Pulmonary Arterial Hypertension Drug Summary

Last updated: 17 March 2026
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Calcium Antagonists


Drug Dosage Remarks
Benzothiazepine
Diltiazem 60 mg PO 12 hourly
Target dose: 120-360 mg PO 12 hourly
Max dose: 720 mg/day
Should be started at low dose and titrated as tolerated 		Adverse Reactions
  • CV effects (depression of cardiac function, hypotension, worsening heart failure, edema, flushing, bradycardia); GI effect (constipation); CNS effects (headache, dizziness)
  • Diltiazem: AV dissociation, AV block, bradycardia and sinus node dysfunction
  • Short-acting dihydropyridine agents should be avoided because they have the potential to enhance risk of adverse cardiac events
Special Instructions
  • Contraindicated in patients with overt decompensated heart failure, though vasoselective dihydropyridines (eg Amlodipine) are tolerated in patients with decreased LV ejection fraction
  • HR-modulating Ca antagonists are contraindicated in patients with bradycardia, sinus node dysfunction and AV nodal block
Dihydropyridines
Amlodipine 5 mg PO 24 hourly
Target dose: 15-30 mg PO 24 hourly
Increase dose as tolerated
Max dose: 30 mg/day
Nifedipine 10 mg PO 8 hourly or 30 mg PO 24 hourly
Target dose: Up to 120-240 mg/day PO or 20-60 mg PO 8-12 hourly
Max dose: 240 mg/day
Should be started at low dose and titrated as tolerated

