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Pulmonary hypertension

MedGen UID:
9376
Concept ID:
C0020542
Finding; Pathologic Function
Synonyms: Hypertension, Pulmonary; Pulmonary Hypertension
SNOMED CT: Pulmonary hypertension (70995007); PHT - Pulmonary hypertension (70995007)
 
Monarch Initiative: MONDO:0005149

Definition

Increased pressure within the pulmonary circulation due to lung or heart disorder. [from NCI]

Professional guidelines

PubMed

Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, Carlsen J, Coats AJS, Escribano-Subias P, Ferrari P, Ferreira DS, Ghofrani HA, Giannakoulas G, Kiely DG, Mayer E, Meszaros G, Nagavci B, Olsson KM, Pepke-Zaba J, Quint JK, Rådegran G, Simonneau G, Sitbon O, Tonia T, Toshner M, Vachiery JL, Vonk Noordegraaf A, Delcroix M, Rosenkranz S; ESC/ERS Scientific Document Group
Eur Heart J 2022 Oct 11;43(38):3618-3731. doi: 10.1093/eurheartj/ehac237. PMID: 36017548
Ruopp NF, Cockrill BA
JAMA 2022 Apr 12;327(14):1379-1391. doi: 10.1001/jama.2022.4402. PMID: 35412560
Thenappan T, Ormiston ML, Ryan JJ, Archer SL
BMJ 2018 Mar 14;360:j5492. doi: 10.1136/bmj.j5492. PMID: 29540357Free PMC Article

Recent clinical studies

Etiology

Mocumbi A, Humbert M, Saxena A, Jing ZC, Sliwa K, Thienemann F, Archer SL, Stewart S
Nat Rev Dis Primers 2024 Jan 4;10(1):1. doi: 10.1038/s41572-023-00486-7. PMID: 38177157
Yang J, Madani MM, Mahmud E, Kim NH
Chest 2023 Aug;164(2):490-502. Epub 2023 Mar 28 doi: 10.1016/j.chest.2023.03.029. PMID: 36990148Free PMC Article
Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, Carlsen J, Coats AJS, Escribano-Subias P, Ferrari P, Ferreira DS, Ghofrani HA, Giannakoulas G, Kiely DG, Mayer E, Meszaros G, Nagavci B, Olsson KM, Pepke-Zaba J, Quint JK, Rådegran G, Simonneau G, Sitbon O, Tonia T, Toshner M, Vachiery JL, Vonk Noordegraaf A, Delcroix M, Rosenkranz S; ESC/ERS Scientific Document Group
Eur Heart J 2022 Oct 11;43(38):3618-3731. doi: 10.1093/eurheartj/ehac237. PMID: 36017548
Poch D, Mandel J
Ann Intern Med 2021 Apr;174(4):ITC49-ITC64. Epub 2021 Apr 13 doi: 10.7326/AITC202104200. PMID: 33844574
Hoeper MM, Humbert M, Souza R, Idrees M, Kawut SM, Sliwa-Hahnle K, Jing ZC, Gibbs JS
Lancet Respir Med 2016 Apr;4(4):306-22. Epub 2016 Mar 12 doi: 10.1016/S2213-2600(15)00543-3. PMID: 26975810

Diagnosis

Mocumbi A, Humbert M, Saxena A, Jing ZC, Sliwa K, Thienemann F, Archer SL, Stewart S
Nat Rev Dis Primers 2024 Jan 4;10(1):1. doi: 10.1038/s41572-023-00486-7. PMID: 38177157
Poch D, Mandel J
Ann Intern Med 2021 Apr;174(4):ITC49-ITC64. Epub 2021 Apr 13 doi: 10.7326/AITC202104200. PMID: 33844574
Kim D, George MP
Med Clin North Am 2019 May;103(3):413-423. doi: 10.1016/j.mcna.2018.12.002. PMID: 30955510
Mullin CJ, Klinger JR
Heart Fail Clin 2018 Jul;14(3):339-351. doi: 10.1016/j.hfc.2018.02.009. PMID: 29966632
Hoeper MM, Ghofrani HA, Grünig E, Klose H, Olschewski H, Rosenkranz S
Dtsch Arztebl Int 2017 Feb 3;114(5):73-84. doi: 10.3238/arztebl.2017.0073. PMID: 28241922Free PMC Article

Therapy

Shlobin OA, Adir Y, Barbera JA, Cottin V, Harari S, Jutant EM, Pepke-Zaba J, Ghofrani HA, Channick R
Eur Respir J 2024 Oct;64(4) Epub 2024 Oct 31 doi: 10.1183/13993003.01200-2024. PMID: 39209469Free PMC Article
Weatherald J, Boucly A, Peters A, Montani D, Prasad K, Psotka MA, Zannad F, Gomberg-Maitland M, McLaughlin V, Simonneau G, Humbert M; Evolving Landscape of Pulmonary Arterial Hypertension and Redesigning Pulmonary Arterial Hypertension Clinical Trials Task Force of the 18th Global CardioVascular Clinical Trialists Forum
Lancet 2022 Nov 26;400(10366):1884-1898. doi: 10.1016/S0140-6736(22)01601-4. PMID: 36436527
Gupta R, Baughman RP
Semin Respir Crit Care Med 2020 Oct;41(5):700-715. Epub 2020 Aug 10 doi: 10.1055/s-0040-1709495. PMID: 32777853
Vachiéry JL, Tedford RJ, Rosenkranz S, Palazzini M, Lang I, Guazzi M, Coghlan G, Chazova I, De Marco T
Eur Respir J 2019 Jan;53(1) Epub 2019 Jan 24 doi: 10.1183/13993003.01897-2018. PMID: 30545974Free PMC Article
Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, Heimbach JK, Ramsay MA, Sitbon O, Sokol RJ
Transplantation 2016 Jul;100(7):1440-52. doi: 10.1097/TP.0000000000001229. PMID: 27326810

