An Update on the Diagnosis and Management of Bronchopulmonary Dysplasia (BPD)-associated Pulmonary Hypertension

The past decade of neonatal care has been highlighted by increased survival rates in smaller and more premature infants. Despite reduction in mortality associated with extreme prematurity, long term pulmonary morbidities remain a concern, with growing recognition of the clinical burden attributable to infants with bronchopulmonary dysplasia (BPD)-associated pulmonary hypertension (PH). Recent publications shed light on the critical contributions of maternal placental pathology and compromised intrauterine growth to fetal pulmonary vascular development. A body of literature has further clarified postnatal risk factors for PH, most notably the severity of BPD but surprisingly the additional presence of non-pulmonary morbidities including necrotizing enterocolitis (NEC). Limitations of current diagnostics persist with growing consideration of novel echocardiographic approaches as well as complementary non-invasive biomarkers to better identify infants at risk. In 2015, a joint report published by the American Heart Association and American Thoracic Society provided the first guidelines for the care of children with PH with limited content to address BPD-associated PH. These guidelines were expanded upon in an expert consensus report produced by the Pediatric Pulmonary Hypertension Network (PPHNet). These recommendations encouraged the use of standardized screening protocols and emphasized the importance of evaluation and treatment of comorbidities when PH is identified. Cardiac catheterization was recommended prior to initiation of therapy for more accurate quantification of pulmonary pressures, clarification of anatomy and guidance in the use of pharmacotherapy. Despite these guidelines, significant practice variation persists and gaps remain with respect to optimal evaluation and management of BPD-associated PH.