Pulmonary hypertension in the child with bronchopulmonary dysplasia

Abstract

Bronchopulmonary dysplasia (BPD) is the most common chronic lung disease of prematurity resulting from complex interactions of perinatal factors that often lead to prolonged respiratory support and increased pulmonary morbidity. There is also growing appreciation for the dysmorphic pulmonary bed characterized by vascular growth arrest and remodeling, resulting in pulmonary vascular disease and its most severe form, pulmonary hypertension (PH) in children with BPD. In this review, we comprehensively discuss the pathophysiology of PH in children with BPD, evaluate the current recommendations for screening and diagnosis of PH, discern associated comorbid conditions, and outline the current treatment options.

Keywords: chronic lung disease; premature; pulmonary vascular disease; sildenafil; vasodilator.

Figure 1.

An example approach to care that includes pharmacotherapy for a child with BPD-PH (adapted from reference 78) without significant contribution from intracardiac or extracardiac shunt. We suggest a screening echocardiogram for all infants born less than 32 weeks when they reach 36 weeks PMA (or at the time of NICU discharge), regardless of BPD determination. Following the initial screen, if there are features suggestive of PH, infants should be evaluated at least monthly by echocardiogram, or sooner if clinically indicated. For example, those children with any degree of right ventricular dysfunction may deserve more frequent reassessments by echocardiogram as well as BNP/NTproBNP and vigilance to reduce concurrent insults or features of worsening. Features of worsening are varied, but include recurrent cardiopulmonary exacerbations with or without hypoxemia, inability to reduce respiratory support settings, failure of growth, escalating BNP and/or NTpro-BNP value over time, or other features of clinical decline and/or stagnation. Interval for reassessment by clinical re-evaluation, echocardiogram, labs, and other metrics require flexibility depending on clinical severity and degree of impairment, if any, of ventricular function. * For infants with severe BPD who require positive pressure support for oxygenation and/or ventilation, strategies to best support these children should be pursued, as recently described43. ** PH-specific Rx refers to chronic pulmonary vasodilator therapy, such as a PDE5i.