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pulm.pulmonary_htn_group2_to_5.v1PRODUCTION
pulm.pulmonary_htn_group2_to_5.v1

Pulmonary hypertension Groups 2–5 (non-PAH)

pulmonologychronicsubacuteacuteadult
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Encounter flow

12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

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

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Set
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PH confirmed + WHO group assigned (or Group 1 routed out)

Patient inputs (17)

Group 2 (LHD) skews elderly; Group 1 younger — shifts the group prior (ESC/ERS 2022 §7)

Hypoxemia drives Group 3 and gates LTOT (ESC/ERS 2022 §8)

The primary group-classification input — LHD → 2, lung/hypoxia → 3, VTE → 4, multifactorial → 5 (ESC/ERS 2022 §5)

Drug-induced PAH red flags (methamphetamine, dasatinib, interferons); riociguat–PDE5i / nitrate contraindication; PAH-drug-misuse-in-Group-2 detection (ESC/ERS 2022 §7,§13)

ESC/ERS 2022 4-strata risk + serial RV-load monitoring (NT-proBNP <300 low / >1100 high)

Anemia worsens PH symptoms; erythrocytosis in chronic hypoxic Group 3; haemolysis screen for Group 5 (ESC/ERS 2022 §10)

Diuretic + anticoagulant + riociguat renal dosing; CKD itself a Group 5 substrate (ESC/ERS 2022 §10)

Porto-pulmonary differential (Group 1) + riociguat hepatic caution (ESC/ERS 2022 §7)

First-line PH screen: TRV, RV size/function/strain, RA area, LV diastolic dysfunction (post-cap clue), pericardial effusion (ESC/ERS 2022 §6.2)

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)

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)

Parenchymal lung disease (Group 3), PA dilation, mosaic perfusion + chronic webs/stenoses (CTEPH morphology to plan PEA/BPA) (ESC/ERS 2022 §8-9)

Group 3 obstructive/restrictive pattern; isolated very low DLCO with preserved volumes suggests PAH/CTEPH rather than parenchymal Group 3 (ESC/ERS 2022 §8)

Hemodynamic stability; RV-failure shock severity (ESC/ERS 2022 §12)

Antiphospholipid syndrome is a CTEPH risk factor AND mandates warfarin (not DOAC) — changes Group 4 regimen (ESC/ERS 2022 §9; TRAPS)

OSA / obesity-hypoventilation is a reversible Group 3 driver — treat before considering any PAH-class drug (ESC/ERS 2022 §8)

CTD-PAH / HIV-PAH are Group 1 — screen to route to the PAH sibling, not author here (ESC/ERS 2022 §7)

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (9)

9 need judgement
  • informationallife_threateningrv_failure_with_shock
    Hypotension (SBP <90 / MAP <65) + lactate >2 + echo RV dysfunction in known/suspected PH
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningpregnancy_in_established_PH
    Pregnancy (or pregnancy intent) in a patient with any significant PH (Groups 2–5)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverevq_mismatch_mandates_cteph_pathway
    Any segmental-or-larger mismatched V/Q perfusion defect in a PH work-up (with ≥3 mo therapeutic anticoagulation) — Group 4 CTEPH until proven otherwise
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverecteph_operable_pea_referral
    Group 4 CTEPH with surgically accessible (proximal) disease on CT pulmonary angiography / conventional angiography
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverecteph_inoperable_escalation
    Group 4 CTEPH with distal disease not amenable to PEA, or persistent/recurrent PH after PEA
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepah_drug_misuse_in_group_2
    Sildenafil / tadalafil / ambrisentan / macitentan / riociguat prescribed in a Group 2 (left-heart-disease) PH patient
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveresevere_hypoxemia_group_3
    PaO2 ≤55 mmHg (or ≤59 with cor pulmonale / polycythemia) or resting SpO2 ≤88% in Group 3 PH
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderategroup3_phild_increase_eligibility
    Group 3 PH due to interstitial lung disease (PH-ILD) with RHC-confirmed pre-capillary PH (mPAP >20, PCWP ≤15, PVR >2 WU) and deteriorating exercise tolerance on O2 + lung-disease therapy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatedrug_induced_pah_red_flag
    Current/past confirmed-association agent: methamphetamine, amphetamine, fenfluramine/dexfenfluramine, aminorex, dasatinib, interferons, leflunomide, mitomycin C
    Trigger could not be auto-evaluated — needs clinician judgement.

