From the Guidelines
Pulmonary hypertension is typically treated with several medication classes, including phosphodiesterase-5 inhibitors, endothelin receptor antagonists, prostacyclin analogs, and soluble guanylate cyclase stimulators, with treatment guided by the severity of the condition and patient response, as recommended by the most recent guidelines 1.
Medication Classes
- Phosphodiesterase-5 inhibitors: sildenafil (20 mg every 8 h) or tadalafil (40 mg once daily) 1
- Endothelin receptor antagonists: bosentan (125 mg twice daily), ambrisentan (5 or 10 mg once daily), or macitentan (10 mg once daily) 1
- Prostacyclin analogs: epoprostenol (continuous IV infusion starting at 2 ng/kg/min), treprostinil (subcutaneous, IV, inhaled, or oral forms), or iloprost (inhaled 6-9 times daily) 1
- Soluble guanylate cyclase stimulators: riociguat (0.5-1.0 mg every 8 h) 1
- Prostacyclin receptor agonists: selexipag (200 mg twice daily, increased as tolerated to a maximum dose of 1,600 mg twice daily) 1
Treatment Approach
Treatment typically begins with oral medications for less severe cases, progressing to more intensive therapies as needed, with combination therapy using multiple drug classes increasingly common for better symptom control 1.
Supportive Treatments
Diuretics, oxygen therapy, and anticoagulants may be added as supportive treatments, with regular monitoring of liver function, blood counts, and clinical response essential due to potential significant side effects of these medications 1.
Guideline Recommendations
The most recent guidelines recommend a patient-centered approach, considering the individual's disease severity, response to treatment, and quality of life when selecting pharmacologic therapies for pulmonary arterial hypertension 1.
From the Research
Pulmonary Hypertension Medications
- Pulmonary hypertension medications include endothelin receptor antagonists, such as bosentan, ambrisentan, and sitaxsentan, which have been shown to improve exercise capacity and hemodynamics in patients with pulmonary hypertension 2, 3, 4.
- These medications work by blocking the action of endothelin-1, a potent vasoconstrictor that is overexpressed in patients with pulmonary hypertension 3, 4.
- Other medications used to treat pulmonary hypertension include phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, and prostacyclin analogues 5.
- The choice of medication depends on the severity and underlying cause of the pulmonary hypertension, as well as the patient's individual needs and medical history 3, 5, 4.
Endothelin Receptor Antagonists
- Bosentan is a dual endothelin receptor antagonist that has been shown to improve exercise capacity and hemodynamics in patients with pulmonary hypertension 2, 6.
- Ambrisentan is a selective endothelin receptor antagonist that has been shown to improve exercise capacity and delay clinical worsening in patients with pulmonary hypertension 3, 6.
- Sitaxsentan is another selective endothelin receptor antagonist that has been approved for use in Europe, Canada, and Australia 3, 4.
Treatment of Pulmonary Hypertension
- Treatment of pulmonary hypertension depends on the underlying cause and severity of the disease 5.
- Patients with pulmonary arterial hypertension (PAH) may be treated with targeted therapies, such as endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, and prostacyclin analogues 5.
- Patients with chronic thromboembolic pulmonary hypertension (CTEPH) may be treated with surgical pulmonary endarterectomy or targeted therapies, such as riociguat 5.
- Patients with pulmonary hypertension due to left-sided heart disease or chronic lung disease may be treated with medications that target the underlying condition 5.