Related Experiment Video
Updated: Jun 18, 2025
Shunt Surgery, Right Heart Catheterization, and Vascular Morphometry in a Rat Model for Flow-induced Pulmonary Arterial Hypertension
Published on: February 11, 2017
Circulating High Mobility Group Box-1 Does Not Predict Pulmonary Arterial Hypertension in Children with Congenital
Bruno Caracci1, Carolyne Pehora1, Lee Benson2
1Department of Anesthesiology and Pain Medicine, The Hospital for Sick Children and The University of Toronto, Ontario, Canada.
Insights
High mobility group box-1 (HMGB1) is not a reliable biomarker for pulmonary arterial hypertension (PAH) in children with congenital heart disease (CHD). Further research is needed to identify effective biomarkers for this vulnerable pediatric population.
Area of Science:
- Pediatric Cardiology
- Biomarker Discovery
- Pulmonary Hypertension
Background:
- Pulmonary arterial hypertension (PAH) is a severe complication of pediatric congenital heart disease (CHD).
- High mobility group box-1 (HMGB1) protein has shown promise as a diagnostic biomarker for PAH in adults with CHD.
- HMGB1 levels in adults correlated with disease severity and improved with treatment.
Purpose of the Study:
- To investigate whether HMGB1 serves as a diagnostic biomarker for pediatric CHD-associated PAH.
- To compare HMGB1 levels in children with and without CHD-PAH.
Main Methods:
- Prospective cohort study conducted at a quaternary pediatric academic hospital.
- Inclusion of children ≤18 years with and without known pulmonary hypertension secondary to CHD.
- Measurements included pulmonary hemodynamics, echocardiography, and biomarker analysis (HMGB1, NT-proBNP).
Main Results:
- No significant difference in HMGB1 or NT-proBNP levels was observed between children with and without CHD-PAH.
- Neither HMGB1 nor NT-proBNP levels correlated with pulmonary hypertension severity in the pediatric cohort.
- Mean pulmonary vascular resistance index in patients with CHD-PAH was 10 Wood units/m².
Conclusions:
- Contrary to findings in adults, HMGB1 is not a suitable biomarker for PAH in pediatric CHD.
- The search for reliable biomarkers in this high-risk pediatric population must continue.
- Further research is warranted to identify effective diagnostic and prognostic tools for pediatric CHD-PAH.
Objectives:
Pulmonary arterial hypertension (PAH) is a devastating complication of pediatric congenital heart disease (CHD). A recent study has identified the protein high mobility group box-1 (HMGB1) as a diagnostic tool in adults with CHD-associated PAH. HMGB1 levels in adults with CHD-associated PAH correlated with mean pulmonary artery pressure and pulmonary vascular resistance, and HGMB1 levels fell in response to sildenafil therapy. We wanted to assess if HGMB1 was a biomarker of pediatric CHD-PAH.
Design:
Prospective cohort study.
Setting:
Quaternary pediatric academic hospital PARTICIPANTS: Children ≤18 years with CHD with and without known pulmonary hypertension. Controls were children undergoing dental or urologic surgery with no known heart disease.
Interventions:
Pulmonary hemodynamics, echocardiographic assessment, and biomarker measurement. Controls had biomarker measurement only.
Measurements And Main Results:
Patients with CHD-PAH had mean pulmonary vascular resistance index of 10 Wood units/m2. Neither HGMB1 nor N-terminal pro-brain-type natriuretic peptide levels were significantly different between the groups. Neither marker correlated with pulmonary hypertension.
Conclusions:
Unlike in adults, HGMB1 is not a biomarker of PAH in pediatric CHD. Further work will continue to explore for biomarkers for this high-risk population.
More Related Videos
Related Concept Videos
01:30Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
01:26Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...