Workflow calculators

Run this disease's risk and dosing calculators inline.

RISK_STRATIFICATIONoptionalDrives risk stratification
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Recommended regimen

Group-specific therapy — treat the underlying disease (ESC/ERS 2022)
axis: ph_group_specific_underlying
Selected axis "Group-specific therapy — treat the underlying disease (ESC/ERS 2022)" by default fallback (first axis)
  • furosemide
    first line
    loop_diuretic
    20–80 mg • PO/IV • daily–BID, titrate to euvolemia
    triggers: Group2_LHD, Group3_RV_failure_volume_overload, volume_overload
    Decongestion in HFpEF/HFrEF Group-2 PH and volume-overloaded RV failure; cornerstone of Group 2 is LHD optimisation, NOT pulmonary vasodilation (ESC/ERS 2022 §7). RxCUI 4603 RxNav-verified 2026-05-16 (prior dossier carried 4337 = FENTANYL — corrected).
    rxcui 4603
  • spironolactone
    add on
    MRA
    12.5–25 mg • PO • daily
    triggers: HFrEF_with_Group2_PH, HFpEF_phenotype
    GDMT pillar for left-heart disease driving Group 2 PH (ESC/ERS 2022 §7; 2022 AHA/ACC/HFSA HF)
    rxcui 9997
  • sacubitril_valsartan
    add on
    ARNI
    24/26–49/51 mg BID, titrate • PO • BID
    triggers: HFrEF_with_Group2_PH
    PARADIGM-HF / PARAGON-HF — treating HF lowers post-capillary pulmonary pressures; the correct Group 2 lever (ESC/ERS 2022 §7)
    rxcui 1656328
  • dapagliflozin
    add on
    SGLT2i
    10 mg • PO • daily
    triggers: HFrEF_or_HFpEF_with_Group2_PH
    DAPA-HF + DELIVER — across-EF HF benefit; Group 2 PH improves with LHD control (ESC/ERS 2022 §7)
    rxcui 1488564
  • oxygen
    first line
    oxygen
    titrate to SpO2 ≥90% (resting PaO2 ≤55 or ≤59 with cor pulmonale) • inhaled • continuous ≥15 h/day
    triggers: Group3_hypoxemia_PaO2_le_55_or_SpO2_le_88
    Long-term O2 is the survival-modifying base therapy in chronic hypoxic Group 3 PH (ESC/ERS 2022 §8; NOTT/MRC)
    rxcui 7806
  • treprostinil (inhaled)
    comorbidity specific
    prostacyclin_analogue
    3 breaths (18 µg) QID, titrate up to 12 breaths (72 µg) QID • inhaled • four times daily
    triggers: Group3_PH_ILD_RHC_confirmed_precapillary, deteriorating_6MWD_on_O2_and_lung_Rx
    INCREASE (Waxman NEJM 2021 PMID 33440084): in RHC-confirmed PH-ILD, inhaled treprostinil 6MWD +31.12 m, NT-proBNP treatment-ratio 0.58, clinical-worsening HR 0.61 — the FIRST PAH-class drug with a positive Group 3 RCT. ONLY this Group 3 subset; start at a PH expert centre (V/Q-mismatch worsening risk in some Group 3). RxCUI 343048 RxNav-verified 2026-05-16.
    rxcui 343048
  • apixaban
    first line
    DOAC_factor_Xa
    5 mg BID (2.5 mg BID if ≥2 of age ≥80 / weight ≤60 kg / Cr ≥1.5) • PO • BID
    triggers: CTEPH_no_APS
    CTEPH requires LIFELONG anticoagulation; DOAC acceptable when no antiphospholipid syndrome — Worldwide CTEPH Registry (Delcroix Circulation 2024 PMID 39286890) showed no survival difference VKA vs DOAC (P=0.756). RxCUI 1364430 RxNav-verified 2026-05-16.
    rxcui 1364430
  • warfarin
    comorbidity specific
    vitamin_K_antagonist
    titrate to INR 2.0–3.0 • PO • daily
    triggers: CTEPH_with_antiphospholipid_syndrome, CTEPH_with_DOAC_intolerance
    Antiphospholipid-syndrome CTEPH MUST receive VKA (warfarin), NOT a DOAC (TRAPS — DOAC excess arterial events in triple-positive APS); also the fallback for DOAC intolerance (ESC/ERS 2022 §9). RxCUI 11289 RxNav-verified 2026-05-16.
