Key result
A multi-marker approach utilizing physiological, functional, circulating, and imaging biomarkers appears most useful for guiding therapy decisions and improving outcomes in pediatric PAH.
Why the study?
Reliable prognostic biomarkers are needed in pediatric PAH to inform clinicians on disease progression and mortality risk to guide therapy escalation, surgical options, or transplantation listing.
What are the potential prognostic biomarkers for clinical management in pediatric pulmonary arterial hypertension?
What are the potential prognostic biomarkers for clinical management in pediatric pulmonary arterial hypertension?
A multi-marker approach utilizing physiological, functional, circulating, and imaging biomarkers is recommended to guide clinical management and therapy escalation in pediatric PAH.
Supports multi-marker strategies in pediatric PAH; hypothesis-generating and requires prospective validation before changing practice.
Pulmonary arterial hypertension (PAH) is a progressive life-threatening disease of the pulmonary vasculature. Despite the introduction of targeted therapies, prognosis remains poor. In pediatric PAH, reliable prognostic biomarkers are needed to inform clinicians on disease progression and risk of mortality, in order to be able to assess the need for escalation of medical therapy, consider surgical options such as Pott's shunt and listing for (heart)-lung transplantation. This review provides an overview of prognostic biomarkers that are considered to carry potential for the clinical management of pediatric PAH. These include conventional physiological biomarkers [resting heart rate, heart rate variability (HRV), a child's growth], biomarkers of functional status [World Health Organization functional class, 6-minute walk distance (6MWD), parameters derived from cardiopulmonary exercise testing (CPET), daily physical activity level], electrocardiographic biomarkers, circulating serum biomarkers (natriuretic peptides, uric acid, neurohormones, inflammatory markers, and novel circulating biomarkers), and multiple hemodynamic biomarkers and imaging biomarkers [echocardiography and cardiac magnetic resonance (CMR)]. In recent years, many potential prognostic biomarkers have become available for the management of PAH in children. As the available prognostic biomarkers reflect different aspects of the disease process and functional implications, a multi-marker approach appears the most useful for guiding therapy decisions and improve outcome in pediatric PAH.
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Ploegstra et al. (2021) conducted a review in pediatric pulmonary arterial hypertension. Prognostic biomarkers was evaluated. A multi-marker approach utilizing physiological, functional, circulating, and imaging biomarkers appears most useful for guiding therapy decisions and improving outcomes in pediatric PAH.
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