Biomarkers for pediatric pulmonary arterial hypertension: challenges and recommendations

Pediatric pulmonary arterial hypertension (PAH) is an uncommon disease that can occur in neonates, infants, and children, and is associated with high morbidity and mortality. Despite advances in treatment strategies over the last two decades, the underlying structural and functional changes to the pulmonary arterial circulation are progressive and lead eventually to right heart failure. The management of PAH in children is complex due not only to the developmental aspects but also because most evidence-based practices derive from adult PAH studies. As such, the pediatric clinician would be greatly aided by specific characteristics (biomarkers) objectively measured in children with PAH to determine appropriate clinical management. This review highlights the current state of biomarkers in pediatric PAH and looks forward to potential biomarkers, and makes several recommendations for their use and interpretation.