Prognosis

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
Farber HW, Miller DP, Poms AD, Badesch DB, Frost AE, Muros-Le Rouzic E, Romero AJ, Benton WW, Elliott CG, McGoon MD, Benza RL
Chest 2015 Oct;148(4):1043-54. doi: 10.1378/chest.15-0300. PMID: 26066077
Sharifi M, Bay C, Skrocki L, Rahimi F, Mehdipour M; “MOPETT” Investigators
Am J Cardiol 2013 Jan 15;111(2):273-7. Epub 2012 Oct 24 doi: 10.1016/j.amjcard.2012.09.027. PMID: 23102885
Dennis CM, McNeil KD, Dunning J, Stewart S, Friend PJ, Alexander G, Higenbottam TW, Calne RY, Wallwork J
J Heart Lung Transplant 1996 May;15(5):536-8. PMID: 8771510
D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Kernis JT
Ann Intern Med 1991 Sep 1;115(5):343-9. doi: 10.7326/0003-4819-115-5-343. PMID: 1863023

Clinical prediction guides

Raimondi F, Migliaro F, Corsini I, Meneghin F, Dolce P, Pierri L, Perri A, Aversa S, Nobile S, Lama S, Varano S, Savoia M, Gatto S, Leonardi V, Capasso L, Carnielli VP, Mosca F, Dani C, Vento G, Lista G
Pediatrics 2021 Apr;147(4) Epub 2021 Mar 9 doi: 10.1542/peds.2020-030528. PMID: 33688032
Chatterjee D, Ing RJ, Gien J
Anesth Analg 2020 Sep;131(3):808-821. doi: 10.1213/ANE.0000000000004324. PMID: 31335403
Guazzi M, Bandera F, Ozemek C, Systrom D, Arena R
J Am Coll Cardiol 2017 Sep 26;70(13):1618-1636. doi: 10.1016/j.jacc.2017.08.012. PMID: 28935040
Otto CM
Heart 2016 Mar;102(5):333-4. doi: 10.1136/heartjnl-2015-309220. PMID: 26869632
McLaughlin VV, Davis M, Cornwell W
Curr Probl Cardiol 2011 Dec;36(12):461-517. doi: 10.1016/j.cpcardiol.2011.08.002. PMID: 22035608

Recent systematic reviews

Morris NR, Kermeen FD, Jones AW, Lee JY, Holland AE
Cochrane Database Syst Rev 2023 Mar 22;3(3):CD011285. doi: 10.1002/14651858.CD011285.pub3. PMID: 36947725Free PMC Article
Desaunay P, Eude LG, Dreyfus M, Alexandre C, Fedrizzi S, Alexandre J, Uguz F, Guénolé F
Paediatr Drugs 2023 May;25(3):247-265. Epub 2023 Feb 28 doi: 10.1007/s40272-023-00561-2. PMID: 36853497
Araiza-Garaygordobil D, García-Martínez CE, Burgos LM, Saldarriaga C, Liblik K, Mendoza I, Martinez-Selles M, Scatularo CE, Farina JM, Baranchuk A; Neglected Tropical Diseases and other Infectious Diseases affecting the Heart (the NET‐Heart) project
Cardiovasc J Afr 2021 Sep-Oct 23;32(5):276-283. Epub 2021 Jul 20 doi: 10.5830/CVJA-2021-033. PMID: 34292294Free PMC Article
Fields JM, Davis J, Girson L, Au A, Potts J, Morgan CJ, Vetter I, Riesenberg LA
J Am Soc Echocardiogr 2017 Jul;30(7):714-723.e4. Epub 2017 May 9 doi: 10.1016/j.echo.2017.03.004. PMID: 28495379
Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y, Distler O, Clements P, Cutolo M, Czirjak L, Damjanov N, Del Galdo F, Denton CP, Distler JHW, Foeldvari I, Figelstone K, Frerix M, Furst DE, Guiducci S, Hunzelmann N, Khanna D, Matucci-Cerinic M, Herrick AL, van den Hoogen F, van Laar JM, Riemekasten G, Silver R, Smith V, Sulli A, Tarner I, Tyndall A, Welling J, Wigley F, Valentini G, Walker UA, Zulian F, Müller-Ladner U; EUSTAR Coauthors
Ann Rheum Dis 2017 Aug;76(8):1327-1339. Epub 2016 Nov 9 doi: 10.1136/annrheumdis-2016-209909. PMID: 27941129

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