Pulmonary hypertension due to left heart disease (Group 2 PH, cross-system)
Encounter flow
12/12 authoredCanonical 12-phase frame with authored status for this dossier.
Frame
PH suspected with a left-heart driver — frame as Group 2 (not Group 1/3/4)
left-heart driver + Group-2 hypothesis framed
Patient inputs (10)
HFpEF/Group-2 prevalence; transplant candidacy
Detect inappropriate PAH-specific therapy in Group 2 PH
HFrEF vs HFpEF/HFmrEF vs valvular vs LA disease — the driver to treat
PAWP >15 mmHg defines post-capillary (Group 2) PH
PVR ≤2 WU (Ipc-PH) vs >2 WU (Cpc-PH) — phenotype + prognosis
Functional class; transplant evaluation
HF GDMT dosing; RHC contrast
Comorbid lung disease → combined Group 2+3 PH
RV strain/dysfunction — severity + transplant implications
LHD severity + monitoring; supports Group 2 over Group 1
* = hard-required. Engine cannot meaningfully run until these are filled.
Severity triggers (9)
- informationalsevereno_pah_drugs_in_group2Group 2 PH — PAH-specific therapy (ERA/PDE5i/prostacyclin/riociguat) is NOT indicated and is HARMFUL (pulmonary edema); deprescribe if started, treat the LHD instead — 2022 ESC/ERS PHTrigger could not be auto-evaluated — needs clinician judgement.
- informationalseverecpc_ph_branchCombined post-/pre-capillary PH (PAWP >15 + PVR >2 WU) — worse prognosis; optimise LHD then PH-expert centre (PAH drugs only in trial/specialist context) — 2022 ESC/ERS PHTrigger could not be auto-evaluated — needs clinician judgement.
- informationalseverevalvular_group2_branchSevere AS/MS/MR-driven Group 2 PH — valve intervention often substantially reverses PH — 2020 ACC/AHA VHDTrigger could not be auto-evaluated — needs clinician judgement.
- informationalseveretransplant_pvr_branchHeart-transplant evaluation with elevated PVR — vasoreactivity testing; fixed high PVR may require combined strategy / contraindicate isolated heart transplant — 2022 ESC/ERS PHTrigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderatehfpef_group2_branchHFpEF/HFmrEF-driven Group 2 PH — treat HFpEF (SGLT2i + finerenone; comorbidity-directed); commonest contemporary Group 2 cause — 2022 ACC/AHA HFTrigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderatehfref_group2_branchHFrEF-driven Group 2 PH — 4-pillar GDMT lowers filling pressures and post-capillary PH — 2022 ACC/AHA HFTrigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderatecombined_group2_3_branchHFpEF + significant lung disease (combined Group 2+3 PH) — co-manage both; avoid attributing solely to one; PAH drugs still not routinely indicated — 2022 ESC/ERS PHTrigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderatepvr_reversibility_branchReassess PVR/PASP after LHD optimisation — substantial reversibility is expected; persistent elevation despite optimised LHD prompts re-phenotyping — 2022 ESC/ERS PHTrigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderatesecondary_tr_branchChronic Group 2 PH causing secondary tricuspid regurgitation / RV failure — co-manage TR + RV — 2020 ACC/AHA VHDTrigger could not be auto-evaluated — needs clinician judgement.
Workflow calculators
Run this disease's risk and dosing calculators inline.
Recommended regimen
Group 2 PH — treat the left heart disease, NO PAH-specific drugs (2022 ESC/ERS PH; 2022 AHA/ACC/HFSA HF)- do NOT initiate PAH-specific therapy (ERA/PDE5i/prostacyclin/riociguat) in Group 2 PHfirst linesafety_guardrailtriggers: group2_ph_confirmed_or_suspected2022 ESC/ERS PH — PAH drugs in Group 2 PH are not beneficial and can cause pulmonary edema/harm; deprescribe if inappropriately started
outpatient playbook — drug actions (3)
- 1. stop/avoid PAH-specific drugsn/a • n/a • n/atrigger: Group 2 PH (2022 ESC/ERS PH)PAH drugs are harmful here
- 2. phenotype-specific HF GDMT + decongestionper HF phenotype • PO • per drugtrigger: HF-driven Group 2 PH (2022 ACC/AHA HF)Lower filling pressures = treat the PH
- 3. valve intervention if valvular driverprocedure • surgical/transcatheter • n/atrigger: Severe left-sided valve disease (2020 ACC/AHA VHD)Often reverses Group 2 PH
Auto-drafted A&P note
outpatientSubjective
- Possible entry pathways: Echo: elevated PASP/RV strain with HFrEF/HFpEF/valve/LA disease; Worsening dyspnea in known left heart disease; Rising NT-proBNP with PH features + left heart disease.
Objective
- No vitals, labs, or imaging entered for this encounter.
Assessment
**Pulmonary hypertension due to left heart disease (Group 2 PH, cross-system)** (cardio.cardio-pulmonary.group2-ph.chronic.v1). Phenotype framing: Group 2 vs Group 1/3/4; Ipc-PH vs Cpc-PH; combined Group 2+3 Scope: PH suspected with a left-heart driver — frame as Group 2 (not Group 1/3/4) No severity triggers fired against current inputs.
