pulm.pulmonary_htn_group2_to_5.v1

Pulmonary hypertension Groups 2–5 (non-PAH)

pulmonologychronicsubacuteacuteadultoutpatientacuteinpatienttransition

Start encounter →Print handoutPRODUCTION

Non-PAH PH dossier — WHO Groups 2/3/4/5; the cornerstone, encoded as data, is treating the underlying disease per 2022 ESC/ERS (current May 2026, WebSearch-verified; supplemented by 7th WSPH 2024). Group 1 PAH is OUT OF SCOPE and owned by the sibling cardio.idiopathic-pulmonary-arterial-hypertension.v1 — route there; sotatercept (STELLAR) is Group 1 only. Group 2 (LHD): NO PAH-targeted drugs — RELAX (sildenafil, PMID 23478662) and MELODY-1 (macitentan, PMID 29437943) show futility/harm; treat the left heart. Group 3: O2 + lung-disease therapy; inhaled treprostinil ONLY for RHC-confirmed PH-ILD (INCREASE PMID 33440084, 6MWD +31.12 m). Group 4 CTEPH: V/Q scan MANDATORY (sens 96-97% vs CTPA 51%, Tunariu PMID 17475953); lifelong anticoagulation; PEA potentially curative (3-yr survival 94%, PMID 39286890); riociguat (CHEST-1 PMID 23883377)/BPA/macitentan (MERIT-1 republished PMID 38548406) if inoperable; APS → warfarin not DOAC. CTEPH is referred FROM pulm.pe.core.v1. Group 5: cause-specific; PAH-targeted therapy not routinely indicated. PMID audit (2026-05-16): 3 corrupt PMIDs from the prior depth payload CULLED — 37018461 (trout genetics → replaced by STELLAR 36877098), 23448530 (sinonasal sarcoma → replaced by RELAX 23478662), 32437315 (drug-design editorial → replaced by MELODY-1 29437943); MERIT-1 2017 PMID 28919201 RETRACTED → using republished 38548406. RxCUI fix: furosemide corrected 4337→4603 (4337 resolved to FENTANYL on RxNav 2026-05-16); treprostinil 343048, apixaban 1364430, warfarin 11289, riociguat 1439816, macitentan 1442132 all RxNav-verified. Schema-gap log (see _briefs/...depth.md): no PH/CTEPH-specific workup or calculator exists in clinical-tools-registry.ts (reused acute_pulm_edema/copd_exacerbation/fuo + calc.reveal_lite as a Group-1 comparator); WHO FC and ESC/ERS 2022 4-strata are header-documented (no structured severity_classification field on EngineDossier); operability/PEA + BPA encoded as non_pharm regimen entries (no procedure schema). Open: manifest/problem-package/atoms unbuilt.

Entry points (5)

symptom
Unexplained exertional dyspnea / pre-syncope / RV failure signs — raised JVP, RV heave, loud P2, edema (ESC/ERS 2022 §6)
unexplained_dyspnea_or_RV_failure
imaging
Echo peak TRV >2.8 m/s or RV dysfunction → intermediate/high echo probability of PH (ESC/ERS 2022 §6.2)
echo_TRV_gt_2_8_or_RV_dysfunction
history
Prior PE/DVT with persistent dyspnea ≥3 mo on anticoagulation → suspect Group 4 CTEPH (ESC/ERS 2022 §9; route from pulm.pe.core.v1)
prior_pe_or_DVT_with_persistent_dyspnea
history
HFpEF / HFrEF / valvular OR advanced COPD/ILD with PH features → Group 2 vs 3 (ESC/ERS 2022 §7-8)
left_heart_disease_or_lung_disease
history
Sarcoidosis / sickle cell / chronic haemolysis / CKD on dialysis / metabolic with PH → Group 5 (ESC/ERS 2022 §10)
multifactorial_disease

