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.

Supportive Therapy

Oral Anticoagulant

  • Eg Warfarin
  • Oral anticoagulation is associated with improved survival in idiopathic pulmonary arterial hypertension (IPAH) patients & should also be considered in heritable pulmonary arterial hypertension (PAH), PAH due to anorexigen use & associated pulmonary arterial hypertension (APAH)
  • Anticoagulation in patients with PAH associated with underlying diseases such as scleroderma or congenital heart disease is controversial & 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 & clinical benefits are seen in PAH patients with right heart failure
  • Choice & type of diuretics need to be individualized
  • Serum electrolytes & renal function should be monitored closely in those receiving diuretics


  • Digoxin produces favorable acute hemodynamic effects in patients with right heart failure & 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 & desaturation during exercise is corrected
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