What are the treatment options for pulmonary hypertension (PH)?

From the Guidelines

The treatment of pulmonary hypertension should prioritize the use of phosphodiesterase-5 inhibitors, such as sildenafil (20 mg every 8 h) or tadalafil (40 mg once daily), as first-line therapy, with the option to add endothelin receptor antagonists like bosentan (125 mg twice daily) or ambrisentan (5 or 10 mg once daily) for combination therapy, as recommended by the most recent guidelines 1.

When considering the management of pulmonary arterial hypertension, it's essential to focus on the most effective and safe treatment options. The current guidelines suggest the following medications for treatment:

• Prostacyclin derivatives: epoprostenol (2 ng/kg/min IV infusion), iloprost (2.5 or 5.0 mg 6-9 inhalations/d), and treprostinil (0.25 mg bid or 0.125 mg tid oral, or 18–54 mg 4 times daily inhaled)
• Endothelin receptor antagonists: bosentan (125 mg twice daily), ambrisentan (5 or 10 mg once daily), and macitentan (10 mg once daily)
• Phosphodiesterase type-5 inhibitors: sildenafil (20 mg every 8 h) and tadalafil (40 mg once daily)
• Soluble cGMP stimulators: riociguat (0.5-1.0 mg every 8 h)
• Prostacyclin receptor agonists: selexipag (200 mg twice daily)

The selection of therapy should be based on the patient's specific condition, disease severity, and response to treatment. Combination therapy is often necessary to achieve optimal results, and supportive measures like oxygen therapy, diuretics, and anticoagulation may be added as needed 1.

In terms of non-pharmacologic interventions, cardiopulmonary rehabilitation, palliative care, supportive care, and preventive care are also essential components of pulmonary hypertension management, as they can improve patient outcomes and quality of life 1.

It's crucial to note that the treatment of pulmonary hypertension should be individualized, and the choice of therapy should be guided by the most recent and highest-quality evidence, with a focus on minimizing morbidity, mortality, and improving quality of life 1.

From the FDA Drug Label

Sildenafil tablets are a prescription medicine used in adults to treat pulmonary arterial hypertension (PAH). Epoprostenol for injection is a prostacyclin vasodilator indicated for the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise capacity.

Treatment of Pulmonary Hypertension:

• Sildenafil tablets are used to treat pulmonary arterial hypertension (PAH) in adults.
• Epoprostenol for injection is used to treat PAH (WHO Group 1) to improve exercise capacity. The dosage of sildenafil tablets is 3 times a day about 4 to 6 hours apart 2. The dosage of epoprostenol for injection is not specified in the provided text 3.

From the Research

Treatment Options for Pulmonary Hypertension

• The treatment of pulmonary arterial hypertension has advanced substantially over the past 20 years, with five different classes of drugs available, including endothelin receptor antagonists, phosphodiesterase-5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogues, and prostacyclin receptor agonists 4.
• For patients with chronic thromboembolic pulmonary hypertension, surgical pulmonary endarterectomy is the treatment of choice, while riociguat, a stimulator of soluble guanylate cyclase, has proven efficacious for inoperable patients 4, 5.
• 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 pulmonary arterial hypertension 5.
• Management of pulmonary hypertension resulting from left-sided heart disease primarily involves treatment of the underlying condition 5.

Current and Emerging Therapies

• Traditional medications for treating pulmonary arterial hypertension include prostacyclin analogs and receptor agonists, phosphodiesterase 5 inhibitors, endothelin-receptor antagonists, and cGMP activators 6.
• Current FDA-approved drugs have shown improvements in quality of life and hemodynamic parameters, but have limited beneficial effects on survival and disease progression 6.
• Innovative therapeutic approaches, such as stem cell-based therapies, gene transfer, and epigenetic therapies, are being explored in preclinical studies 6.

Diagnosis and Treatment of Pulmonary Arterial Hypertension

• Pulmonary arterial hypertension is characterized by pulmonary arterial remodeling and is defined by a mean pulmonary artery pressure greater than 20 mm Hg 7.
• Early presenting symptoms are nonspecific and typically consist of dyspnea on exertion and fatigue 7.
• Currently approved therapy for pulmonary arterial hypertension consists of drugs that enhance the nitric oxide-cyclic guanosine monophosphate biological pathway, prostacyclin pathway agonists, and endothelin pathway antagonists 7.
• Combination drug therapy that targets multiple biological pathways has shown demonstrable improvement in morbidity and mortality compared to single-pathway targeted monotherapy 7, 8.