Bronchopulmonary dysplasia and chronic neonatal lung disease

Clinical Case

History

This former 25-week gestation infant was born at 580 g to a 32-year-old primigravida following preterm labour with histological chorioamnionitis. She was intubated in the delivery room and received two doses of surfactant for RDS. Her NICU course included a significant PDA treated with ibuprofen, late-onset sepsis at day 14, and poor growth despite fortification. She was extubated to CPAP at day 21 and transitioned to HFNC at day 35 [1].

Examination at 42 weeks PMA

The infant is on 35 percent oxygen via HFNC at 2 L/min with subcostal and intercostal retractions. Auscultation reveals bilateral crackles and a loud single second heart sound. Liver is palpable 3 cm below the costal margin. Weight is at the 3rd centile for corrected age with mildly decreased tone [2].

Key Questions

1. What is this infant's BPD classification and what complication is suspected? She has severe BPD by the NIH consensus definition, requiring 35 percent oxygen at 36 weeks PMA [2]. The loud P2 and hepatomegaly strongly suggest pulmonary hypertension, complicating 8 to 25 percent of moderate-to-severe BPD cases with mortality up to 40 percent. Urgent echocardiography is required [1].

2. How should the pulmonary hypertension be investigated and managed? Echocardiography estimates pulmonary artery pressure and assesses right ventricular function. Contributing factors including hypoxia, acidosis, and anaemia must be corrected. Management includes inhaled nitric oxide at 10 to 20 ppm, oral sildenafil at 0.5 to 2 mg/kg per dose three times daily, and specialist cardiology input [3].

3. What are the discharge considerations? Discharge requires four weeks of stable respiratory support, adequate weight gain, no apnoea for five to seven days off caffeine, and a documented home oxygen plan. Palivizumab for RSV season and multidisciplinary follow-up should be arranged [3].

4. What is the long-term prognosis? Severe BPD with pulmonary hypertension carries the worst prognosis with mortality up to 40 percent. Survivors face increased readmission risk, persistent symptoms, and neurodevelopmental impairment. Even mild BPD is associated with lifelong reduced lung function [1].