PMID- 34527535 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210917 IS - 2223-3652 (Print) IS - 2223-3660 (Electronic) IS - 2223-3652 (Linking) VI - 11 IP - 4 DP - 2021 Aug TI - Prognostic biomarkers in pediatric pulmonary arterial hypertension. PG - 1089-1101 LID - 10.21037/cdt-20-374 [doi] AB - 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. CI - 2021 Cardiovascular Diagnosis and Therapy. All rights reserved. FAU - Ploegstra, Mark-Jan AU - Ploegstra MJ AD - Center for Congenital Heart Diseases, Department of Pediatric Cardiology, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen, The Netherlands. FAU - Berger, Rolf M F AU - Berger RMF AD - Center for Congenital Heart Diseases, Department of Pediatric Cardiology, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen, The Netherlands. LA - eng PT - Journal Article PT - Review PL - China TA - Cardiovasc Diagn Ther JT - Cardiovascular diagnosis and therapy JID - 101601613 PMC - PMC8410486 OTO - NOTNLM OT - Biomarkers OT - outcome OT - pediatric pulmonary arterial hypertension (pediatric PAH) OT - prognosis COIS- 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. EDAT- 2021/09/17 06:00 MHDA- 2021/09/17 06:01 PMCR- 2021/08/01 CRDT- 2021/09/16 07:16 PHST- 2020/03/18 00:00 [received] PHST- 2020/04/29 00:00 [accepted] PHST- 2021/09/16 07:16 [entrez] PHST- 2021/09/17 06:00 [pubmed] PHST- 2021/09/17 06:01 [medline] PHST- 2021/08/01 00:00 [pmc-release] AID - cdt-11-04-1089 [pii] AID - 10.21037/cdt-20-374 [doi] PST - ppublish SO - Cardiovasc Diagn Ther. 2021 Aug;11(4):1089-1101. doi: 10.21037/cdt-20-374.