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Published on: May 11, 2015
Prognostic biomarkers in pediatric pulmonary arterial hypertension
Mark-Jan Ploegstra1, Rolf M F Berger1
1Center for Congenital Heart Diseases, Department of Pediatric Cardiology, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen, The Netherlands.
Insights
Pediatric pulmonary arterial hypertension (PAH) needs better prognostic biomarkers. A multi-marker approach using physiological, functional, and imaging data can guide treatment and improve outcomes for children with PAH.
Area of Science:
- Pediatric Cardiology
- Pulmonary Hypertension Research
- Biomarker Discovery
Background:
- Pediatric pulmonary arterial hypertension (PAH) is a severe, progressive vascular disease with a poor prognosis despite current treatments.
- Accurate prognostic biomarkers are crucial for managing pediatric PAH, guiding therapy escalation, and considering interventions like transplantation.
Purpose of the Study:
- To review potential prognostic biomarkers for clinical management of pediatric PAH.
- To highlight the need for a comprehensive approach to improve patient outcomes.
Main Methods:
- Review of existing literature on prognostic biomarkers in pediatric PAH.
- Categorization of biomarkers into physiological, functional, electrocardiographic, circulating serum, hemodynamic, and imaging types.
- Analysis of biomarkers including heart rate variability, 6-minute walk distance, cardiopulmonary exercise testing, natriuretic peptides, and cardiac MRI.
Main Results:
- Numerous potential prognostic biomarkers exist for pediatric PAH, reflecting diverse aspects of the disease.
- Biomarkers encompass physiological measures, functional capacity, cardiac electrical activity, serum markers, hemodynamics, and imaging findings.
- A multi-marker strategy is most effective for guiding therapeutic decisions.
Conclusions:
- A multi-marker approach integrating various biomarker types is essential for optimizing treatment strategies in pediatric PAH.
- Improved prognostic assessment through biomarkers can lead to better clinical management and improved outcomes for affected children.
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.
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