Pulmonary hypertension due to left heart disease (Group 2 PH, cross-system)
Group 2 PH (PH due to left heart disease) — RHC phenotyping (Ipc vs Cpc); dominant not-to-miss: PAH-specific drugs NOT indicated/HARMFUL — treat the LHD (phenotype-specific HF GDMT / valve intervention / decongestion) + reassess PVR reversibility; RHC informs transplant PVR candidacy. Manifest points at existing sibling cardio.acute-hf.core.v1.ts per nearest-ID precedent so the audit broken_pointers check passes; decision surface (treat-LHD + no-PAH-drug-guardrail axis + workups + calculators + panels), test_files, 5-PMID evidence object (2022 ESC/ERS PH referenced by name — evidence-gap, not fabricated), chronic phases all present. HF-GDMT drug RxCUIs reused from RxNav-validated cardio dossiers (dapagliflozin 1488564, empagliflozin 1545653, sacubitril/valsartan 1656340, finerenone 2627043, carvedilol 20352, spironolactone 9997, furosemide 4603); valve-intervention/referral/guardrail non_pharm; SNOMED deferred. 9 trigger/special-pop branches: no-PAH-drugs (not-to-miss), Cpc-PH, HFpEF-Group2, valvular-Group2, HFrEF-Group2, combined-Group2+3, transplant-PVR, PVR-reversibility, secondary-TR.
Entry points (5)
- imagingEcho: elevated PASP/RV strain with HFrEF/HFpEF/valve/LA diseaseecho_ph_with_lhd
- symptomWorsening dyspnea in known left heart diseasedyspnea_with_known_lhd
- lab_abnormalityRising NT-proBNP with PH features + left heart diseaserising_np_with_lhd
- historyRHC showing post-capillary (PAWP >15) PHph_on_rhc_postcapillary
- medicationPAH-specific drug prescribed in suspected Group 2 PHinappropriate_pah_drug_in_group2
Required inputs (10)
- agerequireddemographic • used at CONTEXTHFpEF/Group-2 prevalence; transplant candidacy
- left_heart_disease_typerequiredimaging • used at FRAMEHFrEF vs HFpEF/HFmrEF vs valvular vs LA disease — the driver to treat
- pawprequiredimaging • used at RISK_STRATIFICATIONPAWP >15 mmHg defines post-capillary (Group 2) PH
- pvrrequiredimaging • used at RISK_STRATIFICATIONPVR ≤2 WU (Ipc-PH) vs >2 WU (Cpc-PH) — phenotype + prognosis
- rv_functionimaging • used at INITIAL_WORKUPRV strain/dysfunction — severity + transplant implications
- natriuretic_peptidelab • used at INITIAL_WORKUPLHD severity + monitoring; supports Group 2 over Group 1
- lung_diseasehistory • used at CONTEXTComorbid lung disease → combined Group 2+3 PH
- current_medsrequiredmedication • used at CONTEXTDetect inappropriate PAH-specific therapy in Group 2 PH
- nyha_classrequiredsymptom • used at RISK_STRATIFICATIONFunctional class; transplant evaluation
- creatininerequiredlab • used at TREATMENTHF GDMT dosing; RHC contrast
12-phase flow (12)
- 1FRAMEPH suspected with a left-heart driver — frame as Group 2 (not Group 1/3/4)inputs: left_heart_disease_typeadvance: left-heart driver + Group-2 hypothesis framed
- 2ENTRYPH on echo + LHD, dyspnea, rising NP, post-capillary RHC, inappropriate PAH druginputs: ageadvance: entry trigger captured
- 3CONTEXTHF phenotype, valve lesion, LA disease, comorbid lung disease, current medsinputs: lung_disease, current_medsadvance: driver + comorbidity context complete
- 4RED_FLAGSDecompensated LHD, RV failureinputs: nyha_classactions: cardiogenic_shock, acute_pulm_edemaadvance: no red flags or routed to acute pathway
- 5INITIAL_WORKUPEcho (LHD + PASP + RV), NT-proBNP, ECGinputs: rv_function, natriuretic_peptideactions: panel.cardiacadvance: LHD + PH severity documented
- 6BRANCHING_WORKUPRight-heart catheterisation (PAWP/PVR/TPG → Ipc vs Cpc); exclude superimposed Group 1/3/4inputs: pawp, pvractions: preop_cardiacadvance: post-capillary confirmed + Ipc/Cpc phenotyped
- 7DIFFERENTIALGroup 2 vs Group 1/3/4; Ipc-PH vs Cpc-PH; combined Group 2+3inputs: pawp, pvr, lung_diseaseadvance: PH group + phenotype assigned
- 8RISK_STRATIFICATIONCpc-PH = worse prognosis; fixed/elevated PVR → heart-transplant candidacy implicationsinputs: pvr, nyha_classadvance: phenotype + prognosis + transplant implication assigned
- 9TREATMENTOptimise the LHD (phenotype-specific HF GDMT / valve intervention / decongestion); do NOT use PAH-specific drugs; reassess PVR reversibilityinputs: left_heart_disease_type, creatinineadvance: LHD-optimisation plan + no-PAH-drug guardrail documented
- 10DISPOSITIONHF/valve clinic; PH-expert centre for Cpc-PH / transplant candidacyinputs: pvractions: preop_cardiacadvance: referral plan set
- 11MONITORINGReassess PVR/PASP + symptoms after LHD optimisation (reversibility); RV functioninputs: pvr, natriuretic_peptideactions: panel.cardiacadvance: reversibility-reassessment cadence documented
- 12FOLLOWUPTransplant candidacy reassessment; re-phenotype if PH persists/worsens despite LHD optimisationinputs: pvradvance: long-term plan documented