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Patient handout

Pulmonary hypertension due to left heart disease (Group 2 PH, cross-system)

PRODUCTION

1. Your condition

This handout is for pulmonary hypertension due to left heart disease (group 2 ph, cross-system). Your care team identified this based on: echo: elevated pasp/rv strain with hfref/hfpef/valve/la disease.

Other reasons your team may use this plan: worsening dyspnea in known left heart disease; rising nt-probnp with ph features + left heart disease; rhc showing post-capillary (pawp >15) ph.

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

MedicationStarting doseHowWhenWhat it does
do NOT initiate PAH-specific therapy (ERA/PDE5i/prostacyclin/riociguat) in Group 2 PH2022 ESC/ERS PH — PAH drugs in Group 2 PH are not beneficial and can cause pulmonary edema/harm; deprescribe if inappropriately started

Plan: Group 2 PH — treat the left heart disease, NO PAH-specific drugs (2022 ESC/ERS PH; 2022 AHA/ACC/HFSA HF)

3. When to call your provider

Contact your care team if any of the following happen:

  • 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

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

  • 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
  • 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 AS/MS/MR-driven Group 2 PH — valve intervention often substantially reverses PH — 2020 ACC/AHA VHD
  • Heart-transplant evaluation with elevated PVR — vasoreactivity testing; fixed high PVR may require combined strategy / contraindicate isolated heart transplant — 2022 ESC/ERS PH

5. Follow-up

Transplant candidacy reassessment; re-phenotype if PH persists/worsens despite LHD optimisation

6. Sources

Guideline: 2022 ESC/ERS Pulmonary Hypertension Guideline (Humbert) + 2022 AHA/ACC/HFSA HF Guideline

  1. pubmed.ncbi.nlm.nih.gov/35379504
  2. pubmed.ncbi.nlm.nih.gov/37622666
  3. pubmed.ncbi.nlm.nih.gov/36027570