Atlas One · Host Biology · Scale 07 — System

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.

Also known as: PAH, Group 1 pulmonary hypertension, pulmonary hypertension, idiopathic PAH, IPAH, heritable PAH, HPAH, connective tissue disease PAH, CTD-PAH

Scale 07 — SystemScale
draftStatus
2026-06-07Last Reviewed
This is an auto-generated stub page built from atlas entry frontmatter. A richer hand-crafted page is planned. See the atlas source for the full entry including detailed body sections.
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

FieldValue
IDpulmonary-arterial-hypertension
NamePulmonary Arterial Hypertension
Statusdraft
Last reviewed2026-06-07
Atlas01-human
Scale07-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