Required inputs (17)

agerequired
demographic • used at CONTEXT
Group 2 (LHD) skews elderly; Group 1 younger — shifts the group prior (ESC/ERS 2022 §7)
spo2required
vital • used at CONTEXT
Hypoxemia drives Group 3 and gates LTOT (ESC/ERS 2022 §8)
sbprequired
vital • used at RED_FLAGS
Hemodynamic stability; RV-failure shock severity (ESC/ERS 2022 §12)
bnp_ntprobnprequired
lab • used at INITIAL_WORKUP
ESC/ERS 2022 4-strata risk + serial RV-load monitoring (NT-proBNP <300 low / >1100 high)
cbcrequired
lab • used at INITIAL_WORKUP
Anemia worsens PH symptoms; erythrocytosis in chronic hypoxic Group 3; haemolysis screen for Group 5 (ESC/ERS 2022 §10)
creatinine_egfrrequired
lab • used at INITIAL_WORKUP
Diuretic + anticoagulant + riociguat renal dosing; CKD itself a Group 5 substrate (ESC/ERS 2022 §10)
liver_panelrequired
lab • used at INITIAL_WORKUP
Porto-pulmonary differential (Group 1) + riociguat hepatic caution (ESC/ERS 2022 §7)
hiv_anti_centromere_anti_scl70_ana
lab • used at INITIAL_WORKUP
CTD-PAH / HIV-PAH are Group 1 — screen to route to the PAH sibling, not author here (ESC/ERS 2022 §7)
thrombophilia_panel
lab • used at BRANCHING_WORKUP
Antiphospholipid syndrome is a CTEPH risk factor AND mandates warfarin (not DOAC) — changes Group 4 regimen (ESC/ERS 2022 §9; TRAPS)
tterequired
imaging • used at INITIAL_WORKUP
First-line PH screen: TRV, RV size/function/strain, RA area, LV diastolic dysfunction (post-cap clue), pericardial effusion (ESC/ERS 2022 §6.2)
right_heart_cathrequired
imaging • used at INITIAL_WORKUP
DEFINITIVE diagnosis + group assignment: mPAP >20, PVR >2 WU, PCWP ≤15 (pre-cap) vs >15 (post-cap), vasoreactivity if Group-1 suspected (ESC/ERS 2022 §6.3)
vq_scanrequired
imaging • used at INITIAL_WORKUP
MANDATORY to exclude Group 4 CTEPH — V/Q sensitivity 96-97% vs CTPA 51% (Tunariu J Nucl Med 2007 PMID 17475953); CT/CTPA CANNOT replace it (ESC/ERS 2022 §9 Class I)
ct_chest_pa_protocolrequired
imaging • used at INITIAL_WORKUP
Parenchymal lung disease (Group 3), PA dilation, mosaic perfusion + chronic webs/stenoses (CTEPH morphology to plan PEA/BPA) (ESC/ERS 2022 §8-9)
pft_dlcorequired
imaging • used at INITIAL_WORKUP
Group 3 obstructive/restrictive pattern; isolated very low DLCO with preserved volumes suggests PAH/CTEPH rather than parenchymal Group 3 (ESC/ERS 2022 §8)
sleep_study
imaging • used at BRANCHING_WORKUP
OSA / obesity-hypoventilation is a reversible Group 3 driver — treat before considering any PAH-class drug (ESC/ERS 2022 §8)
lhd_lung_vte_historyrequired
history • used at CONTEXT
The primary group-classification input — LHD → 2, lung/hypoxia → 3, VTE → 4, multifactorial → 5 (ESC/ERS 2022 §5)
current_medsrequired
medication • used at CONTEXT
Drug-induced PAH red flags (methamphetamine, dasatinib, interferons); riociguat–PDE5i / nitrate contraindication; PAH-drug-misuse-in-Group-2 detection (ESC/ERS 2022 §7,§13)

12-phase flow (12)