    rxcui 11289
  • riociguat
    second line
    sGC_stimulator
    1 mg TID, titrate q2 wk to max 2.5 mg TID • PO • TID
    triggers: inoperable_CTEPH, persistent_or_recurrent_PH_after_PEA
    CHEST-1 (Ghofrani NEJM 2013 PMID 23883377): in inoperable / post-PEA persistent CTEPH, 6MWD +39 m, PVR −246 dyn·s·cm-5, NT-proBNP and WHO-FC improved — the only PAH-class drug FDA-approved for CTEPH; CHEST-2 sustained (PMID 25395036). Absolutely CONTRAINDICATED with PDE5i and nitrates. RxCUI 1439816 RxNav-verified 2026-05-16.
    rxcui 1439816
  • macitentan
    second line
    endothelin_receptor_antagonist
    10 mg • PO • once daily
    triggers: inoperable_CTEPH_persistent_symptoms_on_riociguat
    MERIT-1 (Ghofrani Lancet Respir Med 2024, RETRACTED-AND-REPUBLISHED; cite republished PMID 38548406 / DOI 10.1016/S2213-2600(24)00027-4 — NOT the retracted 2017 PMID 28919201): in inoperable CTEPH, PVR fell to a geometric-means ratio 0.84 vs placebo. Adjunct/alternative for inoperable CTEPH at expert centres. RxCUI 1442132 RxNav-verified 2026-05-16.
    rxcui 1442132
  • pulmonary endarterectomy (PEA)
    first line
    surgical_procedure
    deep-hypothermic circulatory-arrest endarterectomy at a high-volume CTEPH surgical centre • surgical • once (potentially curative)
    triggers: operable_CTEPH_surgically_accessible_disease
    PEA is the potentially CURATIVE first-choice for operable Group 4 CTEPH — Worldwide CTEPH Registry (Delcroix Circulation 2024 PMID 39286890): PEA 3-yr survival 94% vs 71% no-mechanical-intervention; perioperative management (Semin Respir Crit Care Med 2023 PMID 37487525). All CTEPH must be assessed for operability at an expert centre BEFORE committing to drug therapy (ESC/ERS 2022 §9).
  • balloon pulmonary angioplasty (BPA)
    second line
    interventional_procedure
    staged segmental/subsegmental balloon dilation over multiple sessions at a CTEPH centre • interventional • staged sessions
    triggers: inoperable_CTEPH, residual_PH_after_PEA, distal_disease_not_PEA_amenable
    BPA for technically inoperable distal CTEPH or residual post-PEA PH — Worldwide CTEPH Registry (Delcroix Circulation 2024 PMID 39286890): BPA 3-yr survival 92%, comparable to PEA and far above no-intervention 71%; often combined with riociguat (ESC/ERS 2022 §9).
  • cause-specific therapy for Group 5 substrate
    first line
    disease_specific
    treat the driver: hydroxyurea/exchange transfusion (sickle), immunosuppression (sarcoidosis), splenectomy-thrombosis surveillance, dialysis optimisation (CKD) • varies • per substrate
    triggers: Group5_multifactorial
    Group 5 PH is multifactorial/unclear-mechanism — manage the underlying disease; PAH-targeted therapy is NOT routinely indicated and is decided case-by-case at a PH expert centre only (ESC/ERS 2022 §10).
  • discontinue drug-induced-PAH agent
    first line
    deprescribing
    stop/switch with prescribing-specialty input (methamphetamine, dasatinib, interferons, leflunomide, mitomycin C, anorexigens) • n/a • one-time + echo surveillance
    triggers: confirmed_drug_induced_PAH_association
    Class-I PH drug associations (ESC/ERS 2022 §7,§13); methamphetamine cessation can partly reverse PAH — switch the offending agent with oncology/specialty input before any PAH-class drug.

