From the Guidelines
Pulmonary hypertension treatment should prioritize targeted therapies based on disease classification, including phosphodiesterase-5 inhibitors, endothelin receptor antagonists, prostacyclin analogs, or soluble guanylate cyclase stimulators, as supported by the most recent evidence from 1.
Treatment Options
The treatment of pulmonary hypertension involves a range of medications and therapies aimed at managing symptoms, slowing disease progression, and improving quality of life. Key treatment options include:
- Phosphodiesterase-5 inhibitors such as sildenafil (20mg three times daily) or tadalafil (40mg daily) 1
- Endothelin receptor antagonists like bosentan (62.5mg twice daily, increasing to 125mg twice daily after 4 weeks), ambrisentan (5-10mg daily), or macitentan (10mg daily) 1
- Prostacyclin analogs including epoprostenol (IV starting at 2ng/kg/min, titrated upward), iloprost (inhaled, initial dose 2.5μg, maintenance 2.5-5μg per dose), or treprostinil (subcutaneous or IV infusion, starting at 0.625-1.25ng/kg/min) 1
- Soluble guanylate cyclase stimulators like riociguat (initial dose 0.5-1mg three times daily, titrated up to 2.5mg) 1
Combination Therapy and Supportive Measures
Combination therapy is often necessary for optimal results, and supportive measures such as oxygen therapy, diuretics for fluid overload, and anticoagulation in specific cases are also crucial 1. Lifestyle modifications, including moderate exercise, sodium restriction, and avoiding pregnancy, are important components of comprehensive management.
Disease Monitoring and Advanced Cases
Regular monitoring of functional capacity, echocardiography, and right heart catheterization guides treatment adjustments. Advanced cases may require atrial septostomy or lung transplantation, as outlined in guidelines and expert panel reports 1. The choice of therapy should be guided by the most recent and highest quality evidence, prioritizing patient outcomes in terms of morbidity, mortality, and quality of life.
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 treatment for pulmonary hypertension includes:
- Selexipag (PO): to delay disease progression and reduce the risk of hospitalization for PAH 2
- Sildenafil (PO): to improve the ability to exercise and can slow down worsening changes in physical condition 3
- Epoprostenol (IV): to improve exercise capacity 4
From the Research
Treatment Options for Pulmonary Hypertension
- The treatment of pulmonary hypertension (PH) depends on the underlying cause and severity of the disease 5.
- For patients with chronic thromboembolic PH (CTEPH), surgical pulmonary endarterectomy is the treatment of choice for eligible patients 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 (PAH) 5, 6, 7.
- The soluble guanylate cyclase stimulator riociguat is the only licensed targeted therapy for patients with inoperable or persistent/recurrent CTEPH 5.
- Management of PH resulting from left-sided heart disease primarily involves treatment of the underlying condition 5.
Combination Therapy for PAH
- Combination drug therapy that targets more than one biological pathway has shown demonstrable improvement in morbidity and mortality compared with single-pathway targeted monotherapy 7, 8.
- Adding bosentan to sildenafil therapy in PAH patients has been investigated, and while it was not superior to sildenafil monotherapy in delaying the time to the first morbidity/mortality event, it did show an improvement in 6-min walk distance 8.
- Weaning from intravenous epoprostenol to oral therapy with bosentan or sildenafil is possible, even with persistent pulmonary hypertension, but patients with persistently abnormal hemodynamics are at risk for hemodynamic and clinical deterioration and require close follow-up 9.
Current Therapies for PAH
- Currently approved therapies for PAH include drugs that enhance the nitric oxide-cyclic guanosine monophosphate biological pathway, prostacyclin pathway agonists, and endothelin pathway antagonists 6, 7.
- These therapies have improved 5-year survival rates for PAH patients from 34% in 1991 to more than 60% in 2015 7.