Pulmonary arterial hypertension
Senast uppdaterad 28 feb 2023
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Pulmonary arterial hypertension is a progressive disorder characterised by abnormally high blood pressure (hypertension) in the pulmonary artery, the blood vessel that carries blood from the heart to the lungs. Pulmonary arterial hypertension is one form of a broader condition known as pulmonary hypertension. Pulmonary hypertension occurs when most of the very small arteries throughout the lungs narrow in diameter, which increases the resistance to blood flow through the lungs. To overcome the increased resistance, blood pressure increases in the pulmonary artery and in the right ventricle of the heart, which is the chamber that pumps blood into the pulmonary artery. Ultimately, the increased blood pressure can damage the right ventricle of the heart.
Beskrivning
Pulmonary arterial hypertension is a progressive disorder characterised by abnormally high blood pressure (hypertension) in the pulmonary artery, the blood vessel that carries blood from the heart to the lungs. Pulmonary arterial hypertension is one form of a broader condition known as pulmonary hypertension. Pulmonary hypertension occurs when most of the very small arteries throughout the lungs narrow in diameter, which increases the resistance to blood flow through the lungs. To overcome the increased resistance, blood pressure increases in the pulmonary artery and in the right ventricle of the heart, which is the chamber that pumps blood into the pulmonary artery. Ultimately, the increased blood pressure can damage the right ventricle of the heart.
Signs and symptoms of pulmonary arterial hypertension occur when increased blood pressure cannot fully overcome the elevated resistance. As a result, the flow of oxygenated blood from the lungs to the rest of the body is insufficient. Shortness of breath (dyspnea) during exertion and fainting spells are the most common symptoms of pulmonary arterial hypertension. People with this disorder may experience additional symptoms, particularly as the condition worsens. Other symptoms include dizziness, swelling (edema) of the ankles or legs, chest pain, and a rapid heart rate.
Inheritance patterns
The source lists the following possible inheritance patterns: Autosomal dominant. More than one pattern can apply in different cases.
Related genes
Other names
Ayerza syndrome
Familial primary pulmonary hypertension
FPPH
Idiopathic pulmonary hypertension
PAH
PPH
PPHT
Primär pulmonell hypertension
Sporadic primary pulmonary hypertension
Related patient information and medicines
Medicine leaflet
Macitentan för pulmonell arteriell hypertension
About macitentan Type of medicine A vasodilator antihypertensive medicine Used for Pulmonell hypertension Also called Opsumit® Available as Tablets Pulmonary arterial hypertension (PAH) is a condition where there is too high a blood pressure in the arteries which supply blood to your lungs from your heart. The arteries become narrowed so your.
Medicine leaflet
Iloprost för pulmonell arteriell hypertension
About iloprost Type of medicine A vasodilator antihypertensive medicine Used for Pulmonary arterial hypertension Also called Ventavis® Available as Nebuliser solution for inhalation Pulmonary arterial hypertension (PAH) is a condition where there is too high a blood pressure in the blood vessels which supply blood to your lungs from your heart..
Medicine leaflet
Sildenafil för pulmonell arteriell hypertension
Om sildenafil for pulmonary arterial hypertension Type of medicine A vasodilator antihypertensive medicine Used for Pulmonary arterial hypertension Also called Revatio®; Granpidam® Available as Tablets and oral liquid medicine Pulmonary arterial hypertension (PAH) is a condition where there is too high a blood pressure in the arteries which.
Patient information leaflet
Användning av syrgasbehandling vid KOL
How does oxygen help chronic obstructive pulmonary disease. Oxygen is used to take the strain off your heart, not your lungs. It helps to prevent something called 'pulmonary artery hypertension'. This is quite an unusual condition where the right-hand side of your heart gets worn out by trying to pump blood harder and harder to your lungs. Some.
Patient information leaflet
Systemisk skleros
Systemic sclerosis is a type of scleroderma, which means hard skin. If you have scleroderma, you have areas of hardening of the skin. In systemic sclerosis, you can also g... Lung symptoms Scarring of the lung tissue, called interstitial fibrosis. This can give you a cough or make you feel breathless as the lungs can't work as well as usual. High.
Patient information leaflet
Medfödd hjärtsjukdom
Patent ductus arteriosus (PDA). The ductus arteriosus is a tube which connects the two main arteries of the heart (the pulmonary artery and the aorta). This connection is important when the baby is in the womb, but after birth it closes off. An increased risk of blood clots and strokes. Pulmonary hypertension. This is a knock-on effect where there.
Vidare läsning och referenser
- Pulmonary arterial hypertension; MedlinePlus, National Library of Medicine.
- Austin ED, Loyd JE. The genetics of pulmonary arterial hypertension. Circ Res. 2014 Jun 20;115(1):189-202. doi: 10.1161/CIRCRESAHA.115.303404.
- Geiger H, Tinker RJ, Talbert J, Phillips JA III, Austin ED. Heritable Pulmonary Arterial Hypertension Overview. 2002 Jul 18 [updated 2026 Apr 9]. In: Adam MP, Bick S, Mirzaa GM, Wallace SE, Amemiya A, editors. GeneReviews(R) [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2026. Available from http://www.ncbi.nlm.nih.gov/books/NBK1485/
- Machado RD, Aldred MA, James V, Harrison RE, Patel B, Schwalbe EC, Gruenig E, Janssen B, Koehler R, Seeger W, Eickelberg O, Olschewski H, Elliott CG, Glissmeyer E, Carlquist J, Kim M, Torbicki A, Fijalkowska A, Szewczyk G, Parma J, Abramowicz MJ, Galie N, Morisaki H, Kyotani S, Nakanishi N, Morisaki T, Humbert M, Simonneau G, Sitbon O, Soubrier F, Coulet F, Morrell NW, Trembath RC. Mutations of the TGF-beta type II receptor BMPR2 in pulmonary arterial hypertension. Hum Mutat. 2006 Feb;27(2):121-32. doi: 10.1002/humu.20285.
- Machado RD, Southgate L, Eichstaedt CA, Aldred MA, Austin ED, Best DH, Chung WK, Benjamin N, Elliott CG, Eyries M, Fischer C, Graf S, Hinderhofer K, Humbert M, Keiles SB, Loyd JE, Morrell NW, Newman JH, Soubrier F, Trembath RC, Viales RR, Grunig E. Pulmonary Arterial Hypertension: A Current Perspective on Established and Emerging Molecular Genetic Defects. Hum Mutat. 2015 Dec;36(12):1113-27. doi: 10.1002/humu.22904. Epub 2015 Oct 12.
- Montani D, Gunther S, Dorfmuller P, Perros F, Girerd B, Garcia G, Jais X, Savale L, Artaud-Macari E, Price LC, Humbert M, Simonneau G, Sitbon O. Pulmonary arterial hypertension. Orphanet J Rare Dis. 2013 Jul 6;8:97. doi: 10.1186/1750-1172-8-97.
- Soubrier F, Chung WK, Machado R, Grunig E, Aldred M, Geraci M, Loyd JE, Elliott CG, Trembath RC, Newman JH, Humbert M. Genetics and genomics of pulmonary arterial hypertension. J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D13-21. doi: 10.1016/j.jacc.2013.10.035.
Artikelhistorik
Artikeln finns också på Engelska, Tyska, Spanska, Franska, Italienska, Portugisiska, Hindi, Hebreiska, Arabiska, och Svenska.
28 feb 2023 | Senaste versionen
1 Jan 2016 | Ursprungligen publicerad
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