General measures

Pulmonary arterial hypertension (PAH) is a chronic disease with a dismal prognosis that is comparable to some neoplasia if left untreated. Specific drug therapy of PAH has significantly improved prognosis for many patients. However, management of PAH patients remains complex, several drugs and combinations are available and thus, treatment of PAH should be in expert centers in order to address all aspects of this complex disease, including regular follow-up, counseling about drug combination therapies, rehabilitation and daily activities.

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].