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Prostacyclin (PGI2) — Severe Pulmonary Arterial Hypertension Pregnancy: Use with extreme caution — PAH in pregnancy carries very high maternal mortality; epoprostenol used as bridge in specialist centres

Epoprostenol

Brand names: Flolan, Veletri

Adult dose

Dose: Initial: 2 nanograms/kg/min IV; titrate by 2 ng/kg/min every 15 minutes to tolerance; maintenance typically 20–40 ng/kg/min
Route: Continuous intravenous infusion via central line (Hickman/PICC)
Frequency: Continuous 24 hours/day
Max: Titrated individually — no fixed maximum; limited by side effects
Extremely short half-life (3–5 minutes) — must never be interrupted (risk of rebound PAH crisis and death). Requires dedicated central venous catheter, ambulatory pump, and specialist PAH centre management. Veletri more thermostable than Flolan — no ice required. Reconstitution in alkaline diluent (pH 10–12).

Paediatric dose

Dose: 1–2 nanograms/kg/min initially ng/min/kg
Route: Continuous IV infusion
Frequency: Continuous
Max: Titrated by specialist
Used in paediatric severe PAH under specialist cardiology; same principles as adult dosing

Dose adjustments

Renal

No dose adjustment required

Hepatic

No dose adjustment required

Paediatric weight-based calculator

Used in paediatric severe PAH under specialist cardiology; same principles as adult dosing

Clinical pearls

  • Gold standard for WHO functional class IV PAH — demonstrated mortality benefit in landmark trials (Barst 1996)
  • Half-life 3–5 minutes: pump failure, line occlusion, or even brief interruption can cause fatal rebound pulmonary hypertension — patients carry emergency protocols
  • Jaw pain with meals/eating is pathognomonic of prostacyclin therapy — caused by increased blood flow to jaw muscles
  • Pulmonary veno-occlusive disease (PVOD): epoprostenol can cause fatal pulmonary oedema — HRCT showing centrilobular nodules/septal lines should prompt caution
  • Dose requirements often increase over years — tachyphylaxis partial; tolerance develops

Contraindications

  • Pulmonary oedema (suggests pulmonary veno-occlusive disease — may worsen)
  • Chronic heart failure with severe LV dysfunction
  • Known hypersensitivity

Side effects

  • Jaw pain (very common on dose increase)
  • Flushing
  • Headache
  • Nausea and diarrhoea
  • Musculoskeletal pain
  • Hypotension
  • Catheter-related infections (central line)
  • Thrombocytopaenia (long-term)
  • Rebound PAH crisis if infusion interrupted

Interactions

  • Anticoagulants — additive antiplatelet/bleeding effect (patients usually anticoagulated concurrently)
  • Antihypertensives — additive hypotension
  • Digoxin — epoprostenol may reduce digoxin levels

Monitoring

  • Continuous BP and heart rate monitoring during initiation/uptitration
  • 6MWD and echocardiography/RHC
  • FBC (thrombocytopaenia)
  • Catheter site infection surveillance
  • Pump function and line integrity daily

Reference: BNFc; BNF 90; Barst RJ et al. NEJM 1996; NICE TA238 (Epoprostenol for PAH); ESC/ERS PAH Guidelines 2022. Verify against your local formulary and the latest BNF before prescribing.

Related

Curated clinical cross-links plus same-class fallbacks.

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