outpatient playbook — drug actions (3)

  1. 1. group-specific underlying therapy
    per group axis • PO/IV/inhaled • per agent
    trigger: WHO group classified on RHC
    Group 2 → LHD GDMT; Group 3 → O2 + lung Rx; Group 4 → lifelong AC; Group 5 → cause-specific (ESC/ERS 2022 §7-10)
  2. 2. inhaled treprostinil (Group 3 PH-ILD subset only)
    3 breaths (18 µg) QID up-titrated to 12 breaths (72 µg) QID • inhaled • QID
    trigger: RHC-confirmed PH-ILD with deteriorating 6MWD on O2 + lung Rx
    INCREASE PMID 33440084 — only positive Group 3 PAH-class RCT; start at PH expert centre
  3. 3. riociguat (inoperable / post-PEA persistent CTEPH)
    1 mg TID titrated to max 2.5 mg TID • PO • TID
    trigger: Group 4 CTEPH deemed inoperable OR persistent PH after PEA
    CHEST-1 PMID 23883377; never with PDE5i/nitrates

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Unexplained exertional dyspnea / pre-syncope / RV failure signs — raised JVP, RV heave, loud P2, edema (ESC/ERS 2022 §6); Echo peak TRV >2.8 m/s or RV dysfunction → intermediate/high echo probability of PH (ESC/ERS 2022 §6.2); Prior PE/DVT with persistent dyspnea ≥3 mo on anticoagulation → suspect Group 4 CTEPH (ESC/ERS 2022 §9; route from pulm.pe.core.v1).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Pulmonary hypertension Groups 2–5 (non-PAH)** (pulm.pulmonary_htn_group2_to_5.v1).
Phenotype framing: §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
Scope: 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

No severity triggers fired against current inputs.

