Pulmonary hypertension

Pulmonary hypertension
Other namesAyerza syndrome[1]
Pulmonary hypertension
SpecialtyPulmonology, cardiology
SymptomsChest pain, fatigue[2]
Usual onset20 to 60 years old[3]
DurationLong term[4]
CausesUnknown[4]
Risk factorsFamily history, pulmonary embolism, HIV/AIDS, sickle cell disease, cocaine use, COPD, sleep apnea, living at high altitudes[5][3]
Diagnostic methodFollowing ruling out other potential causes[4]
TreatmentSupportive care, various medications, lung transplantation[4][6]
MedicationEpoprostenol, treprostinil, iloprost, bosentan, ambrisentan, macitentan, sildenafil[4]
Frequency1,000 new cases a year (US)[1]

Pulmonary hypertension (PH or PHTN) is a condition of increased blood pressure in the arteries of the lungs.[7] Symptoms include shortness of breath, fainting, tiredness, chest pain, swelling of the legs, and a fast heartbeat.[7][1] The condition may make it difficult to exercise.[7] Onset is typically gradual.[8] According to the definition at the 6th World Symposium of Pulmonary Hypertension in 2018, a patient is deemed to have pulmonary hypertension if the pulmonary mean arterial pressure is greater than 20mmHg at rest, revised down from a purely arbitrary 25mmHg, and pulmonary vascular resistance (PVR) greater than 3 Wood units.

The cause is often unknown.[4] Risk factors include a family history, prior pulmonary embolism (blood clots in the lungs), HIV/AIDS, sickle cell disease, cocaine use, chronic obstructive pulmonary disease, sleep apnea, living at high altitudes, and problems with the mitral valve.[5][3] The underlying mechanism typically involves inflammation and subsequent remodeling of the arteries in the lungs.[5] Diagnosis involves first ruling out other potential causes.[4] High cardiac output states, such as advanced liver disease or the presence of large arteriovenous fistulas, may lead to an elevated mean pulmonary artery pressure (mPAP) greater than 20 mm Hg despite a pulmonary vascular resistance (PVR) less than 2 Wood units, which does not necessarily indicate pulmonary vascular disease.[9]

As of 2022 there was no cure for pulmonary hypertension,[6] although research to find a cure is ongoing. Treatment depends on the type of disease.[6] A number of supportive measures such as oxygen therapy, diuretics, and medications to inhibit blood clotting may be used.[4] Medications specifically used to treat pulmonary hypertension include epoprostenol, treprostinil, iloprost, bosentan, ambrisentan, macitentan, and sildenafil, tadalafil, selexipag, riociguat.[4] Lung transplantation may be an option in severe cases.[6]

The frequency of occurrence is estimated at 1,000 new cases per year in the United States.[3][1] Females are more often affected than males.[1] Onset is typically between 20 and 60 years of age.[3] Pulmonary hypertension was identified by Ernst von Romberg in 1891.[10][4]

  1. ^ a b c d e "Pulmonary arterial hypertension". Genetics Home Reference. January 2016. Archived from the original on 28 July 2017. Retrieved 30 July 2017.
  2. ^ Cite error: The named reference www.nhlbi.nih.gov was invoked but never defined (see the help page).
  3. ^ a b c d e "Who Is at Risk for Pulmonary Hypertension?". NHLBI – NIH. 2 August 2011. Archived from the original on 31 July 2017. Retrieved 30 July 2017.
  4. ^ a b c d e f g h i j Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RM, Brida M, et al. (October 2022). "2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension". European Heart Journal. 43 (38): 3618–3731. doi:10.1093/eurheartj/ehac237. PMID 36017548.
  5. ^ a b c "Causes and Risk Factors". Health Topics: Pulmonary Hypertension. National Heart, Lung and Blood Institute. National Library of Medicine. 24 March 2022.
  6. ^ a b c d "Treatment". Health Topics: Pulmonary Hypertension. National Heart, Lung and Blood Institute. National Library of Medicine. 24 March 2022.
  7. ^ a b c NHLBI & What Is Pulmonary Hypertension? 2023
  8. ^ "Diagnosis". Health Topics: Pulmonary Hypertension. National Heart, Lung and Blood Institute. National Library of Medicine. March 24, 2022.
  9. ^ Maron, Bradley A. (2023). "Revised Definition of Pulmonary Hypertension and Approach to Management: A Clinical Primer". Journal of the American Heart Association. 12 (8): e029024. doi:10.1161/JAHA.122.029024. PMC 10227272. PMID 37067284.
  10. ^ von Romberg E (1891–1892). "Über Sklerose der Lungenarterie". Dtsch Arch Klin Med (in German). 48: 197–206.
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