Biomarkers for Pediatric Pulmonary Arterial Hypertension – A Call to Collaborate

Kelley L Colvin,D Dunbar Ivy,Michael E Yeager,Kurt R Stenmark,Ryan P Delaney,Melanie J Dufva

doi:10.3389/fped.2014.00007

Abstract

Therapeutic approaches in pediatric pulmonary arterial hypertension (PAH) are based primarily on clinician experience, in contrast to the evidence-based approach in adults with pulmonary hypertension. There is a clear and present need for non-invasive and objective biomarkers to guide the accurate diagnosis, treatment, and prognosis of this disease in children. The multifaceted spectrum of disease, clinical presentation, and association with other diseases makes this a formidable challenge. However, as more progress is being made in the understanding and management of adult PAH, the potential to apply this knowledge to children has never been greater. This review explores the state of the art with regard to non-invasive biomarkers in PAH, with an eye toward those adult PAH biomarkers potentially suitable for application in pediatric PAH.

Highlights

Our understanding of pulmonary hypertension (PH) in children has been hampered over the years by a number of factors
This review explores the state of the art with regard to non-invasive biomarkers in pulmonary arterial hypertension (PAH), with an eye toward those adult PAH biomarkers potentially suitable for application in pediatric PAH
This review focuses on the unrealized promise of biomarkers in pediatric PH, their potential to improve our ability to Abbreviations: CEC, circulating endothelial cell; CHD, congenital heart disease; CT, computed tomography; EC, endothelial cell; Echo, echocardiography; EPC, endothelial progenitor cell; MRI, magnetic resonance imaging; PAH, pulmonary arterial hypertension; Positron emission tomography (PET), positron emission tomography; PH, pulmonary hypertension; PVR, pulmonary vascular resistance; RHC, right heart catheterization; RV, right ventricle; Single photon emission computed tomography (SPECT), single photon emission computed tomography; TDI, tissue Doppler imaging

Summary

Introduction

Our understanding of pulmonary hypertension (PH) in children has been hampered over the years by a number of factors. Another study conducted by the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) reported survival rates of IPAH/FPAH and APAH-CHD children, treated with etiologic specific therapies, at 96, 84, and 74% for 1, 3, and 5 years, respectively [16].

Results

Conclusion