Plan

Regimen axis: **Group-specific therapy — treat the underlying disease (ESC/ERS 2022)**.
1. furosemide 20–80 mg PO/IV daily–BID, titrate to euvolemia (loop_diuretic, first line) — Decongestion in HFpEF/HFrEF Group-2 PH and volume-overloaded RV failure; cornerstone of Group 2 is LHD optimisation, NOT pulmonary vasodilation (ESC/ERS 2022 §7). RxCUI 4603 RxNav-verified 2026-05-16 (prior dossier carried 4337 = FENTANYL — corrected).
2. spironolactone 12.5–25 mg PO daily (MRA, add on) — GDMT pillar for left-heart disease driving Group 2 PH (ESC/ERS 2022 §7; 2022 AHA/ACC/HFSA HF)
3. sacubitril_valsartan 24/26–49/51 mg BID, titrate PO BID (ARNI, add on) — PARADIGM-HF / PARAGON-HF — treating HF lowers post-capillary pulmonary pressures; the correct Group 2 lever (ESC/ERS 2022 §7)
4. dapagliflozin 10 mg PO daily (SGLT2i, add on) — DAPA-HF + DELIVER — across-EF HF benefit; Group 2 PH improves with LHD control (ESC/ERS 2022 §7)
5. oxygen titrate to SpO2 ≥90% (resting PaO2 ≤55 or ≤59 with cor pulmonale) inhaled continuous ≥15 h/day (oxygen, first line) — Long-term O2 is the survival-modifying base therapy in chronic hypoxic Group 3 PH (ESC/ERS 2022 §8; NOTT/MRC)
6. treprostinil (inhaled) 3 breaths (18 µg) QID, titrate up to 12 breaths (72 µg) QID inhaled four times daily (prostacyclin_analogue, comorbidity specific) — INCREASE (Waxman NEJM 2021 PMID 33440084): in RHC-confirmed PH-ILD, inhaled treprostinil 6MWD +31.12 m, NT-proBNP treatment-ratio 0.58, clinical-worsening HR 0.61 — the FIRST PAH-class drug with a positive Group 3 RCT. ONLY this Group 3 subset; start at a PH expert centre (V/Q-mismatch worsening risk in some Group 3). RxCUI 343048 RxNav-verified 2026-05-16.
7. apixaban 5 mg BID (2.5 mg BID if ≥2 of age ≥80 / weight ≤60 kg / Cr ≥1.5) PO BID (DOAC_factor_Xa, first line) — CTEPH requires LIFELONG anticoagulation; DOAC acceptable when no antiphospholipid syndrome — Worldwide CTEPH Registry (Delcroix Circulation 2024 PMID 39286890) showed no survival difference VKA vs DOAC (P=0.756). RxCUI 1364430 RxNav-verified 2026-05-16.
8. warfarin titrate to INR 2.0–3.0 PO daily (vitamin_K_antagonist, comorbidity specific) — Antiphospholipid-syndrome CTEPH MUST receive VKA (warfarin), NOT a DOAC (TRAPS — DOAC excess arterial events in triple-positive APS); also the fallback for DOAC intolerance (ESC/ERS 2022 §9). RxCUI 11289 RxNav-verified 2026-05-16.
9. riociguat 1 mg TID, titrate q2 wk to max 2.5 mg TID PO TID (sGC_stimulator, second line) — CHEST-1 (Ghofrani NEJM 2013 PMID 23883377): in inoperable / post-PEA persistent CTEPH, 6MWD +39 m, PVR −246 dyn·s·cm-5, NT-proBNP and WHO-FC improved — the only PAH-class drug FDA-approved for CTEPH; CHEST-2 sustained (PMID 25395036). Absolutely CONTRAINDICATED with PDE5i and nitrates. RxCUI 1439816 RxNav-verified 2026-05-16.
10. macitentan 10 mg PO once daily (endothelin_receptor_antagonist, second line) — MERIT-1 (Ghofrani Lancet Respir Med 2024, RETRACTED-AND-REPUBLISHED; cite republished PMID 38548406 / DOI 10.1016/S2213-2600(24)00027-4 — NOT the retracted 2017 PMID 28919201): in inoperable CTEPH, PVR fell to a geometric-means ratio 0.84 vs placebo. Adjunct/alternative for inoperable CTEPH at expert centres. RxCUI 1442132 RxNav-verified 2026-05-16.
11. pulmonary endarterectomy (PEA) deep-hypothermic circulatory-arrest endarterectomy at a high-volume CTEPH surgical centre surgical once (potentially curative) (surgical_procedure, first line) — PEA is the potentially CURATIVE first-choice for operable Group 4 CTEPH — Worldwide CTEPH Registry (Delcroix Circulation 2024 PMID 39286890): PEA 3-yr survival 94% vs 71% no-mechanical-intervention; perioperative management (Semin Respir Crit Care Med 2023 PMID 37487525). All CTEPH must be assessed for operability at an expert centre BEFORE committing to drug therapy (ESC/ERS 2022 §9).
12. balloon pulmonary angioplasty (BPA) staged segmental/subsegmental balloon dilation over multiple sessions at a CTEPH centre interventional staged sessions (interventional_procedure, second line) — BPA for technically inoperable distal CTEPH or residual post-PEA PH — Worldwide CTEPH Registry (Delcroix Circulation 2024 PMID 39286890): BPA 3-yr survival 92%, comparable to PEA and far above no-intervention 71%; often combined with riociguat (ESC/ERS 2022 §9).
13. cause-specific therapy for Group 5 substrate treat the driver: hydroxyurea/exchange transfusion (sickle), immunosuppression (sarcoidosis), splenectomy-thrombosis surveillance, dialysis optimisation (CKD) varies per substrate (disease_specific, first line) — Group 5 PH is multifactorial/unclear-mechanism — manage the underlying disease; PAH-targeted therapy is NOT routinely indicated and is decided case-by-case at a PH expert centre only (ESC/ERS 2022 §10).
14. discontinue drug-induced-PAH agent stop/switch with prescribing-specialty input (methamphetamine, dasatinib, interferons, leflunomide, mitomycin C, anorexigens) n/a one-time + echo surveillance (deprescribing, first line) — Class-I PH drug associations (ESC/ERS 2022 §7,§13); methamphetamine cessation can partly reverse PAH — switch the offending agent with oncology/specialty input before any PAH-class drug.