1FRAME
Scope = adult non-PAH PH (Groups 2/3/4/5). Confirm PH on RHC (mPAP >20, PVR >2 WU per ESC/ERS 2022) and assign group; Group 1 PAH is OUT OF SCOPE → route to cardio.idiopathic-pulmonary-arterial-hypertension.v1
inputs: right_heart_cath
advance: PH confirmed + WHO group assigned (or Group 1 routed out)
2ENTRY
Exertional dyspnea / pre-syncope / RV-failure signs / incidental echo TRV >2.8 m/s or prior PE with persistent dyspnea (ESC/ERS 2022 §6)
inputs: age
advance: Entry trigger identified
3CONTEXT
Left-heart disease, lung disease/hypoxia, VTE history, sickle/sarcoid/CKD, drug-induced-PAH agents, current meds (riociguat interaction screen) (ESC/ERS 2022 §5,§7-10,§13)
inputs: lhd_lung_vte_history, current_meds, age, spo2
advance: Substrate + drug history complete
4RED_FLAGS
RV-failure cardiogenic shock, exertional syncope, severe hypoxemia, acute pulmonary edema (Group 2 decompensation) (ESC/ERS 2022 §12)
inputs: sbp, spo2
actions: acute_pulm_edema
advance: Stabilised or ICU pathway initiated
5INITIAL_WORKUP
TTE → V/Q scan (MANDATORY CTEPH rule-out, NOT CTPA) → CT chest + PFT/DLCO + ABG → BNP/NT-proBNP, CBC, renal, LFT → RHC (mPAP/PVR/PCWP, pre vs post-capillary) (ESC/ERS 2022 §6,§9; Tunariu 2007)
inputs: tte, vq_scan, ct_chest_pa_protocol, pft_dlco, bnp_ntprobnp, cbc, creatinine_egfr, liver_panel, right_heart_cath
actions: panel.cardiac, panel.cbc, panel.lft, panel.renal, panel.abg
advance: RHC + V/Q + lung phenotyping returned
6BRANCHING_WORKUP
V/Q-positive → CTEPH expert-centre referral + thrombophilia/APS + CT pulmonary angiography for operability mapping; HFpEF context → coronary angio/diastolic stress; OSA → polysomnography; CTD/HIV/hepatic → Group-1 route-out screen (ESC/ERS 2022 §7-9)
inputs: sleep_study, thrombophilia_panel, hiv_anti_centromere_anti_scl70_ana
actions: fuo
advance: Branch resolved + group substrate confirmed
7DIFFERENTIAL
§5.5.2 group discrimination as data — Group 1 PAH (pre-cap, V/Q normal, no LHD/lung/VTE → route to PAH sibling) vs Group 2 LHD (PCWP >15, post/combined-cap, LV diastolic dysfunction, common) vs Group 3 lung/hypoxia (pre-cap + parenchymal disease/DLCO↓ + hypoxemia) vs Group 4 CTEPH (pre-cap + ≥1 segmental V/Q mismatch + ≥3 mo AC — MUST NOT MISS, potentially curable) vs Group 5 multifactorial. Pivots: PCWP ≤15 vs >15; V/Q sens 96-97% (Tunariu 2007); echo TRV/RV; DLCO pattern
inputs: right_heart_cath, vq_scan, pft_dlco
advance: Group locked + Group 1/CTEPH explicitly excluded or routed
8RISK_STRATIFICATION
Underlying-disease severity is primary; ESC/ERS 2022 4-strata (WHO FC, 6MWT, NT-proBNP, RAP, CI, SvO2) as comparator/severity anchor; CTEPH operability assessment at PEA centre (ESC/ERS 2022 §9,§11)
inputs: right_heart_cath, bnp_ntprobnp
actions: calc.reveal_lite, calc.mmrc
advance: Severity tier + CTEPH operability documented
9TREATMENT
Group 2 → optimise LHD/GDMT/valve, NO PAH-targeted drugs (RELAX/MELODY-1 harm). Group 3 → O2 + treat COPD/ILD/OSA + selected inhaled treprostinil for RHC-confirmed PH-ILD (INCREASE). Group 4 CTEPH → lifelong AC + PEA if operable (curative) ELSE BPA + riociguat (CHEST-1)/macitentan (MERIT-1). Group 5 → cause-specific (ESC/ERS 2022 §7-10)
inputs: creatinine_egfr, right_heart_cath
advance: Group-specific plan + contraindication screen documented
10DISPOSITION
Admit if RV-failure/shock/pulmonary-edema/syncope; otherwise outpatient PH expert centre; CTEPH → PEA-surgical-centre referral (ESC/ERS 2022 §11-12)
advance: Disposition + referral set
11MONITORING
ESC/ERS 2022 risk re-stratification at 3-4 mo + serial: WHO FC, 6MWT, NT-proBNP, TTE q6-12 mo; INR if warfarin (CTEPH/APS); residual PH post-PEA reassessment by RHC (ESC/ERS 2022 §11)
inputs: bnp_ntprobnp
advance: Risk-based monitoring schedule documented
12FOLLOWUP
Pulm + cardiology q3-6 mo; CTEPH expert centre lifelong; advanced/refractory → lung transplant evaluation + parallel palliative care; vaccination + pregnancy-avoidance counselling (ESC/ERS 2022 §11,§13)
advance: Follow-up + advanced-care pathway booked