Other Antihypertensives

Drug Dosage Remarks
Activin Signaling Inhibitor
Sotatercept-csrk 0.3 mg/kg/dose SC injection every 3 weeks
May increase to 0.7 mg/kg/dose SC injection every 3 weeks if Hb and platelet count are acceptable 		Adverse Reactions
  • CNS effects (headache, dizziness); Hematologic effects (erythrocytosis, thrombocytopenia, bleeding); Other effects (epistaxis, rash, telangiectasia, diarrhea, erythema, infertility)
Special Instructions
  • Do not administer if patient is experiencing serious bleeding
  • Monitor Hb and platelet count before each dose for the first 5 doses or longer if values are unstable, and periodically thereafter to determine if dose adjustments are needed
  • Advise females of childbearing potential of the potential risk to a fetus and use of effective contraception
Endothelin Receptor Antagonists
Ambrisentan 5-10 mg PO 24 hourly
In combination therapy with Tadalafil:
10 mg PO 24 hourly 		Adverse Reactions
  • CNS effects (headache, fatigue, syncope); CV effects (flushing, hypotension, palpitations, edema); Other effects (nasopharyngitis, abnormal hepatic function, GI disturbances, pruritus, anemia)
Special Instructions
  • Avoid in patients with moderate-severe hepatic function
  • Check LFT prior to therapy and then monthly
  • Measure Hb after 1 and 3 months of therapy and every 3 months thereafter
  • Adequate contraception should be used in women of childbearing age
Bosentan <40 kg
Initial and maintenance dose:
62.5 mg PO 12 hourly
≥40 kg
Initial dose: 62.5 mg PO 12 hourly x 4 weeks
Then increase to:
Maintenance dose: 125 mg PO 12 hourly
Macitentan 10 mg PO 24 hourly Adverse Reactions
  • Respiratory effects (nasopharyngitis, bronchitis, pharyngitis, influenza infection, nasal congestion); CV effects (hypotension, fluid retention, edema); Hematologic effects (anemia, leukopenia, thrombocytopenia); Other effects (headache, UTI, aminotransferase elevation)
Special Precautions
  • Avoid use in patients with severe hepatic impairment, baseline values of liver aminotransferase >3x ULN, severe anemia
  • Use with caution in patients with severe anemia, PVOD, moderate-severe renal impairment, moderate hepatic impairment; obtain baseline liver enzymes
  • Monitor HB concentration, liver enzymes, BP
  • Adequate contraception should be used in women of childbearing age
Phosphodiesterase Inhibitors
Sildenafil 5 mg or 20 mg PO 8 hourly
May increase dose to 40-80 mg PO 8 hourly as tolerated or
2.5 mg or 10 mg IV 8 hourly 		Adverse Reactions
  • CNS effects (headache, dizziness, vertigo); CV effects (flushing, fluid retention); GI effects (stomach upset, dyspepsia, diarrhea, abdominal distention); Ophthalmologic effects (visual disturbances eg transient altered color vision, blurred vision); Musculoskeletal effects (myalgia, back pain); Respiratory effects (nasal congestion, epistaxis); Other effects (sudden loss or decrease in hearing, priapism, anemia, alopecia, erythema, night sweats, pyrexia)
Special Precautions
  • Avoid grapefruit juice
  • Contraindicated in patients with known hypersensitivity to any component of the drug, concurrently or intermittently using organic nitrates in any form, severe CV disorders (eg unstable angina, cardiac failure), recent history of stroke or myocardial infarction (MI), severe hepatic impairment, known degenerative retinal disorders, loss of vision in one eye due to non-arteritic anterior chamber ischemic optic neuropathy, hypotension (BP <90/50 mmHg)
  • Use with caution in patients with anatomical deformation of the penis (eg angulation, cavernosal fibrosis or Peyronie’s disease) or in patients who have conditions which may predispose them to priapism (eg sickle cell anemia, multiple myeloma or leukemia), bleeding disorders, mild to moderate hepatic impairment, severe renal impairment, hypotension, uncontrolled hypertension, left ventricular outflow obstruction (eg aortic stenosis), life-threatening arrhythmias, stroke or MI within the last 6 months
  • Sudden cessation of treatment may exacerbate PAH
Tadalafil 40 mg PO 24 hourly Adverse Reactions
  • CNS effects (headache, syncope, dizziness); GI effects (gastroesophageal reflux, dyspepsia, nausea/vomiting); CV effects (flushing, hypotension, anginal chest pain, palpitations, tachycardia); Musculoskeletal effects (back pain, myalgia, pain in extremity); Respiratory effects (nasopharyngitis, respiratory tract infection, nasal congestion, dyspnea, epistaxis); Other effects (sudden hearing loss, hematuria, blurred vision or sudden vision loss)
Special Instructions
  • Contraindicated in patients with known CV disease, MI within the last 90 days, unstable angina or angina occurring during sexual intercourse, NYHA Class ≥ 2 heart failure in the last 6 months, uncontrolled arrhythmias, hypotension (<90/50 mmHg) or uncontrolled hypertension, stroke within the last 6 months, had loss of vision in one eye due to non-arteritic ischemic optic neuropathy (NAION) and those taking nitrates
  • Use with caution in patients with renal or mild to moderate hepatic insufficiency, hematological disorders, galactose intolerance, and those taking antihypertensives and α-blockers
Soluble Guanylate Cyclase Stimulator
Riociguat Initial dose: 1 mg PO 8 hourly x 2 weeks
Increase by 0.5 mg in 2-week intervals to a max of 2.5 mg PO 8 hourly if BP ≥95 mmHg and without signs and symptoms of hypotension
Max dose: 7.5 mg/day 		Adverse Reactions
  • GI effects (nausea/vomiting, dyspepsia, diarrhea, GERD, gastritis, dysphagia, GI and abdominal pains, constipation, abdominal distension); CNS effects (dizziness, headache); CV effects (palpitations, hypotension, peripheral edema); Respiratory effects (hemoptysis, epistaxis, nasal congestion); Other effect (anemia)
Special Precautions
  • Contraindicated in patients concurrently or intermittently using PDE5 inhibitors, nitrates or nitric oxide, hypotension (<95 mmHg), severe hepatic impairment, pulmonary veno-occlusive disease
  • Use with caution in patients with ongoing antihypertensive treatment, resting hypotension, hypovolemia, severe left ventricular outflow obstruction, autonomic dysfunction, moderate renal or hepatic impairment
  • Adequate contraception should be used in women of childbearing age

Platelet Aggregation Inhibitors Excluding Heparin

Drug Dosage Remarks
Beraprost 60 mcg/day PO divided 8 hourly
May gradually increase dose, if
required, up to 180 mcg/day PO
divided 6-8 hourly		 Adverse Reactions
  • CNS effects (headache, dizziness); CV effect (hot flushes); GI effect (GI disturbances); Other effects (bleeding tendency, increased liver enzymes, triglycerides and bilirubin)

Special Instructions
  • Use with caution in patients on anticoagulants, antiplatelet or fibrinolytic agents, menstruating women, patients with bleeding tendency or diathesis
Epoprostenol Initial dose
Adult: 2 ng/kg/min continuous IV
Increase in increments of 2 ng/kg/min every 15 minutes or longer until max hemodynamic benefits/dose-limiting effects are elicited
Infuse with the rate of 4 ng/kg/min less than the max tolerated
infusion rate
If max rate is <5 ng/kg/min, then initial rate should be ½ the max rate
Children: 2 ng/kg/min IV
May increase to 40 ng/kg/min as
necessary
Maintenance dose
Adult: Adjust by 1-2 ng/kg/min
IV at intervals of ≥15 minutes based on response
May adjust dose by 1-2 ng/kg/min IV every 15 minutes or longer if symptoms recur or adverse reactions appear		 Adverse Reactions
  • CV effects (hypotension, bradycardia, tachycardia, pallor, flushing, chest pain); GI effects (GI disturbances, dry mouth); CNS effects (headache, anxiety, drowsiness); Respiratory effect (pulmonary edema); Musculoskeletal effects (myalgia, jaw pain, tremor); Other effects (infusion-site reactions, lassitude, hyperglycemia, flu-like symptoms, pallor, sweating)

