Treatment Guideline Chart
Pulmonary arterial hypertension is a syndrome resulting from restricted flow through the pulmonary arterial circulation resulting in increased pulmonary vascular resistance and ultimately leading to right heart failure.
It is a part of the spectrum of pulmonary hypertension, which is hemodynamic and pathophysiological condition defined as an increase in mean pulmonary arterial pressure ≥25 mmHg at rest.
Typical symptoms include progressive dyspnea on exertion, palpitations, fatigue, weakness, angina, syncope and abdominal distention.

Pulmonary%20arterial%20hypertension Treatment

Supportive Therapy

Oral Anticoagulant

  • Eg Warfarin
  • Oral anticoagulation is associated with improved survival in idiopathic pulmonary arterial hypertension (IPAH) patients and should also be considered in heritable pulmonary arterial hypertension (PAH), PAH due to anorexigen use and associated pulmonary arterial hypertension (APAH)
  • Anticoagulation in patients with PAH associated with underlying diseases such as scleroderma or congenital heart disease is controversial and one must consider risk-benefit ratio before starting Warfarin
  • Patients on long-term intravenous (IV) Epoprostenol in the absence of contraindications should be anticoagulated to prevent catheter-associated thrombosis
  • Therapeutic international normalized ratio (INR) can vary depending on the center: 1.5-2.5 or 2.0-3.0


  • Symptomatic and clinical benefits are seen in PAH patients with right heart failure
  • Choice and type of diuretics need to be individualized
  • Serum electrolytes and renal function should be monitored closely in those receiving diuretics


  • Digoxin produces favorable acute hemodynamic effects in patients with right heart failure and PAH with atrial tachyarrhythmia
    • Long-term benefits are unknown 
  • Use of Digoxin should be based on the judgment of the practitioner

Oxygen (O2) Therapy

  • Advised when oxygen saturation (SaO2) is consistently <60 mmHg to achieve SaO2 >60 mmHg for at least 15 hour/day
  • Ambulatory O2 may be given when there is improvement of symptoms and desaturation during exercise is corrected
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