Treatment Approaches for Group 2 and 3 Pulmonary Hypertension
For patients with pulmonary hypertension due to left heart disease (Group 2) or lung diseases (Group 3), the primary treatment should focus on optimizing the underlying condition rather than using pulmonary arterial hypertension-specific therapies. 1
Group 2 PH (Left Heart Disease)
Primary Treatment Approach
- Optimize treatment of the underlying left heart condition (structural heart disease, heart failure) 1
- Manage volume status carefully to avoid fluid overload 1
- Identify and treat concomitant disorders that may worsen PH, including COPD, sleep apnea syndrome, and pulmonary embolism 1
- Control risk factors for cardiovascular diseases and features of metabolic syndrome 1
Pharmacological Management
- For heart failure with reduced ejection fraction: optimize neurohormonal antagonists (ACE inhibitors, beta-blockers, spironolactone) 1
- For heart failure with preserved ejection fraction: no strong evidence-based recommendations exist 1
- PAH-specific therapies (prostanoids, endothelin receptor antagonists, PDE-5 inhibitors) are not recommended for Group 2 PH 1
Special Considerations
- In patients with severe pre-capillary component (high diastolic pressure gradient and/or high pulmonary vascular resistance), referral to a pulmonary hypertension expert center is recommended 1
- Right heart catheterization should be performed with patients in optimized volume status to accurately assess hemodynamics 1
Group 3 PH (Lung Diseases and/or Hypoxia)
Primary Treatment Approach
- Optimize treatment of the underlying lung disease (COPD, interstitial lung disease, combined pulmonary fibrosis and emphysema) 1
- Long-term oxygen therapy for patients with chronic hypoxemia 1
- Consider referral to a center with expertise in both PH and lung diseases for patients with severe PH disproportionate to their lung disease 1
Pharmacological Management
- PAH-specific therapies are not recommended for Group 3 PH 1
- Patients should be carefully assessed (including right heart catheterization, echocardiography, chest CT scan, 6-minute walk test, and pulmonary function testing) to confirm they do not have PAH before considering any advanced therapies 1
Diagnostic Considerations
Distinguish between Group 2/3 PH and Group 1 PAH through comprehensive evaluation 1:
- Echocardiography to assess probability of PH
- Pulmonary function tests including DLCO
- Arterial blood gas analysis
- High-resolution CT of the chest
- V/Q scan to exclude chronic thromboembolic PH
- Right heart catheterization to confirm diagnosis and classification
Hemodynamic criteria for post-capillary PH (Group 2) 2:
- Mean pulmonary artery pressure (mPAP) ≥25 mmHg
- Pulmonary artery wedge pressure (PAWP) >15 mmHg
Common Pitfalls to Avoid
- Do not use PAH-specific therapies in Group 2 or 3 PH - these medications have not shown consistent benefits and may cause harm 1
- Avoid misclassifying patients with Group 2 or 3 PH as having PAH (Group 1), as treatment strategies differ completely 3
- Be aware that sildenafil and other PAH-specific medications are only FDA-approved for Group 1 PAH, not for other forms of PH 4
- Do not rely solely on pulmonary pressure measurements; comprehensive assessment of hemodynamics, including transpulmonary gradient and pulmonary vascular resistance, is essential 2