Colin J. McMahon MD, Conall Thomas Morgan MB BCh BaO
{"title":"History Repeating: Lessons From Citrus Fruits, Penicillin, and Delayed Adoption in Pediatric Cardiology","authors":"Colin J. McMahon MD, Conall Thomas Morgan MB BCh BaO","doi":"10.1016/j.cjcpc.2026.02.005","DOIUrl":"10.1016/j.cjcpc.2026.02.005","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 4","pages":"Pages 246-248"},"PeriodicalIF":0.0,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148776086","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Luiz Almeida MD, Sunita Vohra MD, MSc, Jeffrey A. Johnson PhD, Erfan Ghasemi PhD, Irina Dinu PhD, Charlene M.T. Robertson MD, Gonzalo Garcia Guerra MD, PhD, Ari R. Joffe MD, Complex Pediatric Therapies Follow-up Program (CPTFP)
{"title":"Comparison of a Cardiac Specific Versus a General Instrument to Assess Health-Related Quality of Life 4-5 Years After Cardiac Surgery in Early Infancy","authors":"Luiz Almeida MD, Sunita Vohra MD, MSc, Jeffrey A. Johnson PhD, Erfan Ghasemi PhD, Irina Dinu PhD, Charlene M.T. Robertson MD, Gonzalo Garcia Guerra MD, PhD, Ari R. Joffe MD, Complex Pediatric Therapies Follow-up Program (CPTFP)","doi":"10.1016/j.cjcpc.2026.02.003","DOIUrl":"10.1016/j.cjcpc.2026.02.003","url":null,"abstract":"<div><h3>Background</h3><div>We aimed to compare health-related quality of life (HRQL) using a disease-specific and a generic instrument in children after complex cardiac surgery in early infancy.</div></div><div><h3>Methods</h3><div>A prospectively followed inception cohort of children having complex cardiac surgery at age ≤6 weeks had HRQL assessed at age 4-5 years using Pediatric Quality of Life Inventory (PedsQL) 3.0 Cardiac Module. Scores were compared with the PedsQL Generic Core Scales in the same population. Predictors of Cardiac Module dimensions were determined by univariate and multivariate linear regression, with <em>P</em> ≤ 0.05 considered statistically significant.</div></div><div><h3>Results</h3><div>Among 425 patients who had the PedsQL 3.0 Cardiac Module, the HRQL mean (standard deviation) scale scores were as follows: Heart Problems and Treatment, 78.7 (19.0); Treatment II, 94.5 (15.2); Perceived Physical Appearance, 93.6 (13.9); Treatment Anxiety, 71.1 (29.5); Cognitive Problems, 67.3 (22.7); and Communication, 67.5 (31.2). Correlations between Cardiac Module and Generic Core Scales were as follows: Heart Problems and Treatment × Physical Functioning 0.57; Perceived Physical Appearance × Psychosocial Summary 0.21; Cognitive Problems × School Functioning 0.48; Treatment Anxiety × Physical Functioning 0.38; and Communication × School Functioning 0.30. Thirteen predictor variables were independently associated with at least 1 dimension of the Cardiac Module, and these only partially overlapped with independent predictors of Generic Core Scale summary scores.</div></div><div><h3>Conclusions</h3><div>The Cardiac Module measured a different HRQL construct than the Generic Core Scales, reflected by low correlations among scores and different independent predictors in the same population of children. Future studies should determine the relation of these scores with longer-term outcomes including school achievement, mental health, and well-being.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 4","pages":"Pages 231-241"},"PeriodicalIF":0.0,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148776084","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Chae Young Kim BHSc, Romina D’Souza MD, Afsaneh Amirabadi PhD, MCCPM, J.A. Gordon Culham MD, Shi-Joon Yoo MD
{"title":"Effect of Aortic Arch Position on the Bronchial Length Ratio in Situs Determination","authors":"Chae Young Kim BHSc, Romina D’Souza MD, Afsaneh Amirabadi PhD, MCCPM, J.A. Gordon Culham MD, Shi-Joon Yoo MD","doi":"10.1016/j.cjcpc.2026.02.001","DOIUrl":"10.1016/j.cjcpc.2026.02.001","url":null,"abstract":"<div><h3>Background</h3><div>Bronchial length ratio (BLR) is widely used for situs determination. However, BLR may also be influenced by the position of the aortic arch. We aimed to evaluate the effect of aortic arch position on BLR.</div></div><div><h3>Methods</h3><div>Patients were identified using the keywords left aortic arch (LAA), right aortic arch (RAA), double aortic arch, situs inversus, right isomerism, and left isomerism. The ratio between the longer and shorter bronchi was calculated. BLR and bronchopulmonary arterial relationship were compared across groups with different combinations of body situs and aortic arch position.</div></div><div><h3>Results</h3><div>Patients included 69 with situs solitus and LAA (group 1A), 20 with situs inversus and RAA (group 1B), 43 with situs solitus and RAA (group 2A), 2 with situs inversus and LAA (group 2B), 11 with situs solitus and double aortic arch (group 3), and 14 with right or left isomerism (group 4). Group 2 demonstrated a significantly smaller BLR than group 1 (<em>P</em> = 0.001). Group 3 showed a smaller BLR than group 1 but without statistical significance. Group 4 had a significantly smaller BLR than groups 1-3 (<em>P</em> < 0.001). None of group 1 had a BLR within the heterotaxy range. BLR ranges of 11% of group 2 and 9% of group 3 overlapped with the heterotaxy range. Bronchopulmonary arterial relationship was consistent with the situs of the remainder of the body in all.