Pulmoten 62.5

• Pulmonary arterial hypertension (PAH) - WHO functional class II and III

Adults (>12 years, >40 kg):
First 4 weeks: 62.5 mg twice daily
Maintenance dose: 125 mg twice daily

Children (≥2 years): 2 mg/kg twice daily (optimal maintenance dose not established in controlled studies)

Elderly (≥65 years): No dose adjustment needed

Hepatic impairment: Mild (Child-Pugh A): No dose adjustment; Moderate-severe (Child-Pugh B/C): Contraindicated

Renal impairment: No dose adjustment needed; dialysis patients also require no adjustment

Discontinuation: Do not stop abruptly (withdraw gradually over 3-7 days by reducing to half the dose to minimize risk of clinical deterioration)

Common side effects:
Headache, ankle swelling (edema), diarrhea, anemia (hemoglobin decrease), respiratory tract infection.

Hepatic: Dose-dependent elevations in liver aminotransferases, hepatitis, jaundice, rare cases of liver cirrhosis and liver failure.

Other: Thrombocytopenia, neutropenia, leukopenia, hypersensitivity reactions, syncope, palpitations, flushing, hypotension, gastroesophageal reflux disease, erythema.

• Liver aminotransferases (AST/ALT) must be measured prior to initiation and monthly during treatment; measure 2 weeks after any dose increase.
• Monitor hemoglobin for anemia.
• Do not stop abruptly (withdraw gradually over 3-7 days by reducing to half the dose to minimize risk of clinical deterioration).
• Consider discontinuation if pulmonary edema occurs.
• Highly unsafe during pregnancy (Category X); women of reproductive potential must use reliable contraception during and for 1 month after stopping treatment.
• May cause dizziness; know your response before driving or operating machinery.
• Inform your doctor if you notice yellowing of skin/eyes, dark urine, stomach pain, weight gain, swelling of arms/legs, or difficulty breathing.