What is the primary treatment for patients with elevated pulmonary arterial pressure?

From the Guidelines

The primary treatment for patients with elevated pulmonary arterial pressure involves pulmonary vasodilator therapy, with endothelin receptor antagonists, such as bosentan, being a first-line therapy option for patients in functional class III. According to the study by 1, bosentan, an orally active nonpeptide antagonist of both endothelin-receptor subtypes (ETA and ETB), prevents and even reverses the development of pulmonary hypertension, pulmonary vascular remodeling, and right ventricular hypertrophy.

Key Treatment Options

• Endothelin receptor antagonists, such as bosentan 62.5mg twice daily initially, increasing to 125mg twice daily, for patients in functional class III
• Phosphodiesterase-5 inhibitors, such as sildenafil, may be considered for patients who have failed or are not candidates for other available therapy
• Prostacyclin analogs, such as epoprostenol, may be used for patients in functional class IV or those who have failed other therapies
• Combination therapy is often necessary, with many patients requiring two or more medication classes simultaneously

Important Considerations

• Treatment should be initiated promptly after diagnosis and requires regular monitoring of clinical response, including exercise capacity, right heart function, and symptom improvement
• Supportive measures include oxygen therapy for hypoxemia, diuretics for fluid overload, anticoagulation in selected cases, and management of underlying conditions
• For patients who fail medical therapy, lung transplantation may be considered as a last resort, as noted in the study by 1.

From the FDA Drug Label

The effects of epoprostenol on mean pulmonary arterial pressure (PAPm) were variable and minor Decreases in PAPm ... were observed in patients who received epoprostenol chronically compared to those who did not. PAPm (mmHg) 60 60 -5† 1

The primary treatment for patients with elevated pulmonary arterial pressure is epoprostenol (IV), which has been shown to decrease mean pulmonary arterial pressure (PAPm) by 5 mmHg in patients with idiopathic or heritable PAH 2, 2, 2.

• Key benefits of epoprostenol include:
  • Decreases in PAPm and pulmonary vascular resistance (PVR)
  • Increases in cardiac index (CI) and stroke volume (SV)
  • Improvements in exercise capacity and survival in patients with PAH.

From the Research

Primary Treatment for Patients with Elevated Pulmonary Arterial Pressure

The primary treatment for patients with elevated pulmonary arterial pressure, also known as pulmonary arterial hypertension (PAH), involves a combination of medications and therapies that target the underlying pathobiologic pathways of the disease.

• The main goals of treatment are to:
  • Reduce pulmonary vascular resistance
  • Improve exercise capacity and quality of life
  • Slow disease progression
• Approved therapies for PAH include:
  • Phosphodiesterase type-5 inhibitors (e.g., sildenafil, tadalafil) 3
  • Soluble guanylate cyclase stimulators
  • Endothelin receptor antagonists
  • Prostacyclin analogs
  • Prostacyclin receptor agonists 4, 5, 6
• Current guidelines recommend:
  • Two oral medications (phosphodiesterase type-5 inhibitor and endothelin receptor antagonist) for low- and intermediate-risk patients 4
  • Triple therapy, including a parenteral prostacyclin, for high- or intermediate-high risk patients 4, 6
  • A single-agent approach with individualized decisions to add-on other PAH therapies for older patients and those with significant comorbid conditions 4
• Other core pillars of PAH management include:
  • Supportive and adjunctive treatments (e.g., oxygen, diuretics, rehabilitation, anticoagulation) 4, 7
  • Referral for lung transplantation for patients who progress despite optimal treatment or are refractory to best medical care 4