Setting playbook (outpatient) — Confirm WHO group on RHC + mandatory V/Q, treat the underlying disease, refer all CTEPH to a PEA expert centre, AVOID PAH-targeted drugs outside the encoded Group-3/Group-4 exceptions (ESC/ERS 2022)
15. group-specific underlying therapy per group axis PO/IV/inhaled per agent — WHO group classified on RHC (Group 2 → LHD GDMT; Group 3 → O2 + lung Rx; Group 4 → lifelong AC; Group 5 → cause-specific (ESC/ERS 2022 §7-10))
16. inhaled treprostinil (Group 3 PH-ILD subset only) 3 breaths (18 µg) QID up-titrated to 12 breaths (72 µg) QID inhaled QID — RHC-confirmed PH-ILD with deteriorating 6MWD on O2 + lung Rx (INCREASE PMID 33440084 — only positive Group 3 PAH-class RCT; start at PH expert centre)
17. riociguat (inoperable / post-PEA persistent CTEPH) 1 mg TID titrated to max 2.5 mg TID PO TID — Group 4 CTEPH deemed inoperable OR persistent PH after PEA (CHEST-1 PMID 23883377; never with PDE5i/nitrates)

Non-pharmacologic actions:
- CTEPH expert-centre referral for operability assessment — ALL Group 4 (ESC/ERS 2022 §9)
- Pulmonary endarterectomy referral if operable (potentially curative; PMID 39286890)
- Balloon pulmonary angioplasty referral if inoperable/distal or residual post-PEA
- Influenza + pneumococcal + COVID vaccination (ESC/ERS 2022 §13)
- Pregnancy-avoidance counselling + effective contraception (Class-I maternal mortality) (ESC/ERS 2022 §13)
- Supervised exercise rehabilitation at an expert centre; salt restriction in Group 2

AVOID / contraindication checks:
- NO_PAH_targeted_drugs_in_Group_2_LHD — RELAX (sildenafil HFpEF, Redfield JAMA 2013 PMID 23478662; no peak VO2/6MWD benefit) and MELODY 1 (macitentan PH LHD, Vachiéry Eur Respir J 2018 PMID 29437943; EXCESS fluid retention, no hemodynamic benefit) — treating LHD is the only correct lever
- PAH_targeted_drugs_in_Group_3_only_INCREASE_subset — restrict to RHC confirmed PH ILD inhaled treprostinil (INCREASE PMID 33440084); broad PAH class use in Group 3 can worsen V/Q matching and oxygenation
- Riociguat_ABSOLUTELY_contraindicated_with_PDE5i_and_nitrates — life threatening hypotension; never co prescribe sildenafil/tadalafil or any nitrate (ESC/ERS 2022 §9; CHEST 1 PMID 23883377)
- CTEPH_must_have_operability_assessed_before_drug_therapy — PEA is potentially curative; do not commit to medical therapy without expert centre operability review (ESC/ERS 2022 §9; PMID 39286890)
- Antiphospholipid_syndrome_CTEPH_requires_warfarin_NOT_DOAC — TRAPS class signal of DOAC excess arterial thrombosis in triple positive APS
- CTEPH_anticoagulation_is_lifelong_indefinite — not time limited like provoked VTE (ESC/ERS 2022 §9)
- Riociguat_and_DOAC_renal_dose_adjustment_by_CKD_EPI_2021 (ESC/ERS 2022 §9,§13)
- Pregnancy_contraindicated_in_any_significant_PH — Class I maternal mortality warning; riociguat + ERAs are teratogenic (ESC/ERS 2022 §13)

