What is the primary treatment approach for babies diagnosed with Bronchopulmonary Dysplasia (BPD)?

Primary Treatment Approach for Babies with Bronchopulmonary Dysplasia (BPD)

The primary treatment approach for babies diagnosed with Bronchopulmonary Dysplasia (BPD) focuses on optimizing respiratory support with supplemental oxygen therapy to maintain oxygen saturations between 92-95%, along with comprehensive evaluation and management of underlying lung disease before initiating pulmonary hypertension-targeted therapies. 1, 2

Respiratory Support and Oxygen Therapy

• Supplemental oxygen therapy is the cornerstone of BPD management, with the goal of maintaining oxygen saturations between 92-95% to prevent adverse effects of hypoxia without increasing the risk of additional lung inflammation and injury 1, 2
• Evaluation for changes in respiratory support is essential, including potential escalation to mechanical ventilation for severe cases where the infant fails to maintain near-normal ventilation or requires high FiO2 levels despite conservative treatment 1, 2
• When mechanical ventilation is required, lung-protective strategies should be implemented, including minimizing peak inspiratory pressure and avoiding large tidal volumes to reduce ventilator-associated lung injury 1, 2
• High-frequency oscillatory ventilation is a reasonable alternative ventilation mode when poor lung compliance, low volumes, and poor gas exchange complicate the clinical course 1

Comprehensive Evaluation of Underlying Lung Disease

• A thorough evaluation of the underlying lung disease is recommended before initiating pulmonary hypertension-targeted therapies 1, 2
• Assessment should include evaluation for:
  • Chronic reflux and aspiration using upper gastrointestinal series, pH or impedance probe, and swallow studies 1
  • Structural airway abnormalities (such as tonsillar and adenoidal hypertrophy, vocal cord paralysis, subglottic stenosis, tracheomalacia) using flexible bronchoscopy 1, 2
  • Bronchoreactivity and need for bronchodilator therapy 1, 2
  • Sleep study to determine the presence of noteworthy episodes of hypoxia and whether hypoxemia has predominantly obstructive, central, or mixed causes 1

Management of Pulmonary Hypertension (PH) in BPD

• Screening for pulmonary hypertension by echocardiogram is recommended in all infants with established BPD 1
• For infants with BPD and PH, the following approach is recommended:
  • Inhaled nitric oxide (iNO) can be effective for infants with established BPD and symptomatic PH, typically initiated at doses of 10-20 ppm and weaned to 2-10 ppm for maintenance 1, 2
  • Sildenafil (0.5-2 mg/kg three times daily) is useful for infants with BPD and PH who are on optimal treatment for underlying respiratory and cardiac disease 1, 2
  • Serial echocardiograms should be performed to monitor response to pulmonary hypertension-targeted therapy, initially every 2-4 weeks with therapy initiation and then at 4-6 month intervals with stable disease 1

Additional Therapeutic Considerations

• Caffeine therapy has shown strong evidence for effectiveness in preventing or lessening the severity of BPD 3, 4
• Diuretics may be used in the initial management, with natural weaning by the relative decrease in dose with increasing weight gain 5
• Bronchodilators should be used selectively in infants with asthma-like symptoms or demonstrable reversibility in lung function, not routinely 2, 5
• Inhaled or systemic corticosteroids are not recommended for routine use in established BPD due to potential adverse effects on neurodevelopment 5, 6
• Vitamin A supplementation has some evidence for effectiveness in prevention of BPD 3, 6

Monitoring and Follow-up

• Long-term monitoring with lung function testing is recommended for all children with BPD 2, 5
• Lung imaging using ionizing radiation should be reserved for subgroups with severe BPD or recurrent hospitalizations 5
• A multidisciplinary approach for children with established severe BPD is preferable, with continued monitoring from the neonatal period into adulthood 5

Common Pitfalls and Caveats

• Brief assessments of oxygenation ("spot checks") are insufficient for determining the appropriate level of supplemental oxygen needed 1
• Despite the growing use of pulmonary vasodilator therapy for PH in BPD, data demonstrating efficacy are limited, and these agents should only be used after thorough diagnostic evaluations and aggressive management of the underlying lung disease 1
• Calcium channel blockers (CCBs) have shown poor acute response in infants with BPD compared to inhaled nitric oxide, and some infants may develop systemic hypotension 1
• Intravenous prostacyclin analogs (epoprostenol, treprostinil) should be used cautiously due to potential worsening of gas exchange by increasing ventilation-perfusion mismatch and risk of systemic hypotension 1