From the Guidelines
Medical therapy for Group III pulmonary hypertension should primarily focus on treating the underlying lung disease, with sildenafil being a potential option in select cases, as evidenced by its successful use in infants with bronchopulmonary dysplasia and congenital diaphragmatic hernia 1. The mainstay of treatment includes optimizing oxygen therapy to maintain oxygen saturation above 90%, as hypoxemia is a major driver of pulmonary vasoconstriction.
- Long-term oxygen therapy should be prescribed for patients with chronic hypoxemia.
- Bronchodilators such as long-acting beta-agonists and long-acting muscarinic antagonists are essential for patients with obstructive lung diseases.
- Inhaled corticosteroids may be added for those with frequent exacerbations.
- Diuretics such as furosemide can help manage right heart failure symptoms. Unlike Group I pulmonary hypertension, pulmonary vasodilators are generally not recommended for Group III pulmonary hypertension as they may worsen ventilation-perfusion mismatch and hypoxemia, as supported by the American Thoracic Society policy statement 1. However, in carefully selected patients with severe pulmonary hypertension disproportionate to their lung disease, a cautious trial of these medications might be considered under specialist supervision. The rationale behind this approach is that Group III pulmonary hypertension results from hypoxic vasoconstriction, destruction of the pulmonary vascular bed, and inflammation due to underlying lung disease, rather than primary vascular pathology. Lung transplantation is also an accepted treatment option for children with Group 3 PH who have progressive disease despite optimal therapy, but their long-term outcomes after transplantation are less favorable compared with those with Group 1 PH 1.
From the Research
Medical Therapy for Group III Pulmonary Hypertension
- Group III pulmonary hypertension is classified as pulmonary hypertension due to chronic respiratory disease and/or hypoxia, with common conditions including chronic obstructive lung disease (COPD), diffuse parenchymal lung disease, and sleep-disordered breathing 2.
- Pulmonary rehabilitation (PR) is a core component in the management of patients with COPD, and may include cardiopulmonary exercise testing, neuromuscular electrical stimulation, gait assessment and training, and add-on therapies such as oxygen, noninvasive ventilator support, or endoscopic lung volume reduction 3.
- The combination of tiotropium and pulmonary rehabilitation has been shown to improve exercise tolerance in patients with COPD, with improvements in endurance time and dyspnea 4, 5.
- Tiotropium, a once-daily, inhaled anticholinergic bronchodilator, provides sustained improvements in airflow and lung hyperinflation reduction, and has been shown to enhance the effects of exercise training in patients with COPD 4, 5.
- Targeted treatments for pulmonary hypertension, such as phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin analogues, and prostacyclin receptor agonists, are licensed for patients with pulmonary arterial hypertension (PAH), but not specifically for Group III pulmonary hypertension 6.
- Management of Group III pulmonary hypertension primarily involves treatment of the underlying respiratory condition, with pulmonary rehabilitation and pharmacological therapies such as tiotropium playing a key role in improving exercise tolerance and reducing symptoms 2, 3, 4, 5.