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Pulmonary hypertension due to chronic exposure to high altitude(HAPH)

MedGen UID:
83314
•Concept ID:
C0340552
•
Disease or Syndrome
Synonym: Pulmonary edema of mountaineers
SNOMED CT: High altitude pulmonary hypertension (233954004); Pulmonary hypertension caused by low air pressure due to chronic exposure to high altitude (233954004)
 
Monarch Initiative: MONDO:0008344
OMIM®: 178400

Clinical features

From HPO
Elevated pulmonary artery pressure
MedGen UID:
812247
•Concept ID:
C3805917
•
Finding
An abnormally elevated blood pressure in the circulation of the pulmonary artery.
See: Feature record | Search on this feature
Pulmonary edema
MedGen UID:
11026
•Concept ID:
C0034063
•
Pathologic Function
Fluid accumulation in the lungs.
See: Feature record | Search on this feature
Abnormality of the respiratory system
MedGen UID:
866322
•Concept ID:
C4018871
•
Anatomical Abnormality
An abnormality of the respiratory system, which include the airways, lungs, and the respiratory muscles.
See: Feature record | Search on this feature
Edema
MedGen UID:
4451
•Concept ID:
C0013604
•
Pathologic Function
An abnormal accumulation of fluid beneath the skin, or in one or more cavities of the body.
See: Feature record | Search on this feature
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Professional guidelines

PubMed

Underlying lung disease and exposure to terrestrial moderate and high altitude: personalised risk assessment.
Holthof K, Bridevaux PO, Frésard I
BMC Pulm Med 2022 May 9;22(1):187. doi: 10.1186/s12890-022-01979-z. PMID: 35534855Free PMC Article
Chronic Mountain Sickness: Clinical Aspects, Etiology, Management, and Treatment.
Villafuerte FC, Corante N
High Alt Med Biol 2016 Jun;17(2):61-9. Epub 2016 May 24 doi: 10.1089/ham.2016.0031. PMID: 27218284Free PMC Article
Interleukin-1 receptor antagonist treatment reduces pulmonary hypertension generated in rats by monocrotaline.
Voelkel NF, Tuder RM, Bridges J, Arend WP
Am J Respir Cell Mol Biol 1994 Dec;11(6):664-75. doi: 10.1165/ajrcmb.11.6.7946395. PMID: 7946395

Recent clinical studies

Etiology

Exercise Stress Echocardiography of the Right Ventricle and Pulmonary Circulation.
Gargani L, Pugliese NR, De Biase N, Mazzola M, Agoston G, Arcopinto M, Argiento P, Armstrong WF, Bandera F, Cademartiri F, Carbone A, Castaldo R, Citro R, Cocchia R, Codullo V, D'Alto M, D'Andrea A, Douschan P, Fabiani I, Ferrara F, Franzese M, Frumento P, Ghio S, Grünig E, Guazzi M, Kasprzak JD, Kolias T, Kovacs G, La Gerche A, Limogelli G, Marra AM, Matucci-Cerinic M, Mauro C, Moreo A, Pratali L, Ranieri B, Rega S, Rudski L, Saggar R, Salzano A, Serra W, Stanziola AA, Vannan MA, Voilliot D, Vriz O, Wierzbowska-Drabik K, Cittadini A, Naeije R, Bossone E; RIGHT Heart International NETwork (RIGHT-NET) Investigators
J Am Coll Cardiol 2023 Nov 21;82(21):1973-1985. doi: 10.1016/j.jacc.2023.09.807. PMID: 37968015
Can Maternal Exercise Prevent High-Altitude Pulmonary Hypertension in Children?
Leslie E, Gibson AL, Gonzalez Bosc LV, Mermier C, Wilson SM, Deyhle MR
High Alt Med Biol 2023 Mar;24(1):1-6. Epub 2023 Jan 25 doi: 10.1089/ham.2022.0098. PMID: 36695730
Pulmonary Hypertension in Acute and Chronic High Altitude Maladaptation Disorders.
Sydykov A, Mamazhakypov A, Maripov A, Kosanovic D, Weissmann N, Ghofrani HA, Sarybaev AS, Schermuly RT
Int J Environ Res Public Health 2021 Feb 10;18(4) doi: 10.3390/ijerph18041692. PMID: 33578749Free PMC Article
Chronic Mountain Sickness: Clinical Aspects, Etiology, Management, and Treatment.
Villafuerte FC, Corante N
High Alt Med Biol 2016 Jun;17(2):61-9. Epub 2016 May 24 doi: 10.1089/ham.2016.0031. PMID: 27218284Free PMC Article
Physiological adaptation of the cardiovascular system to high altitude.
Naeije R
Prog Cardiovasc Dis 2010 May-Jun;52(6):456-66. doi: 10.1016/j.pcad.2010.03.004. PMID: 20417339

