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Idiopathic pulmonary arterial hypertension(PAH)

MedGen UID:
468368
•Concept ID:
C3203102
•
Disease or Syndrome
Synonyms: Hypertension, Idiopathic Pulmonary; Hypertension, Primary Pulmonary; Idiopathic Pulmonary Arterial Hypertension; Idiopathic Pulmonary Hypertension; Primary Pulmonary Hypertension; Pulmonary Hypertension, Idiopathic; Pulmonary Hypertension, Primary
SNOMED CT: Idiopathic pulmonary arterial hypertension (697898008)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Related genes: ATP13A3, SMAD9, KCNK3, CAV1, BMPR2
 
Monarch Initiative: MONDO:0017147
OMIM®: 178600
Orphanet: ORPHA275766

Definition

Idiopathic pulmonary arterial hypertension (IPAH) is a sporadic form of pulmonary arterial hypertension (PAH, see this term) characterized by elevated pulmonary arterial resistance leading to right heart failure. IPAH is progressive and potentially fatal and not associated with an underlying condition or family history of PAH. [from ORDO]

Professional guidelines

PubMed

Targeting IL-11 system as a treatment of pulmonary arterial hypertension.
Milara J, Roger I, Montero P, Artigues E, Escrivá J, Perez-Vizcaino F, Cortijo J
Pharmacol Res 2023 Nov;197:106985. Epub 2023 Nov 9 doi: 10.1016/j.phrs.2023.106985. PMID: 37949331
Emerging pharmacotherapies for the treatment of pulmonary arterial hypertension.
Auth R, Klinger JR
Expert Opin Investig Drugs 2023 Jul-Dec;32(11):1025-1042. Epub 2023 Nov 24 doi: 10.1080/13543784.2023.2274439. PMID: 37881882
Pulmonary hypertension in connective tissue diseases: epidemiology, pathogenesis, and treatment.
Cansu DÜ, Korkmaz C
Clin Rheumatol 2023 Oct;42(10):2601-2610. Epub 2022 Nov 17 doi: 10.1007/s10067-022-06446-y. PMID: 36396789

Recent clinical studies

Etiology

Pulmonary hypertension in connective tissue diseases: epidemiology, pathogenesis, and treatment.
Cansu DÜ, Korkmaz C
Clin Rheumatol 2023 Oct;42(10):2601-2610. Epub 2022 Nov 17 doi: 10.1007/s10067-022-06446-y. PMID: 36396789
Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis.
Hoeper MM, Dwivedi K, Pausch C, Lewis RA, Olsson KM, Huscher D, Pittrow D, Grünig E, Staehler G, Vizza CD, Gall H, Distler O, Opitz C, Gibbs JSR, Delcroix M, Park DH, Ghofrani HA, Ewert R, Kaemmerer H, Kabitz HJ, Skowasch D, Behr J, Milger K, Lange TJ, Wilkens H, Seyfarth HJ, Held M, Dumitrescu D, Tsangaris I, Vonk-Noordegraaf A, Ulrich S, Klose H, Claussen M, Eisenmann S, Schmidt KH, Swift AJ, Thompson AAR, Elliot CA, Rosenkranz S, Condliffe R, Kiely DG, Halank M
Lancet Respir Med 2022 Oct;10(10):937-948. Epub 2022 Jun 28 doi: 10.1016/S2213-2600(22)00097-2. PMID: 35777416Free PMC Article
Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry.
Hoeper MM, Pausch C, Grünig E, Klose H, Staehler G, Huscher D, Pittrow D, Olsson KM, Vizza CD, Gall H, Benjamin N, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Rosenkranz S, Ewert R, Kaemmerer H, Lange TJ, Kabitz HJ, Skowasch D, Skride A, Jureviciene E, Paleviciute E, Miliauskas S, Claussen M, Behr J, Milger K, Halank M, Wilkens H, Wirtz H, Pfeuffer-Jovic E, Harbaum L, Scholtz W, Dumitrescu D, Bruch L, Coghlan G, Neurohr C, Tsangaris I, Gorenflo M, Scelsi L, Vonk-Noordegraaf A, Ulrich S, Held M
J Heart Lung Transplant 2020 Dec;39(12):1435-1444. Epub 2020 Sep 30 doi: 10.1016/j.healun.2020.09.011. PMID: 33082079
Definitions and diagnosis of pulmonary hypertension.
Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, Khanna D, Kurzyna M, Langleben D, Manes A, Satoh T, Torres F, Wilkins MR, Badesch DB
J Am Coll Cardiol 2013 Dec 24;62(25 Suppl):D42-50. doi: 10.1016/j.jacc.2013.10.032. PMID: 24355641
Pulmonary hypertension in chronic lung diseases.
Seeger W, Adir Y, Barberà JA, Champion H, Coghlan JG, Cottin V, De Marco T, Galiè N, Ghio S, Gibbs S, Martinez FJ, Semigran MJ, Simonneau G, Wells AU, Vachiéry JL
J Am Coll Cardiol 2013 Dec 24;62(25 Suppl):D109-16. doi: 10.1016/j.jacc.2013.10.036. PMID: 24355635

