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treatment of pulmonary hypertension

Answer

The treatment of pulmonary hypertension (PH) is multifaceted and depends on the specific type of PH, the underlying cause, and the patient's clinical status.

General management

PAH-specific therapy

Specific circumstances

  • Patients with connective tissue disease: The British Society for Rheumatology (BSR) 2024 guidelines recommend the use of PDE5 inhibitors (tadalafil, sildenafil), endothelin receptor antagonists (ambrisentan, macitentan, bosentan), prostaglandins (inhaled iloprost, IV epoprostenol, subcutaneous or inhaled treprostinil), prostacyclin receptor agonist (selexipag), and soluble guanylate cyclase stimulator (riociguat)
  • Patients with Eisenmenger syndrome: The ERS/ESC 2022 guidelines recommend initiating endothelin receptor antagonists, phosphodiesterase 5 inhibitors, riociguat, prostacyclin analogs, and prostacyclin receptor agonists for these patients
  • Patients with CTEPH: For patients with inoperable CTEPH, the ERS/ESC 2022 guidelines recommend initiating combination therapy with soluble guanylate cyclase stimulators/phosphodiesterase 5 inhibitors, endothelin receptor antagonists, or parenteral prostacyclin analogs

In conclusion, the treatment of PH is complex and requires a personalized approach based on the specific type of PH, the underlying cause, and the patient's clinical status. The goal of treatment is to improve the patient's biochemical, hemodynamic, and functional profile, and limit adverse events

References

1

Pulmonary hypertension

Medical management

Guidelines

PAH-specific therapy, sequential therapy

Pathway
ERS/.../ESC 2022