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pulmonary-arterial-hypertension
Pulmonary Arterial Hypertension
PAH (WHO Group 1) is progressive obliterative pulmonary vascular disease; mPAP >20 mmHg + PVR ≥2 WU; BMPR2/BMP9 mutations in heritable PAH. Three pathways (endothelin/NO/prostacyclin) targeted by ERAs, PDE5i/sGCi, and prostacyclin analogues.
Entry Metadata
| Field | Value |
|---|---|
| ID | pulmonary-arterial-hypertension |
| Name | Pulmonary Arterial Hypertension |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
connects-toEndothelin 1
connects-toNitric Oxide
connects-toProstaglandins
targetsLung
connects-toSystemic Sclerosis
connects-toAngiopoietin
connects-toActivin A
connects-toHeart
connects-toEndothelial Cell
connects-toSmooth Muscle Cell
connects-toVenous Thromboembolism
connects-toHiv Aids
connects-toSickle Cell Disease
connects-toJuvenile Polyposis Syndrome
connects-toHeart Failure
connects-toSystemic Lupus Erythematosus
connects-toSerotonin
connects-toOxygen
connects-toCardiomyocyte
connects-toVegf
connects-toRespiratory System
connects-toCardiovascular System
connects-toLiver
connects-toTgf Beta
connects-toFibroblast
connects-toPotassium
connects-toPlatelet
connects-toHif 1Alpha
connects-toPdgf
connects-toMacrophage
connects-toKidney
connects-toCalcium
connects-toBrain
connects-toFibrosis
connects-toPhoton
connects-toEstrogen
connects-toSpleen
connects-toIron
connects-toCollagen
connects-toErythrocyte
connects-toAntibody
connects-toReproductive System
connects-toNeuron
connects-toIl 6
connects-toThyroid
connects-toMast Cell
connects-toNotch
connects-toRegulatory T Cell
connects-toCopd
connects-toNlrp3 Inflammasome
connects-toThalassemia
connects-toStimulant Use Disorder
connects-toStat3
connects-toCkd
connects-toAnemia Of Chronic Disease
connects-toMajor Depressive Disorder
connects-toStroke
connects-toSepsis
connects-toMyeloproliferative Neoplasms
connects-toCystic Fibrosis
connects-toGeneralized Anxiety Disorder
connects-toDigestive System
connects-toEndocrine System
connects-toNervous System
connects-toRenal System
connects-toImmune System
connects-toIntegumentary System
connects-toCalcium Channel Blockers
connects-toWarfarin
connects-toMusculoskeletal System
connects-toLoop Diuretics
connects-toArterial Wall
connects-toPolycythemia Vera
connects-toMyocardium
connects-toEndocardium
connects-toTuberous Sclerosis Complex
connects-toAlveolus
connects-toCardiac Conduction System
connects-toNeurofibromatosis Type 1
connects-toHepatic Lobule
connects-toCovid 19 Disease
connects-toGlomerulus
connects-toCaspase 3
connects-toAkt
connects-toBnp
connects-toTnf Alpha
connects-toIl 1B
connects-toCcl2
connects-toAdrenomedullin
connects-toAldosterone
connects-toHepcidin
connects-toSerca2A
connects-toXanthine Oxidase
connects-toVon Willebrand Factor
connects-toEpas1
connects-toSmad4
connects-toFgfr
connects-toErk1 2
connects-toMtor
connects-toE2F1
connects-toJak1 2
connects-toNf Kb
connects-toNfe2L2
connects-toGalectin 3
connects-toPten
connects-toComplement C3
connects-toCgas Sting
connects-toFoxo
connects-toStat1
connects-toGsk 3B
connects-toS100A8 A9
connects-toCdk4 6
connects-toPik3Ca
connects-toSrc Kinase
connects-toAmpk
connects-toAutophagy
connects-toCcr5
connects-toDnmt3A
connects-toCxcl12
connects-toArid1A
connects-toIl 33
connects-toSerotonin Transporter
connects-toPotassium
connects-toTroponin Complex
connects-toCgrp
connects-toAngiotensin Ii
connects-toInsulin
connects-toHemoglobin
connects-toProtein C
connects-toIl 13
connects-toIl 4
connects-toRenin
connects-toCalcium
connects-toLeptin
connects-toAdiponectin
connects-toResistin
connects-toThrombin
connects-toType I Interferon
connects-toT Helper Cell
connects-toIl 17A
connects-toIfn Gamma
connects-toIl 12
connects-toIl 5
connects-toIl 23
connects-toIge
connects-toComplement C5
connects-toC5Ar1
connects-toDendritic Cell
connects-toFactor H
connects-toTransferrin
connects-toT Cytotoxic Cell
connects-toTslp
connects-toBradykinin
connects-toErythropoietin
connects-toC1 Esterase Inhibitor
connects-toHistamine
connects-toPeriostin
connects-toMelatonin
connects-toTestosterone
connects-toProlactin
connects-toOxytocin
connects-toVasopressin
connects-toSelenium
connects-toIodine
connects-toSodium
connects-toMagnesium
connects-toCopper
connects-toPhosphorus
connects-toZinc
connects-toCarbon
connects-toChloride
connects-toHydrogen
connects-toNitrogen
connects-toSulfur
connects-toPd 1
connects-toGlp 1
connects-toWnt Beta Catenin
connects-toRankl
connects-toIl 2
connects-toFibronectin
connects-toIgf 1
connects-toCalcitonin
connects-toSubstance P
connects-toInsulin Receptor
connects-toAndrogen Receptor
connects-toNorepinephrine
connects-toAdrenomedullin
connects-toBdnf
connects-toOsteopontin
connects-toEpinephrine
connects-toMyostatin
connects-toCortisol
connects-toGhrelin
Sources
- Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2016;37(1):67-119. · PubMed 26320113
- Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53(1):1801913. · PubMed 30545968
- Sitbon O, Channick R, Chin KM, et al. Selexipag for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med. 2015;373(26):2522-2533. · PubMed 26579977