Monitoring

Regimen monitoring:
- ESC ERS 2022 4 strata risk reassessment at 3 to 4 months then periodically (WHO FC + 6MWT + NT-proBNP + RAP + CI + SvO2)
- TTE q6 12m RV function and TRV (ESC/ERS 2022 §11)
- NT proBNP q3 6m RV load trend
- 6MWT q6m functional capacity
- INR per protocol if warfarin CTEPH or APS; renal-function-adjusted DOAC review if apixaban
- BMP q3m on loop diuretic in Group2
- post PEA residual PH reassessment by RHC at 6 to 12 months (route to riociguat/BPA if persistent — CHEST-1)
- liver function and hypotension surveillance during riociguat titration

Setting (outpatient) monitoring:
- PH clinic q3–6 mo with ESC/ERS 2022 4-strata reassessment (ESC/ERS 2022 §11)
- TTE q6–12 mo (ESC/ERS 2022 §6.2)
- NT-proBNP + 6MWT serial trend
- INR if warfarin / renal-adjusted DOAC review

Follow-up plan: 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)
- Close-out criterion: Follow-up + advanced-care pathway booked

Monitoring phase: 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)

Disposition

Current setting: outpatient — Confirm WHO group on RHC + mandatory V/Q, treat the underlying disease, refer all CTEPH to a PEA expert centre, AVOID PAH-targeted drugs outside the encoded Group-3/Group-4 exceptions (ESC/ERS 2022)

Disposition criteria:
- Stable on group-specific therapy with documented follow-up → continue chronic management (ESC/ERS 2022 §11)

Escalation triggers (move to higher acuity):
- WHO FC IV or ESC/ERS high-risk strata → admit + expert-centre transfer
- Operable CTEPH identified → expedite PEA surgical referral
- Persistent PH after PEA → riociguat / BPA escalation (CHEST-1)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Hypotension (SBP <90 / MAP <65) + lactate >2 + echo RV dysfunction in known/suspected PH
- [LIFE_THREATENING] Pregnancy (or pregnancy intent) in a patient with any significant PH (Groups 2–5)
- [SEVERE] Any segmental-or-larger mismatched V/Q perfusion defect in a PH work-up (with ≥3 mo therapeutic anticoagulation) — Group 4 CTEPH until proven otherwise

Citations

- 2022 ESC/ERS Pulmonary Hypertension Guideline (Humbert) — current as of May 2026; supplemented by 7th WSPH 2024 [PMID:36017548](https://pubmed.ncbi.nlm.nih.gov/36017548/)
- Cited evidence (PMID 23883377) [PMID:23883377](https://pubmed.ncbi.nlm.nih.gov/23883377/)
- Cited evidence (PMID 25395036) [PMID:25395036](https://pubmed.ncbi.nlm.nih.gov/25395036/)
- Cited evidence (PMID 33440084) [PMID:33440084](https://pubmed.ncbi.nlm.nih.gov/33440084/)
- Cited evidence (PMID 23478662) [PMID:23478662](https://pubmed.ncbi.nlm.nih.gov/23478662/)

Last reconciled with current guidelines: 2026-05-16.
References