Characterization of Circulating and Urinary Biomarkers in the Fontan Circulation and Their Correlation With Cardiac Imaging

Ghelani, Sunil J.; Opotowsky, Alexander R.; Harrild, David M.; Powell, A.; Azcue, Nina; Ahmad, Sidra; Clair, Nicole St.; Bradwin, Gary; Rathod, Rahul H.

doi:10.1016/j.amjcard.2021.08.063

Cited by 6 publications

(6 citation statements)

References 28 publications

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“…They suggested NT-proBNP >150 pg/ml may be used as an indicator of ventricular dysfunction. 35 Similarly, a study of 133 Fontan patients with a median age of 13.2 years found that the 27% of patients who had an adverse cardiac outcome had significantly higher NT-proBNP levels at recruitment and their NT-proBNP level correlated with severity of event. 36 This highlights the potential role of NT-proBNP as a prognostic indicator in Fontan patients given that it is elevated prior to clinical symptoms or signs of ventricular dysfunction and correlates to cardiac MRI findings.…”

Section: Brain Naturetic Peptide and Nt-probnpmentioning

confidence: 98%

Section: Brain Naturetic Peptide and Nt-probnpmentioning

confidence: 98%

“…36 Ghelani et al found suppression of tumorigenicity-2 negatively correlated to ejection fraction in clinically stable Fontan patients, but it was not statistically significant and performed worse than NT-proBNP and troponin. 35 Soluble suppression of tumorigenicity-2 may have a role in diagnosis and monitoring of paediatric heart failure, but given the difference in pathophysiology compared to adults, this may be limited and warrants further studies before routine use.…”

Section: Mr-proadmmentioning

confidence: 99%

Section: Sst2mentioning

confidence: 99%

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Paediatric heart failure – understanding the pathophysiology and the current role of cardiac biomarkers in clinical practice

et al. 2023

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Introduction: Paediatric heart failure is a common clinical syndrome that may be experienced by children with congenital heart disease (CHD) and/or cardiomyopathy. It is characterised by clinical signs/symptoms which reflect the underlying pathophysiology based on one of three main clinical states: Pulmonary over-circulation, pressure overload, and ventricular failure. Current diagnosis relies on clinical assessment and echocardiogram imaging as cardiac biomarkers has been predominantly scientific to date. This review provides a comprehensive overview of paediatric heart failure pathophysiology and considers the available evidence for cardiac biomarkers in this setting. Methods: A literature review was completed using MEDLINE ALL, EMBASE, and PubMed on 10th November, 2022. Search terms included biomarkers, heart failure, heart defects, congenital heart disease, fontan circulation, single ventricle circulation, cardiomyopathy, and child. This allowed the identification of individual cardiac biomarkers which are the focus of this review. These included NT-proBNP, MR-proANP, MR-proADM, troponin, sST2, galectin 3, and growth differentiation factor-15. Results: Paediatric studies have established reference ranges for NT-proBNP and troponin for children with structurally normal hearts. Of all the biomarkers reviewed, NT-proBNP appears to correlate most closely with symptoms of heart failure and ventricular dysfunction on echocardiogram. However, there remains limited longitudinal data for NT-proBNP, and no validated reference ranges for patients with CHD and/or cardiomyopathy. None of the other biomarkers reviewed were consistently superior to NT-proBNP. Conclusion: Further large paediatric studies of patients with heart failure are needed to validate NT-proBNP in CHD and to evaluate the role of novel biomarkers in specific types of CHD, e.g. single ventricle physiology.

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Section: Brain Naturetic Peptide and Nt-probnpmentioning

confidence: 98%

Section: Brain Naturetic Peptide and Nt-probnpmentioning

confidence: 98%

Section: Mr-proadmmentioning

confidence: 99%

Section: Sst2mentioning

confidence: 99%

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Paediatric heart failure – understanding the pathophysiology and the current role of cardiac biomarkers in clinical practice

et al. 2023

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“…One means to stimulate ketone metabolism is the use of sodium-glucose co-transporter-2 (SGLT2) inhibitors as studied in BV HF [ 166 , 167 , 168 , 169 , 170 , 171 ], an approach supported by a case report (Fontan circulation, 5 patients), where rehospitalization rate was reduced without acute adverse effects [ 172 ]. Although based on a very limited patient number, objective data such as increases in systemic oxygen saturation, serum albumin level, and estimated glomerular filtration rate, as well as a decrease in plasma NT-proBNP-level are promising, as NT-proBNP > 100 pg/mL has a 91% sensitivity for significant ventricular dilation, and one of 82% for ejection fraction <50% [ 173 ], and is predictive of adverse outcome [ 174 ].…”

Section: Cardiac Energy Metabolismmentioning

confidence: 99%

Comparative Metabolomics in Single Ventricle Patients after Fontan Palliation: A Strong Case for a Targeted Metabolic Therapy

Renaud,

Scholl-Bürgi,

Karall

et al. 2023

Metabolites

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Most studies on single ventricle (SV) circulation take a physiological or anatomical approach. Although there is a tight coupling between cardiac contractility and metabolism, the metabolic perspective on this patient population is very recent. Early findings point to major metabolic disturbances, with both impaired glucose and fatty acid oxidation in the cardiomyocytes. Additionally, Fontan patients have systemic metabolic derangements such as abnormal glucose metabolism and hypocholesterolemia. Our literature review compares the metabolism of patients with a SV circulation after Fontan palliation with that of patients with a healthy biventricular (BV) heart, or different subtypes of a failing BV heart, by Pubmed review of the literature on cardiac metabolism, Fontan failure, heart failure (HF), ketosis, metabolism published in English from 1939 to 2023. Early evidence demonstrates that SV circulation is not only a hemodynamic burden requiring staged palliation, but also a metabolic issue with alterations similar to what is known for HF in a BV circulation. Alterations of fatty acid and glucose oxidation were found, resulting in metabolic instability and impaired energy production. As reported for patients with BV HF, stimulating ketone oxidation may be an effective treatment strategy for HF in these patients. Few but promising clinical trials have been conducted thus far to evaluate therapeutic ketosis with HF using a variety of instruments, including ketogenic diet, ketone esters, and sodium-glucose co-transporter-2 (SGLT2) inhibitors. An initial trial on a small cohort demonstrated favorable outcomes for Fontan patients treated with SGLT2 inhibitors. Therapeutic ketosis is worth considering in the treatment of Fontan patients, as ketones positively affect not only the myocardial energy metabolism, but also the global Fontan physiopathology. Induced ketosis seems promising as a concerted therapeutic strategy.

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Albuminuria Prevalence in Fontan Patients: A Systematic Review and Meta-Analysis

Van Belle,

Van den Eynde,

Cieplucha

et al. 2024

Pediatr Cardiol

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Characterization of Circulating and Urinary Biomarkers in the Fontan Circulation and Their Correlation With Cardiac Imaging

Cited by 6 publications

References 28 publications

Paediatric heart failure – understanding the pathophysiology and the current role of cardiac biomarkers in clinical practice

Paediatric heart failure – understanding the pathophysiology and the current role of cardiac biomarkers in clinical practice

Comparative Metabolomics in Single Ventricle Patients after Fontan Palliation: A Strong Case for a Targeted Metabolic Therapy

Albuminuria Prevalence in Fontan Patients: A Systematic Review and Meta-Analysis

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