General measures

Pulmonary arterial hypertension (PAH) is a chronic disease with a prognosis that is comparable to some neoplasia. By targeting the causes of the vascular remodeling, the emerging specific drug therapy of PAH offers now encouraging perspectives including stabilization or even improvement of the disease. However, management of PAH patients must be global, as it is for other chronic diseases, with adaptation of daily activities, counseling and prevention of a possible social isolation.

It is recommended

  • To practice physical activity that causes only slight breathlessness without any other symptoms (chest pain, fainting, severe dyspnea) [Class of recommendation IIa, level of evidence B].
  • For PAH patients with physical deconditioning to consider supervised exercise rehabilitation in a competence centre for PAH [IIa, B].
  • To vaccinate against influenza and pneumococcal pneumonia [I, C].
  • For women of childbearing age to adopt an effective method of contraception (it should be remembered that the endothelin receptor antagonist (ERA) bosentan may reduce the efficacy of oral contraceptive agents). Emphasis must be made on the high mortality risk associated with pregnancy [I, C].
  • For air flight or travel to an altitude above 1500 – 2000 meters, consider oxygen therapy (2l/min) in patients with functional class NYHA III-IV or hypoxemia (PaO₂< 8 kPa) [IIa, C].
  • To consider psychosocial support [IIa, C].
  • Epidural anaesthesia instead of general anaesthesia should be utilised, if possible, for elective surgery [IIa, C] and performed at a tertiary center with intensive care unit and availability of extracorporeal circulation.

It should be avoided

  • To perform physical activity that leads to distressing symptoms [III, C].
  • To use sauna, hamman or hot showers since it can lead to acute circulatory failure and sudden death [III, C].
  • To plan pregnancy. The patient who becomes pregnant should be informed of the high risk of pregnancy, and termination of pregnancy discussed [I, C].
  • To use beta-blockers because it can worsen hemodynamics and exercise capacity particularly in porto-pulmonary hypertension [III, C].