What is the treatment for pulmonary hypertension?

Treatment of Pulmonary Hypertension

Initial combination therapy with ambrisentan and tadalafil is the recommended first-line treatment for most patients with pulmonary arterial hypertension (PAH) to improve exercise capacity and delay clinical worsening. 1

Classification and Initial Assessment

• Pulmonary hypertension (PH) is classified into five groups based on etiology, with treatment approaches differing significantly between groups 2, 3
• Right heart catheterization is essential to confirm diagnosis (defined as mean pulmonary artery pressure >20 mmHg), establish specific classification, determine severity, and guide therapy 3
• Vasoreactivity testing is mandatory for patients with idiopathic PAH to identify potential responders to calcium channel blockers 3
• Risk assessment using clinical parameters, exercise capacity, biomarkers, and imaging is crucial to guide therapy selection 3

Treatment Based on PAH Classification and Risk Stratification

For Vasoreactive Patients

• High-dose calcium channel blockers are the first-line therapy for patients who demonstrate vasoreactivity during testing 3

For Non-Vasoreactive Patients

• Low or intermediate risk (WHO FC II-III):

  • Initial combination therapy with ambrisentan and tadalafil is recommended as it has proven superior to initial monotherapy in delaying clinical failure 1
  • If combination therapy is not tolerated, monotherapy with either bosentan, macitentan, ambrisentan, riociguat, sildenafil, or tadalafil can be considered 1

• High risk (WHO FC IV):

  • Continuous intravenous epoprostenol should be prioritized as it has reduced the 3-month mortality rate in high-risk PAH patients 1, 4
  • Alternative options include intravenous treprostinil or subcutaneous treprostinil 1

Sequential Combination Therapy

• For patients with inadequate clinical response to initial therapy, sequential addition of medications from different drug classes is recommended 1
• Triple combination therapy should be attempted in cases of inadequate response to double combination therapy 1

Supportive Care and General Measures

• Diuretics are recommended for management of fluid retention and right heart failure 2, 3
• Oxygen supplementation should be used to maintain arterial oxygen saturations >90% 2, 3
• Supervised exercise training should be considered for physically deconditioned PAH patients under medical therapy 5, 3
• Pregnancy should be avoided in patients with PAH due to high mortality risk 5
• Immunization against influenza and pneumococcal pneumonia is recommended 1

Advanced Therapies

• Lung transplantation should be considered for patients with inadequate response to maximal medical therapy 1
• Balloon atrial septostomy may be considered as a palliative or bridging procedure in patients deteriorating despite maximal medical therapy 1
• Hospitalization in intensive care unit is recommended for PH patients with high heart rate (>110 beats/min), low blood pressure (systolic blood pressure <90 mmHg), low urine output and rising lactate levels 1

Treatment for Other PH Groups

• Group 2 (PH due to left heart disease): Treatment should focus on optimizing the underlying cardiac condition; PAH-specific therapies are not recommended 5, 2
• Group 3 (PH due to lung disease): Long-term oxygen therapy is recommended; PAH-specific therapies are generally not recommended 5, 6
• Group 4 (Chronic thromboembolic PH): Pulmonary endarterectomy is the treatment of choice when feasible 5, 3

Monitoring and Follow-up

• Regular follow-up assessments every 3-6 months in stable patients are recommended 3
• Treatment goal is to achieve and maintain low-risk status, with a focus on improving exercise capacity (6-minute walk distance >440m), reducing right ventricular dysfunction, and improving quality of life 5, 3

Important Considerations and Pitfalls

• Patients with PAH should be managed at specialized centers with expertise in pulmonary hypertension 3
• The combination of riociguat and PDE-5 inhibitors (such as sildenafil) is contraindicated due to risk of hypotension 1
• Avoid volume overload in patients with right ventricular failure as it may worsen cardiac function 2
• Sildenafil should never be taken with nitrate medications as this can cause dangerous drops in blood pressure 7