Plan
Regimen axis: **Group 2 PH — treat the left heart disease, NO PAH-specific drugs (2022 ESC/ERS PH; 2022 AHA/ACC/HFSA HF)** — step "Step 1 — Confirm post-capillary (Group 2) + phenotype Ipc vs Cpc; enforce no-PAH-drug guardrail". 1. do NOT initiate PAH-specific therapy (ERA/PDE5i/prostacyclin/riociguat) in Group 2 PH (safety_guardrail, first line) — 2022 ESC/ERS PH — PAH drugs in Group 2 PH are not beneficial and can cause pulmonary edema/harm; deprescribe if inappropriately started Setting playbook (outpatient) — Confirm Group 2 (RHC Ipc/Cpc), treat the LHD, enforce no-PAH-drug guardrail, reassess PVR reversibility (2022 ESC/ERS PH; 2022 ACC/AHA HF) 2. stop/avoid PAH-specific drugs n/a n/a n/a — Group 2 PH (2022 ESC/ERS PH) (PAH drugs are harmful here) 3. phenotype-specific HF GDMT + decongestion per HF phenotype PO per drug — HF-driven Group 2 PH (2022 ACC/AHA HF) (Lower filling pressures = treat the PH) 4. valve intervention if valvular driver procedure surgical/transcatheter n/a — Severe left-sided valve disease (2020 ACC/AHA VHD) (Often reverses Group 2 PH) Non-pharmacologic actions: - HF/valve clinic management of the driver — 2022 ACC/AHA HF - PH-expert-centre referral for persistent Cpc-PH / transplant PVR assessment — 2022 ESC/ERS PH - Combined Group 2+3 → co-manage lung disease — 2022 ESC/ERS PH AVOID / contraindication checks: - PAH specific drugs NOT indicated and HARMFUL in Group 2 PH — 2022 ESC/ERS PH - Treat the left heart disease this is the Group 2 PH therapy — 2022 ESC/ERS PH - RHC required to distinguish Ipc vs Cpc and exclude Group 1 3 4 — 2022 ESC/ERS PH - Fixed elevated PVR affects heart transplant candidacy assess reversibility — 2022 ESC/ERS PH - Cpc PH PAH drugs only in specialist trial context — 2022 ESC/ERS PH
Monitoring
Regimen monitoring: - reassess PASP PVR and symptoms after LHD optimisation — 2022 ESC/ERS PH - NT-proBNP and congestion for LHD control — 2022 ACC/AHA HF - RV function serial echo — 2022 ESC/ERS PH - transplant PVR vasoreactivity if evaluation — 2022 ESC/ERS PH Setting (outpatient) monitoring: - Reassess PASP/PVR/symptoms after LHD optimisation — 2022 ESC/ERS PH Follow-up plan: Transplant candidacy reassessment; re-phenotype if PH persists/worsens despite LHD optimisation - Close-out criterion: long-term plan documented Monitoring phase: Reassess PVR/PASP + symptoms after LHD optimisation (reversibility); RV function
Disposition
Current setting: outpatient — Confirm Group 2 (RHC Ipc/Cpc), treat the LHD, enforce no-PAH-drug guardrail, reassess PVR reversibility (2022 ESC/ERS PH; 2022 ACC/AHA HF) Disposition criteria: - Ipc-PH → treat LHD, routine HF/valve follow-up - Cpc-PH → optimise LHD + PH-expert centre - Transplant candidate → PVR vasoreactivity assessment Escalation triggers (move to higher acuity): - Inappropriate PAH drug found → deprescribe + reassess — 2022 ESC/ERS PH - Persistent Cpc-PH despite optimised LHD → PH-expert centre — 2022 ESC/ERS PH - Decompensated LHD/RV failure → acute pathway — 2022 ACC/AHA HF
Earlier-Return Triggers
Return-precaution thresholds (watch for): - [SEVERE] Group 2 PH — PAH-specific therapy (ERA/PDE5i/prostacyclin/riociguat) is NOT indicated and is HARMFUL (pulmonary edema); deprescribe if started, treat the LHD instead — 2022 ESC/ERS PH - [SEVERE] Combined post-/pre-capillary PH (PAWP >15 + PVR >2 WU) — worse prognosis; optimise LHD then PH-expert centre (PAH drugs only in trial/specialist context) — 2022 ESC/ERS PH - [SEVERE] Severe AS/MS/MR-driven Group 2 PH — valve intervention often substantially reverses PH — 2020 ACC/AHA VHD
Citations
- 2022 ESC/ERS Pulmonary Hypertension Guideline (Humbert) + 2022 AHA/ACC/HFSA HF Guideline [PMID:35379504](https://pubmed.ncbi.nlm.nih.gov/35379504/) - Cited evidence (PMID 37622666) [PMID:37622666](https://pubmed.ncbi.nlm.nih.gov/37622666/) - Cited evidence (PMID 36027570) [PMID:36027570](https://pubmed.ncbi.nlm.nih.gov/36027570/) - Cited evidence (PMID 31535829) [PMID:31535829](https://pubmed.ncbi.nlm.nih.gov/31535829/) - Cited evidence (PMID 39225278) [PMID:39225278](https://pubmed.ncbi.nlm.nih.gov/39225278/) Last reconciled with current guidelines: 2026-05-16.
- 2022 ESC/ERS Pulmonary Hypertension Guideline (Humbert) + 2022 AHA/ACC/HFSA HF Guideline — PMID:35379504
- Cited evidence (PMID 37622666) — PMID:37622666
- Cited evidence (PMID 36027570) — PMID:36027570
- Cited evidence (PMID 31535829) — PMID:31535829
- Cited evidence (PMID 39225278) — PMID:39225278