From the Guidelines
Treatment for pulmonary hypertension (Pulm HTN) should prioritize vasodilator therapy, with first-line medications including phosphodiesterase-5 inhibitors like sildenafil (20mg three times daily) or tadalafil (40mg once daily), and endothelin receptor antagonists such as ambrisentan (5-10mg daily) or bosentan (62.5mg twice daily for 4 weeks, then 125mg twice daily) 1.
Key Considerations
- Initial management should focus on treating any underlying conditions such as left heart disease, lung disease, or chronic thromboembolic disease.
- For patients with pulmonary arterial hypertension (PAH), combination therapy is often required for optimal management.
- Supportive measures include oxygen therapy to maintain saturation above 90%, diuretics for right heart failure symptoms, and anticoagulation in specific cases.
- Lifestyle modifications include moderate exercise within tolerance, avoiding pregnancy, and maintaining vaccinations.
Medication Options
- Phosphodiesterase-5 inhibitors: sildenafil (20mg three times daily) or tadalafil (40mg once daily) 1.
- Endothelin receptor antagonists: ambrisentan (5-10mg daily) or bosentan (62.5mg twice daily for 4 weeks, then 125mg twice daily) 1.
- Prostacyclin analogs: epoprostenol (initiated at 2ng/kg/min and titrated upward) or treprostinil may be necessary for more severe disease 1.
Important Notes
- The evidence supporting the use of advanced vasoactive agents arises primarily from studies of patients with pulmonary arterial hypertension, and there is no consistent evidence that these agents confer clinical benefits in other types of pulmonary hypertension 1.
- Regular follow-up with pulmonary hypertension specialists is essential for monitoring disease progression and adjusting therapy 1.
From the FDA Drug Label
Epoprostenol for injection is indicated for the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise capacity. Studies establishing effectiveness included predominantly patients with NYHA Functional Class III-IV symptoms and etiologies of idiopathic or heritable PAH or PAH associated with connective tissue diseases.
The treatment for pulmonary hypertension is epoprostenol (IV), which is indicated to improve exercise capacity in patients with pulmonary arterial hypertension (PAH). The dosage is initiated at 2 ng/kg/min and increased in increments of 2 ng/kg/min every 15 minutes or longer until a tolerance limit to the drug is established or further increases in the infusion rate are not clinically warranted 2.
- Key points:
- Initiate chronic infusion of epoprostenol for injection at 2 ng/kg/min
- Increase in increments of 2 ng/kg/min every 15 minutes or longer
- Adjust the infusion by 1 ng/kg/min to 2 ng/kg/min increments at intervals sufficient to allow assessment of clinical response
- Avoid abrupt withdrawal of epoprostenol for injection or sudden large reductions in infusion rates 2.
From the Research
Treatment Options for Pulmonary Hypertension
- The treatment of pulmonary hypertension (PH) depends on the underlying cause and classification of the disease 3, 4.
- For patients with pulmonary arterial hypertension (PAH), targeted treatments such as phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin analogues, and prostacyclin receptor agonists are available 3, 5.
- Surgical pulmonary endarterectomy is the treatment of choice for eligible patients with chronic thromboembolic PH (CTEPH) 3, 4.
- Riociguat, a stimulator of soluble guanylate cyclase, is the only licensed targeted therapy for patients with inoperable or persistent/recurrent CTEPH 3, 4.
- Management of PH resulting from left-sided heart disease primarily involves treatment of the underlying condition 3.
Classification and Diagnosis
- PH is classified into 5 clinical subgroups: PAH, PH due to left-sided heart disease, PH due to chronic lung disease, CTEPH, and PH with an unclear and/or multifactorial mechanisms 3.
- Diagnosis of PH involves routine tests such as electrocardiography, chest radiography, and pulmonary function tests, as well as transthoracic echocardiography and right-sided heart catheterization 3.
Treatment Outcomes
- With current treatments, 5-year survival for patients with PAH has improved from 34% in 1991 to more than 60% in 2015 5.
- Combination drug therapy that targets multiple biological pathways has shown demonstrable improvement in morbidity and mortality compared to single-pathway targeted monotherapy 5.
- Three-year survival rates for patients with PAH have improved from 30%-40% to over 85% since the introduction of epoprostenol in 1999 6.