Diagnosis

Altitude illnesses.
Gatterer H, Villafuerte FC, Ulrich S, Bhandari SS, Keyes LE, Burtscher M
Nat Rev Dis Primers 2024 Jun 20;10(1):43. doi: 10.1038/s41572-024-00526-w. PMID: 38902312
Can Maternal Exercise Prevent High-Altitude Pulmonary Hypertension in Children?
Leslie E, Gibson AL, Gonzalez Bosc LV, Mermier C, Wilson SM, Deyhle MR
High Alt Med Biol 2023 Mar;24(1):1-6. Epub 2023 Jan 25 doi: 10.1089/ham.2022.0098. PMID: 36695730
Chronic Mountain Sickness: Clinical Aspects, Etiology, Management, and Treatment.
Villafuerte FC, Corante N
High Alt Med Biol 2016 Jun;17(2):61-9. Epub 2016 May 24 doi: 10.1089/ham.2016.0031. PMID: 27218284Free PMC Article
Postnatal cardiopulmonary adaptations to high altitude.
Huicho L
Respir Physiol Neurobiol 2007 Sep 30;158(2-3):190-203. Epub 2007 May 10 doi: 10.1016/j.resp.2007.05.004. PMID: 17573246
Pulmonary hypertension in high-altitude dwellers: novel mechanisms, unsuspected predisposing factors.
Scherrer U, Turini P, Thalmann S, Hutter D, Salmon CS, Stuber T, Shaw S, Jayet PY, Sartori-Cucchial C, Villena M, Allemann Y, Sartori C
Adv Exp Med Biol 2006;588:277-91. doi: 10.1007/978-0-387-34817-9_23. PMID: 17089896

Therapy

The effect of high altitude (2500 m) on incremental cycling exercise in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: a randomised controlled crossover trial.
Müller J, Titz A, Schneider SR, Bauer M, Mayer L, Lüönd L, Ulrich T, Furian M, Forrer A, Schwarz EI, Bloch KE, Lichtblau M, Ulrich S
Eur Respir J 2024 Mar;63(3) Epub 2024 Mar 7 doi: 10.1183/13993003.01001-2023. PMID: 38423623Free PMC Article
Exercise Stress Echocardiography of the Right Ventricle and Pulmonary Circulation.
Gargani L, Pugliese NR, De Biase N, Mazzola M, Agoston G, Arcopinto M, Argiento P, Armstrong WF, Bandera F, Cademartiri F, Carbone A, Castaldo R, Citro R, Cocchia R, Codullo V, D'Alto M, D'Andrea A, Douschan P, Fabiani I, Ferrara F, Franzese M, Frumento P, Ghio S, Grünig E, Guazzi M, Kasprzak JD, Kolias T, Kovacs G, La Gerche A, Limogelli G, Marra AM, Matucci-Cerinic M, Mauro C, Moreo A, Pratali L, Ranieri B, Rega S, Rudski L, Saggar R, Salzano A, Serra W, Stanziola AA, Vannan MA, Voilliot D, Vriz O, Wierzbowska-Drabik K, Cittadini A, Naeije R, Bossone E; RIGHT Heart International NETwork (RIGHT-NET) Investigators
J Am Coll Cardiol 2023 Nov 21;82(21):1973-1985. doi: 10.1016/j.jacc.2023.09.807. PMID: 37968015
Clinician's Corner: Counseling Patients with Pulmonary Vascular Disease Traveling to High Altitude.
Ulrich S, Lichtblau M, Schneider SR, Saxer S, Bloch KE
High Alt Med Biol 2022 Sep;23(3):201-208. Epub 2022 Jul 18 doi: 10.1089/ham.2022.0051. PMID: 35852848
Pulmonary Vascular Pressure Response to Acute Cold Exposure in Kyrgyz Highlanders.
Sydykov A, Maripov A, Muratali Uulu K, Kushubakova N, Petrovic A, Vroom C, Cholponbaeva M, Duishobaev M, Satybaldyev S, Satieva N, Mamazhakypov A, Sartmyrzaeva M, Omurzakova N, Kerimbekova Z, Baktybek N, Pak O, Zhao L, Weissmann N, Sarybaev A, Avdeev S, Ghofrani HA, Schermuly RT, Kosanovic D
High Alt Med Biol 2019 Dec;20(4):375-382. Epub 2019 Aug 29 doi: 10.1089/ham.2019.0046. PMID: 31464532
Mechanisms and drug therapy of pulmonary hypertension at high altitude.
Scherrer U, Allemann Y, Rexhaj E, Rimoldi SF, Sartori C
High Alt Med Biol 2013 Jun;14(2):126-33. doi: 10.1089/ham.2013.1006. PMID: 23795732

