Approach to managing pulmonary hypertension

The management of pulmonary hypertension is primarily guided by the aetiology and severity; options are:

• treatment of reversible causes
• nonspecific therapies such as anticoagulation, oxygen therapy, diuretics and digoxin
• specific therapies, which are only available on the Pharmaceutical Benefits Scheme (PBS) for patients with pulmonary hypertension who meet specific criteria and are managed by a clinician with expertise in treating pulmonary hypertension.

Pulmonary hypertension is often attributable to a cause (eg pulmonary, vascular, inflammatory or toxic disorders). Some causes require specific management to correct or reduce the pulmonary pressure (eg a loop diuretic for fluid overload associated with heart failure). Other causes (eg pulmonary hypertension secondary to chronic obstructive pulmonary disease) do not require specific management to reduce the pulmonary pressure, and are treated with standard therapy for the primary disease.

Patients of childbearing potential should use reliable contraception and discuss pregnancy planning with their pulmonary hypertension specialist—see Pregnancy and contraception in pulmonary hypertension.

Pulmonary hypertension is associated with a poor prognosis. Although drug therapy and other measures improve outcomes for patients with pulmonary arterial hypertension, pulmonary arterial hypertension should still be recognised as a progressive and life-limiting illness. Early integration of a palliative approach into the patient’s care should be considered. This allows supports to be put in place and gives the patient time to consider their life goals and preferences. See Principles of palliative care for nonmalignant respiratory disease for more detailed advice. See also When can palliative care be introduced? for general information about early introduction of a palliative approach.