Progress in the diagnosis and management of pulmonary hypertension in children

Abstract

Purpose of Review Pulmonary hypertension (PH) is a complex disease that extends beyond merely elevated pulmonary blood pressures and right ventricular dysfunction. Its multiple etiologies and ever-expanding diagnostic tools and therapeutic approaches make it a heterogeneous disease with widely variable clinical sequelae. There are still many unanswered questions that challenge our understanding of this disease. Recent Findings The study of PH in the pediatric patient is as robust as ever, with the creation and inclusion of pediatric-specific disease characteristics in the most recent World Health Organization classification system, improved understanding of the pathophysiology of PH in pediatric diseases like bronchopulmonary dysplasia, and increasingly expanding diagnostic tools and management possibilities. While the use of PH therapies in children previously often relied on expert opinion and inferences from studies involving adults, pediatric-targeted research is becoming more widely supported and pursued, and even has come under recent debate, which at the very least stimulates further collaboration and discussion. Summary This review will highlight the changes in the PH classification system, briefly explore PH in bronchopulmonary dysplasia, and provide updates on the diagnostic and management tools used by experts in the field.

A Computable Phenotype Improves Cohort Ascertainment in a Pediatric Pulmonary Hypertension Registry

“…Review articles of PH 17, 18 and notes of sample patients with and without PH were used to create a dictionary of clinical concepts relevant to PH. We processed these sources using the Narrative Information Linear Extraction (NILE) package, 19, 20 which includes a named entity recognition package that extracts terms and maps them to clinical concepts using the Unified Medical Language System (UMLS).…”

Trends in Hospitalization for Pediatric Pulmonary Hypertension

“…However, our study period does coincide with an era of significant improvement in neonatology care and outcomes, as well as in the detection and medical management of chronic PH, particularly that in children without CHD. 22,23 Improved survival of infants with bronchopulmonary dysplasia and the emergence of pulmonary vasodilator therapy may explain both the decrease in observed mortality per hospitalization as well as the relative increase in the non-CHD group of children admitted with PH. These trends also may relate to increasing success in the early surgical treatment of CHD with a focus on prevention of PH.…”

“…Procedure codes also were used to identify the performance of procedures that were viewed by the multidisciplinary author group as clinically relevant to either the diagnosis or sequelae of pediatric PH and that reliably could be identified by procedure codes: cardiac catheterization (37. 23 2 test for categorical variables, as appropriate. Odds ratios were calculated for intergroup discrete outcome comparisons; for PH versus non-PH comparisons, the non-PH cohort is the reference group.…”

Biomarker and shear stress in secondary pediatric pulmonary hypertension

“…The pathogenesis of PH is primarily based on endothelial dysfunction within pulmonary vasculature, which eventually leads to medial thickening, intimal fibrosis, inflammation, and microthrombosis. Tissue damage, inflammation, and microthrombi formation are also involved in the pathogenesis of PH (1)(2)(3)(4).…”