Diagnosis

Systemic Lupus Erythematosus and Pulmonary Hypertension.
Parperis K, Velidakis N, Khattab E, Gkougkoudi E, Kadoglou NPE
Int J Mol Sci 2023 Mar 7;24(6) doi: 10.3390/ijms24065085. PMID: 36982160Free PMC Article
Pulmonary hypertension in connective tissue diseases: epidemiology, pathogenesis, and treatment.
Cansu DÜ, Korkmaz C
Clin Rheumatol 2023 Oct;42(10):2601-2610. Epub 2022 Nov 17 doi: 10.1007/s10067-022-06446-y. PMID: 36396789
Pulmonary hypertension in connective tissue diseases, new evidence and challenges.
Vonk MC, Vandecasteele E, van Dijk AP
Eur J Clin Invest 2021 Apr;51(4):e13453. Epub 2020 Dec 5 doi: 10.1111/eci.13453. PMID: 33216992Free PMC Article
Definitions and diagnosis of pulmonary hypertension.
Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, Khanna D, Kurzyna M, Langleben D, Manes A, Satoh T, Torres F, Wilkins MR, Badesch DB
J Am Coll Cardiol 2013 Dec 24;62(25 Suppl):D42-50. doi: 10.1016/j.jacc.2013.10.032. PMID: 24355641
Pulmonary hypertension in chronic lung diseases.
Seeger W, Adir Y, Barberà JA, Champion H, Coghlan JG, Cottin V, De Marco T, Galiè N, Ghio S, Gibbs S, Martinez FJ, Semigran MJ, Simonneau G, Wells AU, Vachiéry JL
J Am Coll Cardiol 2013 Dec 24;62(25 Suppl):D109-16. doi: 10.1016/j.jacc.2013.10.036. PMID: 24355635

Therapy

Emerging pharmacotherapies for the treatment of pulmonary arterial hypertension.
Auth R, Klinger JR
Expert Opin Investig Drugs 2023 Jul-Dec;32(11):1025-1042. Epub 2023 Nov 24 doi: 10.1080/13543784.2023.2274439. PMID: 37881882
Pulmonary hypertension in connective tissue diseases: epidemiology, pathogenesis, and treatment.
Cansu DÜ, Korkmaz C
Clin Rheumatol 2023 Oct;42(10):2601-2610. Epub 2022 Nov 17 doi: 10.1007/s10067-022-06446-y. PMID: 36396789
Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis.
Hoeper MM, Dwivedi K, Pausch C, Lewis RA, Olsson KM, Huscher D, Pittrow D, Grünig E, Staehler G, Vizza CD, Gall H, Distler O, Opitz C, Gibbs JSR, Delcroix M, Park DH, Ghofrani HA, Ewert R, Kaemmerer H, Kabitz HJ, Skowasch D, Behr J, Milger K, Lange TJ, Wilkens H, Seyfarth HJ, Held M, Dumitrescu D, Tsangaris I, Vonk-Noordegraaf A, Ulrich S, Klose H, Claussen M, Eisenmann S, Schmidt KH, Swift AJ, Thompson AAR, Elliot CA, Rosenkranz S, Condliffe R, Kiely DG, Halank M
Lancet Respir Med 2022 Oct;10(10):937-948. Epub 2022 Jun 28 doi: 10.1016/S2213-2600(22)00097-2. PMID: 35777416Free PMC Article
Chronic Thromboembolic Pulmonary Hypertension: Epidemiology, Diagnosis, and Management.
Yandrapalli S, Tariq S, Kumar J, Aronow WS, Malekan R, Frishman WH, Lanier GM
Cardiol Rev 2018 Mar/Apr;26(2):62-72. doi: 10.1097/CRD.0000000000000164. PMID: 28832374
Portopulmonary hypertension.
Mukhtar NA, Fix OK
J Clin Gastroenterol 2011 Sep;45(8):703-10. doi: 10.1097/MCG.0b013e31820656bd. PMID: 21325952

Prognosis

Systemic Lupus Erythematosus and Pulmonary Hypertension.
Parperis K, Velidakis N, Khattab E, Gkougkoudi E, Kadoglou NPE
Int J Mol Sci 2023 Mar 7;24(6) doi: 10.3390/ijms24065085. PMID: 36982160Free PMC Article
Pulmonary hypertension in connective tissue diseases: epidemiology, pathogenesis, and treatment.
Cansu DÜ, Korkmaz C
Clin Rheumatol 2023 Oct;42(10):2601-2610. Epub 2022 Nov 17 doi: 10.1007/s10067-022-06446-y. PMID: 36396789
Pulmonary hypertension in connective tissue diseases, new evidence and challenges.
Vonk MC, Vandecasteele E, van Dijk AP
Eur J Clin Invest 2021 Apr;51(4):e13453. Epub 2020 Dec 5 doi: 10.1111/eci.13453. PMID: 33216992Free PMC Article
Definitions and diagnosis of pulmonary hypertension.
Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, Khanna D, Kurzyna M, Langleben D, Manes A, Satoh T, Torres F, Wilkins MR, Badesch DB
J Am Coll Cardiol 2013 Dec 24;62(25 Suppl):D42-50. doi: 10.1016/j.jacc.2013.10.032. PMID: 24355641
Pulmonary hypertension in chronic lung diseases.
Seeger W, Adir Y, Barberà JA, Champion H, Coghlan JG, Cottin V, De Marco T, Galiè N, Ghio S, Gibbs S, Martinez FJ, Semigran MJ, Simonneau G, Wells AU, Vachiéry JL
J Am Coll Cardiol 2013 Dec 24;62(25 Suppl):D109-16. doi: 10.1016/j.jacc.2013.10.036. PMID: 24355635

