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Review
. 2021 Aug;11(4):1089-1101.
doi: 10.21037/cdt-20-374.

Prognostic biomarkers in pediatric pulmonary arterial hypertension

Mark-Jan Ploegstra  1 , Rolf M F Berger  1
Affiliations
Review

Prognostic biomarkers in pediatric pulmonary arterial hypertension

Mark-Jan Ploegstra et al. Cardiovasc Diagn Ther. 2021 Aug.

Abstract

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.

Keywords: Biomarkers; outcome; pediatric pulmonary arterial hypertension (pediatric PAH); prognosis.

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Conflict of interest statement

Conflicts of Interest: Both authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/cdt-20-374). The series “Pediatric Pulmonary Hypertension” was commissioned by the editorial office without any funding or sponsorship. RMFB reports other from Actelion, other from Lilly, other from GSK, other from Pfizer, outside the submitted work. MJP has no conflicts of interest to declare.

Figures

Figure 1
Figure 1
Survival of a cohort of 58 children from the Dutch National Network for Pediatric Pulmonary Hypertension with idiopathic or heritable pulmonary arterial hypertension stratified by the counted number of low risk criteria (103). *, vasodilator response according to Sitbon criteria. PAH, pulmonary arterial hypertension; BNP, brain natriuretic peptide; mPAP/mSAP, ratio of mean pulmonary arterial pressure to mean systemic arterial pressure.
See this image and copyright information in PMC

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