Supportive therapy

The general treatment of respiratory disease and right heart failure are essential to the management of PAH patients.

It is recommended

  • To introduce diuretic treatment in PAH patients with signs of right ventricular failure and fluid retention [I, C].
  • To introduce continuous long-term O₂ therapy in PAH patients when arterial blood O₂ pressure is consistently less than 8 kPa (60 mmHg) [I, C].
  • To consider oral anticoagulant treatment in patients with IPAH (group 1.1), heritable PAH (group 1.2), and PAH due to use of anorexigens (group 1.3) [IIa, C]. Oral anticoagulant treatment may be considered in patients with PAH associated with diseases of group 1.4. [IIb, C]. Hemorrhagic risk must be carefully evaluated, especially for patients with porto-pulmonary hypertension (group 1.4.2).
  • To consider use of digoxin in PAH patients who develop atrial tachyarrhythmia [IIb, C].