What is the management approach for a patient with group 3 pulmonary hypertension (PH) and class 3 chronic obstructive pulmonary disease (COPD) with hyperbicarbonatemia?

From the Guidelines

Management of a patient with Group 3 pulmonary hypertension due to Class 3 COPD with hyperbicarbonatemia requires a multifaceted approach focused on treating the underlying lung disease, with the primary goal of optimizing COPD management and addressing the hyperbicarbonatemia. The primary treatment involves optimizing COPD management with bronchodilators, including long-acting beta-agonists (LABAs) like salmeterol (50 mcg twice daily) or formoterol (12 mcg twice daily), and long-acting muscarinic antagonists (LAMAs) such as tiotropium (18 mcg once daily) 1. Inhaled corticosteroids (ICS) like fluticasone (250-500 mcg twice daily) should be added for patients with frequent exacerbations, as recommended by the American College of Chest Physicians and Canadian Thoracic Society guideline 1. Supplemental oxygen therapy is crucial if hypoxemia is present (PaO2 <55 mmHg or SaO2 <88%), typically starting at 1-3 L/min and titrated to maintain SaO2 >90%, according to the BTS guideline for oxygen use in adults in healthcare and emergency settings 1. The hyperbicarbonatemia, which indicates chronic respiratory acidosis with renal compensation, requires addressing the underlying hypoventilation through optimal bronchodilation and possibly non-invasive ventilation (NIV) such as BiPAP, particularly during sleep if nocturnal hypoventilation is present. Pulmonary vasodilators used in other forms of PH are generally not recommended for Group 3 PH as they may worsen ventilation-perfusion mismatch. Pulmonary rehabilitation, smoking cessation, vaccinations against influenza and pneumococcal disease, and management of comorbidities are essential components of care, as stated in the American Thoracic Society/European Respiratory Society statement on pulmonary rehabilitation 1. Regular monitoring of arterial blood gases, pulmonary function, and echocardiography to assess right ventricular function is necessary to guide ongoing management. Key considerations in managing these patients include:

• Optimizing COPD management with bronchodilators and ICS
• Addressing hyperbicarbonatemia through optimal bronchodilation and possible NIV
• Avoiding pulmonary vasodilators that may worsen ventilation-perfusion mismatch
• Implementing pulmonary rehabilitation and other supportive care measures
• Regular monitoring to guide ongoing management, as recommended by various guidelines, including those from the European Respiratory Society 1 and the American College of Chest Physicians and Canadian Thoracic Society 1.

From the Research

Management Approach for Group 3 Pulmonary Hypertension and Class 3 COPD

• The management of patients with group 3 pulmonary hypertension (PH) and class 3 chronic obstructive pulmonary disease (COPD) involves a comprehensive approach, considering the underlying conditions and symptoms 2.
• For patients with COPD-PH, phosphodiesterase type 5 inhibitors such as Sildenafil have been shown to improve six-minute walking distance (6MWD) and pulmonary artery systolic pressure (PASP) 3.
• However, the use of PAH-targeted therapy in group 3 PH has yielded conflicting or negative results in most trials, with the exception of inhaled treprostinil, which has been associated with improved outcomes in patients with PH-ILD 4.
• The management of PH resulting from left-sided heart disease primarily involves treatment of the underlying condition, while patients with PAH or CTEPH require referral to a specialist center and targeted treatments 5.
• Sildenafil has been approved for the treatment of pulmonary arterial hypertension (PAH) and has been shown to be effective in improving quality of life and functional status 6.

Considerations for Hyperbicarbonatemia

• Hyperbicarbonatemia, indicated by a venous bicarbonate level > 40, may require additional consideration in the management of patients with group 3 PH and class 3 COPD.
• However, there is limited evidence available on the specific management of hyperbicarbonatemia in this patient population.
• Further evaluation and management of the underlying conditions, including COPD and PH, should be prioritized, with consideration of the patient's overall clinical status and symptoms 2, 4, 5, 3, 6.