Background: Pediatric cancer survivors are at increased risk of cardiac dysfunction and heart failure. Reduced peak oxygen consumption (peak VO) is associated with impaired cardiac reserve (defined as the increase in cardiac function from rest to peak exercise) and heart failure risk, but it is unclear whether this relationship exists in pediatric cancer survivors. This study sought to investigate the presence of reduced peak VO in pediatric cancer survivors with increased risk of heart failure, and to assess its relationship with resting cardiac function and cardiac haemodynamics and systolic function during exercise.
Methods: Twenty pediatric cancer survivors (8-24 years; 10 male) treated with anthracycline chemotherapy ± radiation underwent cardiopulmonary exercise testing to quantify peak VO, with a value < 85% of predicted defined as impaired peak VO. Resting cardiac function was assessed using 2- and 3-dimensional echocardiography, with cardiac reserve quantified from resting and peak exercise heart rate, stroke volume index (SVI) and cardiac index (CI) using exercise cardiovascular magnetic resonance (CMR).
Results: Twelve of 20 survivors (60%) had reduced peak VO (70 ± 16% vs. 97 ± 14% of age and gender predicted). There were no differences in echocardiographic or CMR measurements of resting cardiac function between survivors with normal or impaired peak VO. However, those with reduced peak VO had diminished cardiac reserve, with a lesser increase in CI and SVI during exercise (Interaction P < 0.01 for both), whilst the heart rate response was similar (P = 0.71).
Conclusions: Whilst exercise intolerance is common among pediatric cancer survivors, it is poorly explained by resting measures of cardiac function. In contrast, impaired exercise capacity is associated with impaired haemodynamics and systolic functional reserve measured during exercise. Consequently, measures of cardiopulmonary fitness and cardiac reserve may aid in early identification of survivors with heightened risk of long-term heart failure.
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http://dx.doi.org/10.1186/s12968-020-00658-4 | DOI Listing |
Pediatr Blood Cancer
December 2024
Department of Pediatric Oncology, Christchurch Hospital, Christchurch, New Zealand.
Pediatr Blood Cancer
December 2024
Pediatric Oncology Unit, HM CIOCC MADRID (Centro Integral Oncológico Clara Campal), Hospital Universitario HM Montepríncipe, HM Hospitales, Madrid, Spain.
Eur J Pediatr
December 2024
Department of Medical Genetics, Dr. Behçet Uz Children's Hospital, Izmir, Turkey.
Unlabelled: The RASopathies are a group of disorders resulting from a germline variant in the genes encoding the Ras/mitogen-activated protein kinase pathway. These disorders include Noonan syndrome (NS), cardiofaciocutaneous syndrome (CFC), Costello syndrome (CS), Legius syndrome (LS), and neurofibromatosis type 1 (NF1), and have overlapping clinical features due to RAS/MAPK dysfunction. In this study, we aimed to describe the clinical and molecular features of patients exhibiting phenotypic manifestations consistent with RASopathies.
View Article and Find Full Text PDFGastroenterology
December 2024
Department of Clinical Genetics, Erasmus University Medical Center - Sophia Children's Hospital, Rotterdam, The Netherlands; Department of Pediatric Surgery, Erasmus University Medical Center - Sophia Children's Hospital, Rotterdam, The Netherlands. Electronic address:
Background And Aims: The enteric nervous system (ENS), comprised of neurons and glia, regulates intestinal motility. Hirschsprung disease (HSCR) results from defects in ENS formation, yet while neuronal aspects have been extensively studied, enteric glia remain disregarded. This study aimed to explore enteric glia diversity in health and disease.
View Article and Find Full Text PDFValue Health
December 2024
Australian Genomics, Melbourne, Australia; Victorian Clinical Genetics Services, Murdoch Children's Research Institute, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia.
Objectives: The multifaceted ways in which genomics can be valuable to clinicians, patients, families, and society are important for informing prioritisation decisions by policy makers. This study aims to develop a standardised, cumulative and preference-weighted Genomic Utility Valuation (GUV) on a scale 0-100%.
Methods: A multicriteria decision analysis (MCDA) was conducted with experts involved in policy, clinical, research, and consumer advocacy leadership in Australia for the valuation of policy priority indicators of genomic utility.
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