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Pulmonary Arterial Hypertension Pregnancy: Use with caution — PAH in pregnancy is extremely high-risk (maternal mortality 30–50%); epoprostenol used in pregnancy under specialist supervision as treatment for severe PAH when termination not chosen

Epoprostenol

Brand names: Flolan, Veletri

Adult dose

Dose: 2 ng/kg/minute initial dose; increase by 2 ng/kg/minute every 15 minutes to tolerance during initiation; maintenance 20–40 ng/kg/minute (individual titration)
Route: Continuous IV infusion via central line
Frequency: Continuous 24-hour infusion — MUST NOT be interrupted
Max: Individual — titrate to haemodynamic response and tolerability
NEVER stop abruptly — rebound pulmonary hypertensive crisis can be fatal. Requires central venous access. Veletri stable at room temperature for 72 hours; Flolan requires ice packs. Patients carry portable pump 24/7.

Paediatric dose

Dose: 2 ng/kg/minute starting; titrate individually ng/min/kg
Route: Continuous IV
Frequency: Continuous infusion
Max: Individual titration
Used in paediatric PAH at specialist centres; seek specialist paediatric pulmonary hypertension opinion

Dose adjustments

Renal

No dose adjustment — renally excreted metabolites; use with caution in severe renal impairment

Hepatic

Use with caution in hepatic impairment — epoprostenol metabolised by spontaneous hydrolysis and enzymatic degradation; very short half-life (2–3 minutes)

Paediatric weight-based calculator

Used in paediatric PAH at specialist centres; seek specialist paediatric pulmonary hypertension opinion

Clinical pearls

  • Mechanism: synthetic prostacyclin (PGI2) — vasodilates pulmonary and systemic vasculature by adenylate cyclase-mediated cAMP increase in smooth muscle; inhibits platelet aggregation; anti-proliferative on vascular smooth muscle; extremely short half-life (2–3 minutes) requiring continuous IV infusion
  • First-line therapy for severe PAH: epoprostenol is the only PAH drug with proven mortality benefit in randomised trials; MHRA and ESC/ERS guidelines recommend continuous IV epoprostenol for WHO Class III/IV PAH or vasoreactive PAH
  • REBOUND CRISIS: brief interruption of epoprostenol infusion can trigger life-threatening pulmonary hypertensive crisis within minutes — patients must carry a backup pump and spare cassettes AT ALL TIMES; partners/family trained in emergency pump access
  • Central line management: long-term central venous catheter is the greatest infection risk; Flolan requires constant refrigeration with ice packs (limits portability); Veletri (room temperature stable) improves patient mobility significantly
  • MHRA: Flolan and Veletri are licensed for PAH; NICE TA459 (Veletri) recommended as option for WHO class III/IV PAH; prescribing restricted to specialist PAH centres
  • Vasoreactivity testing: epoprostenol is used during right heart catheterisation for acute vasoreactivity testing — a positive response (mean PAP fall above 10 mmHg to below 40 mmHg) identifies patients who may respond to calcium channel blockers long-term

Contraindications

  • Chronic heart failure with severe left ventricular dysfunction (pulmonary oedema risk)
  • Known hypersensitivity to epoprostenol or glycine diluent
  • Interruption to infusion — even brief interruption can cause life-threatening rebound hypertension

Side effects

  • Flushing (most common — prostacyclin vasodilatory effect)
  • Headache
  • Jaw pain (prostacyclin class — during meals)
  • Nausea and diarrhoea
  • Hypotension
  • Catheter-related sepsis (central line — most serious complication with long-term use)
  • Rebound pulmonary hypertension if infusion interrupted (potentially fatal)

Interactions

  • Antihypertensives and diuretics (additive hypotension — monitor BP closely)
  • Antiplatelet agents (epoprostenol inhibits platelet aggregation — additive bleeding risk with antiplatelets/anticoagulants)
  • Vasodilators (additive hypotension)

Monitoring

  • Haemodynamic parameters (RHC at 3–4 months to assess treatment response)
  • 6-minute walk test (monthly initially, then quarterly)
  • WHO functional class
  • Blood pressure (hypotension monitoring)
  • Central line site (infection surveillance)
  • Platelet count (antiplatelet effect)

Reference: BNFc; BNF 90; ESC/ERS PAH Guidelines 2022; NICE TA459; MHRA SPC Flolan/Veletri; Lancet 1996;347(9000):322-328 (first RCT). Verify against your local formulary and the latest BNF before prescribing.

Related

Curated clinical cross-links plus same-class fallbacks.

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