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
- Anticoagulation therapy: Anticoagulation is recommended in certain types of PH. For instance, lifelong therapeutic doses of anticoagulation are recommended for all patients with chronic thromboembolic pulmonary hypertension (CTEPH) . In patients with connective tissue disease–associated pulmonary arterial hypertension, long-term warfarin therapy is recommended . However, the use of anticoagulation in PH is based on retrospective evidence and expert opinion, and its real benefit in different types of PH is still debatable
- Setting of care: All patients with pulmonary arterial hypertension (PAH) should be assessed in a recognized PH center to confirm the diagnosis, direct institution of PH-targeted therapies, and evaluate the response to treatment
PAH-specific therapy
- Sequential therapy: The European Respiratory Society/European Society of Cardiology (ERS/ESC) 2022 guidelines recommend various sequential therapies for PAH. For instance, sildenafil can be added to epoprostenol therapy to improve exercise capacity . Oral treprostinil can be added to endothelin receptor antagonist or PDE5 inhibitor/riociguat monotherapy to reduce the risk of morbidity/mortality events . Macitentan can be added to PDE5 inhibitor or oral/inhaled prostacyclin analog therapy to reduce the risk of morbidity/mortality events
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