Clinical prediction guides

Risk stratification and response to therapy in patients with pulmonary arterial hypertension and comorbidities: A COMPERA analysis.
Rosenkranz S, Pausch C, Coghlan JG, Huscher D, Pittrow D, Grünig E, Staehler G, Vizza CD, Gall H, Distler O, Delcroix M, Ghofrani HA, Ewert R, Kabitz HJ, Skowasch D, Behr J, Milger K, Halank M, Wilkens H, Seyfarth HJ, Held M, Scelsi L, Neurohr C, Vonk-Noordegraaf A, Ulrich S, Klose H, Claussen M, Eisenmann S, Schmidt KH, Remppis BA, Skride A, Jureviciene E, Gumbiene L, Miliauskas S, Löffler-Ragg J, Lange TJ, Olsson KM, Hoeper MM, Opitz C
J Heart Lung Transplant 2023 Jan;42(1):102-114. Epub 2022 Oct 13 doi: 10.1016/j.healun.2022.10.003. PMID: 36333206
Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis.
Hoeper MM, Dwivedi K, Pausch C, Lewis RA, Olsson KM, Huscher D, Pittrow D, Grünig E, Staehler G, Vizza CD, Gall H, Distler O, Opitz C, Gibbs JSR, Delcroix M, Park DH, Ghofrani HA, Ewert R, Kaemmerer H, Kabitz HJ, Skowasch D, Behr J, Milger K, Lange TJ, Wilkens H, Seyfarth HJ, Held M, Dumitrescu D, Tsangaris I, Vonk-Noordegraaf A, Ulrich S, Klose H, Claussen M, Eisenmann S, Schmidt KH, Swift AJ, Thompson AAR, Elliot CA, Rosenkranz S, Condliffe R, Kiely DG, Halank M
Lancet Respir Med 2022 Oct;10(10):937-948. Epub 2022 Jun 28 doi: 10.1016/S2213-2600(22)00097-2. PMID: 35777416Free PMC Article
Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry.
Hoeper MM, Pausch C, Grünig E, Klose H, Staehler G, Huscher D, Pittrow D, Olsson KM, Vizza CD, Gall H, Benjamin N, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Rosenkranz S, Ewert R, Kaemmerer H, Lange TJ, Kabitz HJ, Skowasch D, Skride A, Jureviciene E, Paleviciute E, Miliauskas S, Claussen M, Behr J, Milger K, Halank M, Wilkens H, Wirtz H, Pfeuffer-Jovic E, Harbaum L, Scholtz W, Dumitrescu D, Bruch L, Coghlan G, Neurohr C, Tsangaris I, Gorenflo M, Scelsi L, Vonk-Noordegraaf A, Ulrich S, Held M
J Heart Lung Transplant 2020 Dec;39(12):1435-1444. Epub 2020 Sep 30 doi: 10.1016/j.healun.2020.09.011. PMID: 33082079
Definitions and diagnosis of pulmonary hypertension.
Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, Khanna D, Kurzyna M, Langleben D, Manes A, Satoh T, Torres F, Wilkins MR, Badesch DB
J Am Coll Cardiol 2013 Dec 24;62(25 Suppl):D42-50. doi: 10.1016/j.jacc.2013.10.032. PMID: 24355641
Portopulmonary hypertension.
Mukhtar NA, Fix OK
J Clin Gastroenterol 2011 Sep;45(8):703-10. doi: 10.1097/MCG.0b013e31820656bd. PMID: 21325952

Recent systematic reviews

Can anticoagulants improve the survival rate for patients with idiopathic pulmonary arterial hypertension? A systematic review and meta-analysis.
Wang P, Hu L, Yin Y, Yan D, Zheng H, Zhang J, Li Y
Thromb Res 2020 Dec;196:251-256. Epub 2020 Aug 20 doi: 10.1016/j.thromres.2020.08.024. PMID: 32920295
Dual therapy in IPAH and SSc-PAH. A qualitative systematic review.
Johnson SR, Brode SK, Mielniczuk LM, Granton JT
Respir Med 2012 May;106(5):730-9. Epub 2012 Feb 25 doi: 10.1016/j.rmed.2011.12.018. PMID: 22366298
Factors that prognosticate mortality in idiopathic pulmonary arterial hypertension: a systematic review of the literature.
Swiston JR, Johnson SR, Granton JT
Respir Med 2010 Nov;104(11):1588-607. doi: 10.1016/j.rmed.2010.08.003. PMID: 20810261

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