Nebuliser liquid
▶Ventavis(Bayer Plc)
Iloprost (as Iloprost trometamol) 10 microgram per 1 mlVentavis
10 micrograms/ml nebuliser solution 1 ml ampoules|
30 ampouleP£ 400. 19 | 168 ampouleP£ 2 , 241. 08
Iloprost (as Iloprost trometamol) 20 microgram per 1 mlVentavis
20 micrograms/ml nebuliser solution 1 ml ampoules|
30 ampouleP£ 400. 19 | 168 ampouleP£ 2 , 241. 08ENDOTHELIN RECEPTOR ANTAGONISTS
Bosentan
lINDICATIONS AND DOSE
Pulmonary arterial hypertension (initiated under
specialist supervision)
▶BY MOUTH
▶Child 2–17 years (body-weight 10–20 kg):Initially
31. 25 mg once daily for 4 weeks, then increased to
31. 25 mg twice daily
▶Child 2–17 years (body-weight 20–40 kg):Initially
31. 25 mg twice daily for 4 weeks, then increased to
62. 5 mg twice daily
▶Child 12–17 years (body-weight 40 kg and above):Initially
62. 5 mg twice daily for 4 weeks, then increased to
125 mg twice daily (max. per dose 250 mg)lCONTRA-INDICATIONSAcute porphyrias p. 603
lCAUTIONSNot to be initiated if systemic systolic blood
pressure is below 85 mmHg
lINTERACTIONS→Appendix 1 : bosentan
lSIDE-EFFECTS
▶Common or very commonAnaemia.diarrhoea.flushing.
gastrooesophageal reflux disease.headache.nasal
congestion.palpitations.skin reactions.syncope
▶UncommonHepatic disorders.leucopenia.neutropenia.
thrombocytopenia
▶Rare or very rareAngioedema
▶Frequency not knownVision blurred
lCONCEPTION AND CONTRACEPTIONEffective
contraception required during administration (hormonal
contraception not considered effective). Monthly
pregnancy tests advised.
lPREGNANCYAvoid (teratogenic inanimalstudies).
lBREAST FEEDINGManufacturer advises avoid—no
information available.
lHEPATIC IMPAIRMENTAvoid in moderate and severe
impairment.
lMONITORING REQUIREMENTS
▶Monitor haemoglobin before and during treatment
(monthly forfirst 4 months, then 3 -monthly).
▶Monitor liver function before treatment, at monthly
intervals during treatment, and 2 weeks after dose increase
(reduce dose or suspend treatment if liver enzymes raised
significantly)—discontinue if symptoms of liver
impairment.
lTREATMENT CESSATIONAvoid abrupt withdrawal—
withdraw treatment gradually.
lDIRECTIONS FOR ADMINISTRATIONTablets may be cut, or
suspended in water or non-acidic liquid. Suspension is
stable at room-temperature (max. 25 °C) for 24 hours.lMEDICINAL FORMS
There can be variation in the licensing of different medicines
containing the same drug.
Tablet
▶Bosentan (Non-proprietary)
Bosentan (as Bosentan monohydrate) 62.5 mgBosentan 62. 5 mg
tablets| 56 tabletP£ 1 , 359. 19 – £ 1 , 510. 21
Bosentan (as Bosentan monohydrate) 125 mgBosentan 125 mg
tablets| 56 tabletP£ 1 , 359. 19 – £ 1 , 510. 21▶Stayveer(AMCo)
Bosentan (as Bosentan monohydrate) 62.5 mgStayveer 62. 5 mg
tablets| 56 tabletP£ 1 , 510. 21
Bosentan (as Bosentan monohydrate) 125 mgStayveer 125 mg
tablets| 56 tabletP£ 1 , 510. 21
▶Tracleer(Actelion Pharmaceuticals UK Ltd)
Bosentan (as Bosentan monohydrate) 62.5 mgTracleer 62. 5 mg
tablets| 56 tabletP£ 1 , 510. 21
Bosentan (as Bosentan monohydrate) 125 mgTracleer 125 mg
tablets| 56 tabletP£ 1 , 510. 21PHOSPHODIESTERASE TYPE-5 INHIBITORS
Sildenafil 20-Jul-2017
lINDICATIONS AND DOSE
Pulmonary arterial hypertension (initiated under
specialist supervision)
▶BY MOUTH
▶Neonate:Initially 250 – 500 micrograms/kg every
4 – 8 hours, adjusted according to response, start with
the lower dose and frequency, especially if used with
other vasodilators; maximum 30 mg per day.▶Child 1–11 months:Initially 250 – 500 micrograms/kg
every 4 – 8 hours, adjusted according to response, start
with the lower dose and frequency, especially if used
with other vasodilators; maximum 30 mg per day
▶Child 1–17 years (body-weight up to 20 kg): 10 mg 3 times
a day
▶Child 1–17 years (body-weight 20 kg and above): 20 mg
3 times a day
DOSE ADJUSTMENTS DUE TO INTERACTIONS
▶Manufacturer advises reduce dose with concurrent use
of moderate and potent inhibitors of CYP 3 A 4 (avoid
with ketoconazole, itraconazole and ritonavir)—no
specific recommendation made for children.lUNLICENSED USENot licensed for use in children under
1 year.
lCONTRA-INDICATIONSHereditary degenerative retinal
disorders.history of non-arteritic anterior ischaemic optic
neuropathy.recent history of stroke.sickle-cell anaemia
lCAUTIONSActive peptic ulceration.anatomical
deformation of the penis.autonomic dysfunction.
bleeding disorders.cardiovascular disease.hypotension
(avoid if severe).intravascular volume depletion.left
ventricular outflow obstruction.ocular disorders.
predisposition to priapism.pulmonary veno-occlusive
disease
lINTERACTIONS→Appendix 1 : phosphodiesterase type- 5
inhibitors
lSIDE-EFFECTS
▶Common or very commonDizziness.dry mouth.eye
discomfort.gastrointestinal discomfort.haemorrhage.
headache.myalgia.nasal complaints.nausea.
vasodilation.vertigo.vision disorders
▶UncommonArrhythmias.chest pain.drowsiness.dry eye.
eye disorders.eye inflammation.fatigue.feeling hot.
gastrooesophageal reflux disease.haematospermia.
hearing impairment.hypertension.hypotension.
numbness.pain in extremity.palpitations.rash.rhinitis.
sinus congestion.vomiting
▶Rare or very rareAcute coronary syndrome.
arteriosclerotic retinopathy.cerebrovascular insufficiency
.glaucoma.irritability.optic neuropathy (discontinue if
sudden visual impairment occurs).oral hypoaesthesia.
priapism.retinal occlusion.scleral discolouration.seizure
.severe cutaneous adverse reactions (SCARs).sudden
cardiac death.syncope.throat tightness
lPREGNANCYUse only if potential benefit outweighs risk—
no evidence of harm inanimalstudies.120 Blood pressure conditions BNFC 2018 – 2019
Cardiovascular system2