From the Guidelines
Pulmonary hypertension treatment should prioritize phosphodiesterase-5 inhibitors, such as sildenafil (20mg three times per day orally) and tadalafil (40mg daily orally), as first-line options, due to their ability to promote vasodilation by increasing nitric oxide availability, as supported by the most recent evidence from 1 and 1.
Medication Options
The treatment of pulmonary hypertension involves several medication classes, including:
- Phosphodiesterase-5 inhibitors: sildenafil (20mg three times per day orally) and tadalafil (40mg daily orally) 1
- Endothelin receptor antagonists: bosentan (62.5-125mg twice daily), ambrisentan (5-10mg daily), and macitentan (10mg daily) 1
- Prostacyclin analogs: epoprostenol (continuous IV infusion), treprostinil (subcutaneous, IV, inhaled, or oral), and iloprost (inhaled 6-9 times daily) 1
- Soluble guanylate cyclase stimulators: riociguat (0.5-2.5mg three times daily) 1
Treatment Approach
Treatment typically begins with oral monotherapy and may progress to combination therapy as needed, with the goal of reducing pulmonary vascular resistance, improving cardiac output, and enhancing exercise capacity and quality of life, as recommended by 1 and 1.
Key Considerations
When selecting a treatment option, it is essential to consider the specific type and severity of the condition, as well as the patient's individual needs and response to treatment, as emphasized by 1 and 1. Regular monitoring of liver enzymes and hemoglobin levels is also crucial when using endothelin receptor antagonists, due to the risk of hepatotoxicity and anemia.
From the FDA Drug Label
1 INDICATIONS AND USAGE Epoprostenol for injection is indicated for the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise capacity.
PATIENT COUNSELING INFORMATION ... Sildenafil tablets are a prescription medicine used in adults to treat pulmonary arterial hypertension (PAH).
Pulmonary Hypertension Medication Options:
- Epoprostenol (IV): indicated for the treatment of pulmonary arterial hypertension (PAH) to improve exercise capacity 2.
- Sildenafil (PO): used in adults to treat pulmonary arterial hypertension (PAH) 3 3.
Key Points:
- Epoprostenol and sildenafil are both used to treat PAH.
- Epoprostenol is administered intravenously, while sildenafil is taken orally.
- Sildenafil should not be taken with nitrate medicines, and patients should seek immediate medical attention for sudden loss of vision or hearing 3.
From the Research
Pulmonary Hypertension Medication Options
- The treatment armamentarium of approved drugs for PAH has expanded to include prostacyclin analogues, a dual endothelin receptor antagonist, and a phosphodiesterase-5 inhibitor 4.
- Targeted treatments, such as phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin analogues, and prostacyclin receptor agonists, are licensed for patients with PAH 5.
- Sotatercept has been introduced as a second-line therapy, leading to a revised definition of maximal medical therapy that now encompasses agents from four therapeutic groups: phosphodiesterase-5 inhibitors/soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin pathway agents, and sotatercept 6.
- Phosphodiesterase type 5 inhibitors, such as sildenafil and tadalafil, are recommended first-line treatments for PAH patients in World Health Organization functional classes II or III 7.
- Endothelin receptor antagonists have been shown to improve pulmonary hemodynamics, exercise capacity, functional status, and clinical outcome in patients with PAH 8.
Classes of Medications
- Prostacyclin analogues: used to improve exercise tolerance, functional class, and hemodynamics in PAH patients 4.
- Endothelin receptor antagonists: used to improve pulmonary hemodynamics, exercise capacity, functional status, and clinical outcome in PAH patients 5, 8.
- Phosphodiesterase-5 inhibitors: used to improve clinical status, exercise capacity, and hemodynamics in PAH patients 5, 7.
- Soluble guanylate cyclase stimulators: used to improve exercise capacity and hemodynamics in PAH patients 5.
- Prostacyclin receptor agonists: used to improve exercise capacity and hemodynamics in PAH patients 5.
Combination Therapies
- Combination therapy of PDE-5 inhibitors with prostacyclin analogs and endothelin receptor antagonists may be helpful in the management of PAH, although further studies are needed in this area 7.
- The use of sotatercept in combination with other therapies has been introduced as a second-line therapy for PAH patients 6.