10:20 AM - 11:50 AM
[I-S10-01] Recent Advances in Pediatric Pulmonary Hypertension
Pediatric pulmonary hypertensive vascular disease composes a vasculopathy typified by disordered proliferation, apoptosis, vasoconstriction, inflammation and fibrosis. Pulmonary vascular obstruction increases the afterload of the right ventricle and the ability of the right ventricle to adapt often determines symptoms and outcome. Neonatal, infant and childhood pulmonary hypertensive vascular diseases display a heterogeneous phenotype that impacts on the epidemiology, classification, evaluation, diagnosis and management. The developing pulmonary vascular bed may be subjected to maldevelopment, maladaptation, growth arrest or dysregulation throughout intrauterine and postnatal life. Pulmonary hypertension targeted drug therapies are efficacious in children. However, drug therapy is influenced by developmental changes in renal, hepatic blood flow, drug clearance and toxicity profiles. The Pott’s shunt is emerging as a therapy to unload the right ventricle that may be particularly suited to treatment of children. Clinical endpoints such as exercise capacity, using traditional classifications and testing cannot be applied routinely to children. Many lack the necessary neurodevelopmental skills and equipment may not be appropriate for use in children. Selection of endpoints appropriate to encompass the developmental spectrum from neonate to adolescent is particularly challenging. The development of composite outcome scores that include age and a developmentally specific functional classification, growth and development scores, exercise data, biomarkers and hemodynamics with repeated evaluation throughout the period of growth and development. The search for robust evidence to guide safe therapy of children and neonates with pulmonary hypertensive vascular disease is a crucial and necessary goal.