Clinical context

Patients with pulmonary arterial hypertension (PAH) require ongoing, structured general management throughout the entire course of their disease — not only during acute episodes, but as a sustained component of care alongside any disease-specific therapy.

Treatment approach

General supportive care for PAH encompasses a defined set of evidence-based measures — among them, supervised exercise training for patients under medical therapy, alongside additional targeted interventions applied according to each patient's individual clinical status.

The complete set of recommended measures, their specific indications, and the full structured regimen are available in the protocol below.

References

DOI: 10.1093/eurheartj/ehac237

Supervised exercise training is recommended in patients with PAH under medical therapy.
Diuretic treatment is recommended in patients with PAH with signs of RV failure and fluid retention.
The three main classes of diuretics — loop diuretics, thiazides, and mineralocorticoid receptor antagonists — are used as monotherapy or in combination, as determined by the patient's clinical need and kidney function.
Long-term oxygen therapy is recommended in patients with PAH whose arterial blood oxygen pressure is <8 kPa (60 mmHg).
In the presence of iron-deficiency anaemia, correction of iron status is recommended in patients with PAH.
In patients with severe iron deficiency anaemia (Hb <7–8 g/dL), i.v. supplementation is recommended.
Immunization of patients with PAH against SARS-CoV-2, influenza, and Streptococcus pneumoniae is recommended.
Psychosocial support is recommended in patients with PAH.

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Structured General Supportive Treatment for Pulmonary Arterial Hypertension

References