Biomarkers for pediatric pulmonary arterial hypertension - a call to collaborate

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.

Keywords: biomarkers; echocardiography; imaging; magnetic resonance; pediatric; pulmonary arterial hypertension; right ventricle.

Figure 1

Pulmonary arterial hypertension (PAH) is a syndrome that is idiopathic in origin or arises in association with a large number of disease processes. Biomarkers for PAH should correlate in some fashion to a hemodynamic measurement such as PA pressure, PA wedge pressure, pulmonary vascular resistance, etc., and/or to a clinical measurement such as survival, pulmonary function test, etc. Since the pathobiology of PAH is multifactorial and complex, it should not be surprising that potential biomarkers will likely reflect aspects of the underlying disease process.