From the Guidelines
The recommended treatment for pulmonary hypertension is a combination of pharmacotherapies, including prostacyclin derivatives, endothelin receptor antagonists, phosphodiesterase type-5 inhibitors, and soluble cGMP stimulators, as outlined in the 2019 Chest guideline update 1. The treatment approach should be individualized based on the specific type and severity of the condition, as well as the patient's response to therapy.
- For pulmonary arterial hypertension (PAH), initial therapy may include:
- Prostacyclin derivatives, such as epoprostenol (2 ng/kg/min IV infusion) or treprostinil (0.25 mg bid or 0.125 mg tid oral) 1
- Endothelin receptor antagonists, such as bosentan (125 mg twice daily oral) or ambrisentan (5 or 10 mg once daily oral) 1
- Phosphodiesterase type-5 inhibitors, such as sildenafil (20 mg every 8 h oral) or tadalafil (40 mg once daily oral) 1
- Soluble cGMP stimulators, such as riociguat (0.5-1.0 mg every 8 h oral) 1
- Combination therapy is often required for optimal management, and the choice of medications should be based on the patient's specific needs and response to therapy. The goal of treatment is to improve exercise capacity, reduce symptoms, and slow disease progression, while also minimizing side effects and improving quality of life.
- Regular monitoring of disease progression, medication side effects, and functional capacity is crucial for optimal management, and may include assessments of 6-minute walk distance (6MWD) and World Health Organization (WHO) functional class 1.
From the FDA Drug Label
Selexipag tablets are indicated for the treatment of pulmonary arterial hypertension (PAH, WHO Group I) to delay disease progression and reduce the risk of hospitalization for PAH. Sildenafil tablets are a prescription medicine used in adults to treat pulmonary arterial hypertension (PAH). Epoprostenol for injection is indicated for the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise capacity.
The recommended treatments for pulmonary hypertension are:
- Selexipag tablets, with a starting dosage of 200 micrograms twice daily, increased in increments of 200 micrograms twice daily, up to 1,600 micrograms twice daily 2
- Sildenafil tablets, taken 3 times a day, about 4 to 6 hours apart 3
- Epoprostenol for injection, to improve exercise capacity in patients with PAH (WHO Group 1) 4
From the Research
Treatment Options for Pulmonary Hypertension
The treatment for pulmonary hypertension depends on the severity and underlying cause of the condition. Some of the recommended treatment options include:
- Endothelin receptor antagonists, such as bosentan 5, 6
- Phosphodiesterase-5 inhibitors
- Prostanoids, such as epoprostenol 7, 8
- Soluble guanylate cyclase stimulators, such as riociguat 9
- Prostacyclin receptor agonists, such as selexipag 9
Combination Therapy
Combination therapy, which involves the use of multiple medications, may be recommended for patients with pulmonary hypertension 9, 7. The choice of combination therapy depends on the individual patient's needs and risk stratification.
Intravenous Epoprostenol
Intravenous epoprostenol is a synthetic prostacyclin that has been shown to reduce mortality in patients with pulmonary hypertension 8. It is recommended for patients with high-risk pulmonary hypertension and may be considered for patients with intermediate-risk disease.
Key Considerations
When selecting a treatment for pulmonary hypertension, it is essential to consider the following factors: