What is the management of pulmonary hypertension?

Management of Pulmonary Hypertension

The management of pulmonary hypertension requires a multidisciplinary approach at specialized centers with expertise in pulmonary hypertension, with treatment tailored to the specific classification and risk assessment of the patient to improve morbidity, mortality, and quality of life. 1

Diagnostic Evaluation

• Right heart catheterization is essential to confirm the diagnosis of pulmonary hypertension (defined as mean pulmonary artery pressure ≥25 mmHg at rest), determine the specific classification, assess severity, and guide therapy 1
• Vasoreactivity testing using short-acting agents is mandatory for patients with idiopathic PAH to identify potential responders to calcium channel blockers 1
• Ventilation-perfusion scanning should be performed to rule out chronic thromboembolic pulmonary hypertension (CTEPH) 1
• Classification according to the clinical guidelines (Nice 2013) is crucial to distinguish Group 1 PAH from other forms of pulmonary hypertension, as each requires a unique management approach 2

Treatment Based on PAH Classification and Functional Status

For Vasoreactive Patients

• High-dose calcium channel blockers are the first-line therapy for patients demonstrating a favorable acute response to vasodilator testing 1
• Regular follow-up is essential to confirm sustained response to calcium channel blockers 2

For Non-vasoreactive PAH Patients

WHO Functional Class II

• Oral therapies including phosphodiesterase-5 inhibitors (sildenafil) are recommended as initial therapy 2, 3
• Sildenafil is typically administered three times daily at 4-6 hour intervals 3
• Endothelin receptor antagonists may also be considered as initial therapy 2

WHO Functional Class III

• Consider either oral therapies or prostacyclin analogues depending on risk assessment 2
• Combination therapy may be appropriate for patients not responding adequately to monotherapy 2

WHO Functional Class IV

• Intravenous prostacyclin analogues (epoprostenol) are strongly recommended as they have demonstrated survival benefits 1, 4
• Epoprostenol should be initiated at 2 ng/kg/min and increased in increments of 2 ng/kg/min every 15 minutes or longer until dose-limiting effects occur 4
• Administration requires continuous intravenous infusion via a central venous catheter using an ambulatory infusion pump 4

Supportive Care and General Measures

• Diuretics are recommended for managing fluid overload with careful monitoring of electrolytes and renal function 1
• Oxygen supplementation should be used to maintain arterial oxygen saturations >90% 1
• Anticoagulation with coumarin derivatives may be beneficial, particularly in idiopathic PAH 5
• Careful monitoring for side effects of medications is essential, including hypotension with vasodilators 3

Monitoring and Follow-up

• Regular follow-up assessments every 3-6 months in stable patients are recommended 1
• Treatment goals include achieving and maintaining low-risk status, including a 6-minute walk distance >440m 1
• Adjustments to therapy should be made based on clinical response and hemodynamic parameters 2

Special Considerations

• Patients with severe disease who fail to respond to maximal medical therapy should be referred for lung transplantation evaluation 1
• Management of pulmonary hypertension associated with left heart disease, lung disease, or thromboembolic disease should primarily target the underlying cause 6
• Newer therapeutic approaches include sotatercept (approved in the USA in 2024), which suppresses activin receptor type IIA signaling and has shown substantial efficacy in clinical trials 7

Multidisciplinary Approach

• A shared-care approach involving pulmonologists, cardiologists, rheumatologists, and other specialists is recommended for optimal management 8
• Treatment at specialized centers with expertise in pulmonary hypertension improves outcomes 1

Common Pitfalls to Avoid

• Failure to confirm diagnosis with right heart catheterization before initiating specific PAH therapy 1
• Inappropriate use of calcium channel blockers in non-vasoreactive patients 2
• Abrupt withdrawal of PAH-specific therapy, which can lead to clinical deterioration 4
• Delayed referral for lung transplantation evaluation in appropriate candidates 1