Diagnosis and management of pulmonary hypertension in infants with bronchopulmonary dysplasia: a guide for paediatric respiratory specialists

Pulmonary hypertension (PH) can develop in babies with bronchopulmonary dysplasia (BPD). PH is common in those with severe BPD and is associated with a high mortality rate. However, in babies surviving beyond 6 months, resolution of PH is likely. There is currently no standardised screening protocol for PH in BPD patients. Diagnosis in this group relies heavily on transthoracic echocardiography. Management of BPD-PH should be led by a multidisciplinary team and focus on optimal medical management of the BPD and associated conditions that may contribute to PH. PH-targeted pharmacotherapies have been used in BPD-PH. To date, these have not been investigated in clinical trials and evidence of their efficacy and safety is absent. EDUCATIONAL AIMS: To identify those BPD patients most at risk of developing PH. To be aware of detection, multidisciplinary management, pharmacological treatment and monitoring strategies for BPD-PH patients. To understand the potential clinical course for patients with BPD-PH and that evidence on efficacy and safety of PH-targeted pharmacotherapy in BPD-PH is limited.