Pulmonary Arterial Hypertension Pregnancy: Contraindicated — animal reproductive toxicity; PAH in pregnancy carries high maternal mortality; specialist PAH obstetric management required

Iloprost (Inhaled)

Brand names: Ventavis

Adult dose

Dose: 2.5 mcg per inhalation session starting, increasing to 5 mcg per session as tolerated

Route: Inhaled via I-neb AAD or Breelib nebuliser

Frequency: 6–9 inhalation sessions per day during waking hours

Max: 5 mcg per session; 45 mcg/day maximum

Administer using Ventavis-specific nebuliser devices only (I-neb AAD or Breelib — NOT standard nebulisers). Each session takes 4–10 minutes. Avoid contact with eyes and skin. Effect duration approximately 30–60 minutes.

Paediatric dose

Dose: Seek specialist opinion N/A/kg

Route: Inhaled

Frequency: N/A

Max: N/A

Not established in paediatrics; seek specialist paediatric pulmonary hypertension opinion

Dose adjustments

Renal

No dose adjustment required for inhaled route — minimal systemic absorption

Hepatic

Use with caution in hepatic impairment — iloprost is hepatically metabolised; start at lower dose and titrate slowly

Paediatric weight-based calculator

Patient weight (kg)

Not established in paediatrics; seek specialist paediatric pulmonary hypertension opinion

Clinical pearls

Mechanism: synthetic prostacyclin I2 (PGI2) analogue — more stable than natural prostacyclin; IP receptor agonism causes pulmonary vasodilation; anti-proliferative; anti-platelet; inhaled delivery preferentially targets the pulmonary vasculature with less systemic hypotension than IV route
6–9 sessions per day: inhaled iloprost's short duration of action (30–60 minutes) requires 6–9 daily inhalation sessions — a significant treatment burden compared to once-daily or twice-daily oral agents; compliance is challenging
AIR trial (Lancet 2002): inhaled iloprost vs placebo in PAH/CTEPH — significant improvement in 6-minute walk distance and WHO functional class; first inhaled prostanoid trial in PAH
Dedicated nebuliser device: MUST use Ventavis-compatible device (I-neb or Breelib) — standard nebulisers deliver incorrect particle size and drug distribution; I-neb AAD uses adaptive aerosol delivery to optimise inhalation
MHRA: licensed for PAH WHO Group I in patients with NYHA/WHO functional class III; also used in SSc-related PAH (note: iloprost_rheum is the IV formulation entry for SSc; this entry is inhaled Ventavis); NICE TA459
CTEPH off-label: inhaled iloprost sometimes used in inoperable CTEPH as part of combination regimen; riociguat is the primary licensed treatment for CTEPH and MHRA-approved for this indication

Contraindications

Conditions where vasodilation may be dangerous (severe coronary artery disease, haemodynamic instability)
Known hypersensitivity to iloprost or structurally related compounds
Pulmonary venous hypertension (Group 2 PH) — targeted PAH therapy may worsen pulmonary oedema

Side effects

Cough (most common — up to 39%; prostacyclin irritation in airways)
Jaw pain (prostacyclin class)
Headache and flushing
Hypotension (systemic — less than IV route)
Syncope (around inhalation sessions)
Trismus and limb pain

Interactions

Antihypertensives (additive hypotension — particularly during inhalation sessions)
Anticoagulants (iloprost inhibits platelet aggregation — additive bleeding risk)
Vasodilators (additive hypotension)

Monitoring

6-minute walk test (baseline and quarterly)
WHO functional class
Blood pressure (particularly during inhalation — syncope risk)
Oxygen saturation during inhalation sessions
Compliance with number of sessions per day (adherence monitoring)
Pulmonary haemodynamics (RHC annually)

Reference: BNFc; BNF 90; AIR trial Lancet 2002;360(9337):896-901; ESC/ERS PAH Guidelines 2022; NICE TA459; MHRA SPC Ventavis. Verify against your local formulary and the latest BNF before prescribing.

Curated clinical cross-links plus same-class fallbacks.

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