  1. Be regular: To avoid an irregular sleeping schedule, try to be as regular during the day as you possibly can. ...
  2. Don't sleep in: Even if you had a rough night, resist the urge to sleep in. ...
  3. Don't nap: Or at least try not to.
23 Aug 2017

What organ systems are affected by pulmonary hypertension? ›

Pulmonary hypertension is a type of high blood pressure that affects the arteries in the lungs and the right side of the heart. In one form of pulmonary hypertension, called pulmonary arterial hypertension (PAH), blood vessels in the lungs are narrowed, blocked or destroyed.

What is the first line treatment for pulmonary hypertension? ›

Epoprostenol (Flolan)

This is the first drug specifically approved for the treatment of pulmonary hypertension. Flolan is the most effective drug for the treatment of advanced disease.

How does pulmonary hypertension make you feel? ›

It is a serious medical condition as it results in damage to the right-hand side of the heart muscle, making the heart muscle less efficient at pumping blood and oxygen around the body. This leads to symptoms such as: shortness of breath. fatigue (tiredness)

How rare is primary pulmonary hypertension? ›

PAH is referred to as Group 1: PAH among the five types of pulmonary hypertension. PAH is a rare condition, with about 500-1000 new cases being diagnosed each year in the U.S. About 15-20% of patients with PAH have inherited the condition.

Is pulmonary hypertension a genetic disease? ›

Some cases of PAH are due to genetic changes in the BMPR2 gene and inherited in an autosomal dominant pattern. Most cases of PAH occur in individuals with no family history of the disorder. When PAH is inherited from an affected relative it is called "familial" PAH.

Is pulmonary hypertension a terminal? ›

Pulmonary hypertension cannot be cured, but treatment can reduce the symptoms and help you manage your condition. Pulmonary hypertension usually gets worse over time. Left untreated, it may cause heart failure, which can be fatal, so it's important treatment is started as soon as possible.

Is pulmonary hypertension an orphan disease? ›

Thus, collectively, pulmonary hypertension cannot be considered an orphan condition from a global perspective.

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Name: Gov. Deandrea McKenzie

Birthday: 2001-01-17

Address: Suite 769 2454 Marsha Coves, Debbieton, MS 95002

Phone: +813077629322

Job: Real-Estate Executive

Hobby: Archery, Metal detecting, Kitesurfing, Genealogy, Kitesurfing, Calligraphy, Roller skating

Introduction: My name is Gov. Deandrea McKenzie, I am a spotless, clean, glamorous, sparkling, adventurous, nice, brainy person who loves writing and wants to share my knowledge and understanding with you.