From the Guidelines
Treatment options for pulmonary hypertension (PH) include pharmacologic therapy, surgical interventions, and supportive care.
Treatment Options
- Pharmacologic therapy: includes drugs such as calcium channel blockers, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostacyclin analogs, which are approved for the treatment of pulmonary arterial hypertension (PAH) 1.
- Surgical interventions: such as pulmonary thromboendarterectomy, thoracic transplantation, and atrial septostomy, which are important for patients with chronic thromboembolic pulmonary hypertension (CTEPH) or other forms of PH that are unresponsive to medical therapy 1.
- Supportive care: includes diuretics, supplemental oxygen, and other measures to manage symptoms and improve quality of life 1.
Specific Treatment Approaches
- PAH: treatment is based on the patient's World Health Organization (WHO) functional classification, and may include combination therapy with multiple drugs 1.
- CTEPH: surgical thromboendarterectomy is the optimal therapy for many patients, and balloon pulmonary angioplasty (BPA) is a newly approved treatment option 1.
- PH due to left heart disease: treatment is focused on managing the underlying left heart disease, rather than using PAH-specific therapies 1.
Importance of Expert Referral Centers
- Expert referral centers: play a critical role in the management of PH patients, and are essential for providing accurate diagnosis, treatment, and follow-up care 1.
From the Research
Treatment Options for Pulmonary Hypertension (PH)
The treatment options for PH can be categorized into several approaches, including:
- Medical therapy: This includes the use of targeted pulmonary vasodilator therapies such as endothelin receptor antagonists, prostacyclin analogues, and phosphodiesterase type 5 inhibitors 2, 3, 4, 5.
- Supportive therapy: This includes the administration of diuretics, iron supplementation, and oral anticoagulant 3, 4.
- Surgical procedures: This includes atrial septostomy, thromboendartectomy, and lung or heart-lung transplantation 2, 4, 5.
- General measures: This includes vaccinations and physical training 3.
Specific Treatment Options for Different Forms of PH
- Pulmonary arterial hypertension (PAH): Targeted disease modifiers such as calcium antagonists, prostanoids, endothelin receptor antagonists, and phosphodiesterase type 5 inhibitors are used to treat PAH 2, 4, 5.
- Chronic thromboembolic pulmonary hypertension (CTEPH): Pulmonary endarterectomy is the preferred method of management for CTEPH, and lung transplantation remains a life-saving option for those who experience progressive disease with medical therapy 2, 5.
- Pulmonary hypertension due to other lung or heart diseases: Treatment primarily targets the underlying cause, and emerging treatments are being developed for these forms of PH 6, 5.
Challenges and Future Directions
Despite the advancements in the treatment of PH, there are still major gaps in basic, clinical, and translational knowledge, and further research is needed to better characterize, detect, and effectively treat all forms of PH 6, 5.