Prognosis

Exercise Stress Echocardiography of the Right Ventricle and Pulmonary Circulation.
Gargani L, Pugliese NR, De Biase N, Mazzola M, Agoston G, Arcopinto M, Argiento P, Armstrong WF, Bandera F, Cademartiri F, Carbone A, Castaldo R, Citro R, Cocchia R, Codullo V, D'Alto M, D'Andrea A, Douschan P, Fabiani I, Ferrara F, Franzese M, Frumento P, Ghio S, Grünig E, Guazzi M, Kasprzak JD, Kolias T, Kovacs G, La Gerche A, Limogelli G, Marra AM, Matucci-Cerinic M, Mauro C, Moreo A, Pratali L, Ranieri B, Rega S, Rudski L, Saggar R, Salzano A, Serra W, Stanziola AA, Vannan MA, Voilliot D, Vriz O, Wierzbowska-Drabik K, Cittadini A, Naeije R, Bossone E; RIGHT Heart International NETwork (RIGHT-NET) Investigators
J Am Coll Cardiol 2023 Nov 21;82(21):1973-1985. doi: 10.1016/j.jacc.2023.09.807. PMID: 37968015
Clinician's Corner: Counseling Patients with Pulmonary Vascular Disease Traveling to High Altitude.
Ulrich S, Lichtblau M, Schneider SR, Saxer S, Bloch KE
High Alt Med Biol 2022 Sep;23(3):201-208. Epub 2022 Jul 18 doi: 10.1089/ham.2022.0051. PMID: 35852848
Underlying lung disease and exposure to terrestrial moderate and high altitude: personalised risk assessment.
Holthof K, Bridevaux PO, Frésard I
BMC Pulm Med 2022 May 9;22(1):187. doi: 10.1186/s12890-022-01979-z. PMID: 35534855Free PMC Article
The emerging role of microRNAs in hypoxia-induced pulmonary hypertension.
Mohsenin V
Sleep Breath 2016 Sep;20(3):1059-67. Epub 2016 May 7 doi: 10.1007/s11325-016-1351-y. PMID: 27154628
High altitude-induced borderline pulmonary hypertension impaired cardiorespiratory fitness in healthy young men.
Yang T, Li X, Qin J, Li S, Yu J, Zhang J, Yu S, Wu X, Huang L
Int J Cardiol 2015 Feb 15;181:382-8. Epub 2014 Dec 23 doi: 10.1016/j.ijcard.2014.12.044. PMID: 25555284

Clinical prediction guides

Exercise Stress Echocardiography of the Right Ventricle and Pulmonary Circulation.
Gargani L, Pugliese NR, De Biase N, Mazzola M, Agoston G, Arcopinto M, Argiento P, Armstrong WF, Bandera F, Cademartiri F, Carbone A, Castaldo R, Citro R, Cocchia R, Codullo V, D'Alto M, D'Andrea A, Douschan P, Fabiani I, Ferrara F, Franzese M, Frumento P, Ghio S, Grünig E, Guazzi M, Kasprzak JD, Kolias T, Kovacs G, La Gerche A, Limogelli G, Marra AM, Matucci-Cerinic M, Mauro C, Moreo A, Pratali L, Ranieri B, Rega S, Rudski L, Saggar R, Salzano A, Serra W, Stanziola AA, Vannan MA, Voilliot D, Vriz O, Wierzbowska-Drabik K, Cittadini A, Naeije R, Bossone E; RIGHT Heart International NETwork (RIGHT-NET) Investigators
J Am Coll Cardiol 2023 Nov 21;82(21):1973-1985. doi: 10.1016/j.jacc.2023.09.807. PMID: 37968015
Underlying lung disease and exposure to terrestrial moderate and high altitude: personalised risk assessment.
Holthof K, Bridevaux PO, Frésard I
BMC Pulm Med 2022 May 9;22(1):187. doi: 10.1186/s12890-022-01979-z. PMID: 35534855Free PMC Article
Pulmonary Vascular Pressure Response to Acute Cold Exposure in Kyrgyz Highlanders.
Sydykov A, Maripov A, Muratali Uulu K, Kushubakova N, Petrovic A, Vroom C, Cholponbaeva M, Duishobaev M, Satybaldyev S, Satieva N, Mamazhakypov A, Sartmyrzaeva M, Omurzakova N, Kerimbekova Z, Baktybek N, Pak O, Zhao L, Weissmann N, Sarybaev A, Avdeev S, Ghofrani HA, Schermuly RT, Kosanovic D
High Alt Med Biol 2019 Dec;20(4):375-382. Epub 2019 Aug 29 doi: 10.1089/ham.2019.0046. PMID: 31464532
Acclimatization to chronic intermittent hypoxia in mine workers: a challenge to mountain medicine in Chile.
Farias JG, Jimenez D, Osorio J, Zepeda AB, Figueroa CA, Pulgar VM
Biol Res 2013;46(1):59-67. doi: 10.4067/S0716-97602013000100009. PMID: 23760416
High altitude pulmonary hypertension: role of K+ and Ca2+ channels.
Remillard CV, Yuan JX
High Alt Med Biol 2005 Summer;6(2):133-46. doi: 10.1089/ham.2005.6.133. PMID: 16060848Free PMC Article

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