Special Instructions
  • Avoid in patients with CHF due to severe left ventricular systolic dysfunction and in those who develop pulmonary edema during dose-ranging
  • Administer by continuous IV infusion via central venous catheter using ambulatory infusion pump; during dose-ranging, it may be administered peripherally
  • Avoid extravasation
  • Sudden withdrawal or large dose reductions of Epoprostenol should be avoided due to the risk of rebound PH
  • Use with caution in patients with hemorrhagic diathesis or coronary artery disease
  • Hematological and CV monitoring is required
Iloprost 2.5-5 mcg/dose 6-9x/day via
inhalation
Max dose: 45 mcg/day		 Adverse Reactions
  • CV effects (flushing, hypotension, syncope, palpitation); CNS effects (headache, insomnia); GI effects (nausea/vomiting); Neuromuscular effects (trismus, back pain, muscle cramps, jaw pain); Respiratory effects (cough, flu-like syndrome); Hepatic effect (LFT elevation)

Special Instructions
  • Administration using jet nebulizer, duration of each inhalation takes approximately 15 minutes and can be as low as 5 minutes with ultrasound nebulizers
  • Avoid in patients with hypotension (SBP <85 mmHg)
  • Use with caution in patients with conditions or medications that increase risk of syncope, use with caution in patients with hepatic impairment, active bleeding or at increased risk of bleeding
  • Discontinue therapy if pulmonary edema occurs during administration
  • Sudden withdrawal or large dose reductions should be avoided due to the risk of rebound PH
  • Decrease dose in patients with hepatic and renal impairment
Selexipag Initial dose: 200 mcg PO 12 hourly
May increase dose by 200 mcg PO 12 hourly at weekly intervals to max tolerated dose
Max dose: 1,600 mcg PO 12 hourly		 Adverse Reactions
  • GI effects (GI upset, decreased appetite, diarrhea, nausea/vomiting, abdominal pain); Respiratory effects (nasopharyngitis, nasal congestion, pulmonary edema); Musculoskeletal effects (myalgia, arthralgia, jaw pain, pain in extremity); CV effects (hypotension, flushing); Dermatologic effects (rash, burning sensation, erythema); Metabolic/endocrine effects (hyperthyroidism, weight loss, decreased TSH); Other effects (decreased Hb, anemia)

Special Instructions
  • Contraindicated in patients with severe hepatic impairment (Child-Pugh C); severe CAD, unstable angina, MI within 6 months; decompensated cardiac failure not under close medical supervision; cerebrovascular event within the past 3 months, severe arrhythmia
  • Use with caution in patients with renal or hepatic impairment, ongoing dialysis, hypotension; hypovolemia; severe LV obstruction, autonomic dysfunction; moderate, severe obstructive or restrictive lung disease
  • Adequate contraception should be used in women of childbearing age
Treprostinil Initial dose: 1.25 ng/kg/min
continuous SC infusion or
continuous infusion through
central IV catheter
May be reduced to 0.625 ng/kg/
min if side effects occur or dose cannot be tolerated
Infusion rate may be increased
based on patient response by increments of up to 1.25 ng/kg/min every week for the first 4 weeks
Followed by increases of up to 2.5 ng/kg/min each week
Target dose: 40-80 ng/kg/min
or
18-54 mcg (3-9 inhalations)
6 hourly or 0.25 mg PO 12 hourly or 0.125 mg PO 8 hourly
Increase by 0.125 mg PO 8 hourly or by 0.25 mg or 0.5 mg PO 12 hourly every 3-4 days		 Adverse Reactions
  • CV effects (vasodilation, edema, hypotension, flushing); CNS effects (headache, dizziness); Dermatologic effects (rash, pruritus); GI effects (diarrhea, nausea/vomiting)
  • Local effects: Infusion site pain that may improve over several months of therapy; infusion site reaction

Special Instructions
  • Use with caution in patients with hepatic or renal impairment
  • Abrupt withdrawal or large dose reductions may worsen PH symptoms

Disclaimer

All dosage recommendations are for non-pregnant and non-breastfeeding women, and non-elderly adults with normal renal and hepatic function unless otherwise stated.  
Not all products are available or approved for above use in all countries.  
Products listed in the Drug Summary are based on indications stated in the locally approved product monographs.   
Please refer to local product monographs in Related MIMS Drugs for country-specific prescribing information. 

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