This handout is for pulmonary hypertension groups 2–5 (non-pah). Your care team identified this based on: unexplained exertional dyspnea / pre-syncope / rv failure signs — raised jvp, rv heave, loud p2, edema (esc/ers 2022 §6).
Other reasons your team may use this plan: 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); hfpef / hfref / valvular or advanced copd/ild with ph features → group 2 vs 3 (esc/ers 2022 §7-8).
Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.
| Medication | Starting dose | How | When | What it does |
|---|---|---|---|---|
| furosemide | 20–80 mg | PO/IV | daily–BID, titrate to euvolemia | 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). |
| spironolactone | 12.5–25 mg | PO | daily | GDMT pillar for left-heart disease driving Group 2 PH (ESC/ERS 2022 §7; 2022 AHA/ACC/HFSA HF) |
| sacubitril_valsartan | 24/26–49/51 mg BID, titrate | PO | BID | PARADIGM-HF / PARAGON-HF — treating HF lowers post-capillary pulmonary pressures; the correct Group 2 lever (ESC/ERS 2022 §7) |
| dapagliflozin | 10 mg | PO | daily | DAPA-HF + DELIVER — across-EF HF benefit; Group 2 PH improves with LHD control (ESC/ERS 2022 §7) |
| oxygen | titrate to SpO2 ≥90% (resting PaO2 ≤55 or ≤59 with cor pulmonale) | inhaled | continuous ≥15 h/day | Long-term O2 is the survival-modifying base therapy in chronic hypoxic Group 3 PH (ESC/ERS 2022 §8; NOTT/MRC) |
| treprostinil (inhaled) | 3 breaths (18 µg) QID, titrate up to 12 breaths (72 µg) QID | inhaled | four times daily | 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. |
| apixaban | 5 mg BID (2.5 mg BID if ≥2 of age ≥80 / weight ≤60 kg / Cr ≥1.5) | PO | BID | 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. |
| warfarin | titrate to INR 2.0–3.0 | PO | daily | 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. |
| riociguat | 1 mg TID, titrate q2 wk to max 2.5 mg TID | PO | TID | 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. |
| macitentan | 10 mg | PO | once daily | 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. |
| pulmonary endarterectomy (PEA) | deep-hypothermic circulatory-arrest endarterectomy at a high-volume CTEPH surgical centre | surgical | once (potentially curative) | 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) | staged segmental/subsegmental balloon dilation over multiple sessions at a CTEPH centre | interventional | staged sessions | 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 | treat the driver: hydroxyurea/exchange transfusion (sickle), immunosuppression (sarcoidosis), splenectomy-thrombosis surveillance, dialysis optimisation (CKD) | varies | per substrate | 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 | stop/switch with prescribing-specialty input (methamphetamine, dasatinib, interferons, leflunomide, mitomycin C, anorexigens) | n/a | one-time + echo surveillance | 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. |
Plan: Group-specific therapy — treat the underlying disease (ESC/ERS 2022)
Contact your care team if any of the following happen:
Call 911 or go to the nearest emergency room right away if you have:
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)
Guideline: 2022 ESC/ERS Pulmonary Hypertension Guideline (Humbert) — current as of May 2026; supplemented by 7th WSPH 2024