Progressive dyspnea, ↑RV pressure on echo
Screen → confirm: Echocardiogram → Right heart catheterization + vasoreactivity testing
Vasoreactive → CCB. Otherwise → endothelin antagonist (bosentan/ambrisentan), PDE5-i (sildenafil), prostacyclin (epoprostenol). ESC/ERS 2022: initial oral COMBINATION (ERA + PDE5i, e.g., ambrisentan + tadalafil — AMBITION) for low/intermediate-risk PAH; sotatercept add-on for high-risk/residual PAH (STELLAR)
Order set
- Echo
- Right heart catheterisation + vasoreactivity
- V/Q (CTEPH)
- PFTs, autoimmune screen
Criteria
AdmitRV failure or syncope
ICUDecompensated RV failure
VasopressorsRV failure (careful — noradrenaline)
DischargeConfirmed group, targeted therapy in expert centre
Clinical detail — differentials, red flags, pitfalls, disposition
Red flags
- Syncope
- RV failure
- Rapid deterioration
Differentials
- Left heart disease
- Lung disease/hypoxia
- CTEPH
- Idiopathic PAH
Common mistakes
- Skipping right heart cath
- Missing treatable CTEPH
Disposition & follow-up
Confirm group; targeted PAH therapy in expert centre.
Discharge package
MedicationsTargeted PAH therapy (expert centre)
Follow-upPH centre
Warning symptomsWorsening breathlessness, syncope, oedema
💊 Treatment detail — doses & preparation
SildenafilPDE5 inhibitor (PH)
Dose20 mg PO TDS (pulmonary hypertension, specialist-led)
Preparation20 mg tablets
MonitorNEVER with nitrates (severe hypotension); BP
Bosentanendothelin antagonist (PH)
Dose62.5 mg BD ×4 wk → 125 mg BD (PAH, specialist-led)
Preparation62.5/125 mg tablets
MonitorLFTs monthly (mandatory), Hb; teratogenic
Furosemide POloop diuretic
Dose20–80 mg OD/BD, titrate to dry weight
Preparation20/40/500 mg tablets; take morning/midday
MonitorDaily weight, K⁺, renal function
Specialist referralprinciple
DoseVasodilators only after right heart catheter confirmation; diurese carefully (RV is preload-dependent)
Preparation—
MonitorSpO₂, exertional tolerance, echo
📖 ESC/ERS Pulmonary HypertensionReviewed July 2026