</div></div><div><h3>Conclusions</h3><div>BLR is influenced by aortic arch position, limiting its use for situs determination. Bronchopulmonary arterial relationship should be used as the primary criterion for situs determination.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 4","pages":"Pages 249-254"},"PeriodicalIF":0.0,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148776087","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Exercise, Endothelium, and Muscle in Fontan: Cause, Effect, or Common Culprit?","authors":"Ashish H. Shah MD, MD-Research, FRCP","doi":"10.1016/j.cjcpc.2026.02.002","DOIUrl":"10.1016/j.cjcpc.2026.02.002","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 4","pages":"Pages 255-256"},"PeriodicalIF":0.0,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148776088","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Pediatric to Adult Health Care Transition Preparation, Support, and Education Unmet Needs of Families Living With a Kawasaki Disease Diagnosis","authors":"Nita Chahal PhD, Arnelle Lardizabal HBSc, Janet Rush PhD, Tanveer Collins MD, Jessica Weiss MSc, Rita Nobile MSc, Brian W. McCrindle MD","doi":"10.1016/j.cjcpc.2025.10.004","DOIUrl":"10.1016/j.cjcpc.2025.10.004","url":null,"abstract":"<div><h3>Background</h3><div>Pediatric transition programs aim to maximize autonomy, self-care, and lifelong functioning. Feedback from patients with Kawasaki Disease (KD) is valuable for developing a successful transitional program. This survey aimed to build on previous research and explore gaps in support, education, research, timing, and methods for sharing information.</div></div><div><h3>Methods</h3><div>The Supportive Care Framework, an approach for planning and service delivery, guided the development of a 22-item survey (with areas for free-text responses) regarding KD knowledge, gaps, and transition. The sampling frame included online KD websites for voluntary completion by KD patients and parents. Analysis was descriptive.</div></div><div><h3>Results</h3><div>During the 4-month study period in 2024, a total of 438 surveys from KD patients and parents were analyzed. Key areas of concern included health-related issues, long-term consequences, and psychosocial effects. Prominent interests were learning about physical activity, heart-healthy eating, and navigating adult health care. Most preferred methods of learning included webinars, health passports, or KD family education events. Timing for learning varied from at diagnosis, weekly, monthly, yearly, or before transitioning.</div></div><div><h3>Conclusions</h3><div>The findings provide opportunities for enhancing a seamless KD transition program. The use of online methods was prominent for support and learning. Future directions include establishing a transition roadmap for the KD population, which can also serve as a template in transition planning for other chronic disease populations.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 3","pages":"Pages 145-154"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13343442/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148414552","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mavis C. Lui BS, Latha Palaniappan MD, MS, Mary B. Leonard MD, MSCE, Jin Long PhD, Joseph Dehoney BS, John P. Cooke MD, PhD, Inger Olson MD, Tulsi R. Damase PhD, Sharon Chen MD MPH, Jonathan Myers PhD, Daniel Bernstein MD, Seda Tierney MD
{"title":"Exercise Capacity, Endothelial Function, Muscle Mass, and Strength in Pediatric Patients With Fontan Circulation","authors":"Mavis C. Lui BS, Latha Palaniappan MD, MS, Mary B. Leonard MD, MSCE, Jin Long PhD, Joseph Dehoney BS, John P. Cooke MD, PhD, Inger Olson MD, Tulsi R. Damase PhD, Sharon Chen MD MPH, Jonathan Myers PhD, Daniel Bernstein MD, Seda Tierney MD","doi":"10.1016/j.cjcpc.2025.10.010","DOIUrl":"10.1016/j.cjcpc.2025.10.010","url":null,"abstract":"<div><h3>Background</h3><div>Fontan circulation carries high morbidity and mortality, driven in part by impaired exercise capacity, muscle deficits, and endothelial dysfunction. In a pediatric cohort with Fontan circulation, we examined associations between exercise capacity, endothelial function, and muscle parameters before enrollment in a randomized exercise intervention.</div></div><div><h3>Methods</h3><div>Patients aged 8-19 years who have Fontan circulation were included. Exclusion criteria included New York Heart Association class IV, active illness, protein-losing enteropathy, pacemaker, or cognitive delay. Assessments included cardiopulmonary exercise testing (peak VO<sub>2</sub>), endothelial function testing (reactive hyperemia index [RHI]), fasting lipid profile, dual-energy x-ray absorptiometry and dynamometry (muscle mass and strength), and functional movement screening (FMS).</div></div><div><h3>Results</h3><div>Among 137 participants (median age 12.7 years; 53% single right ventricle), median time since Fontan was 8.6 years. Peak VO<sub>2</sub> was 1402 ± 509 mL/min (75.7% ± 17.9% predicted; 31.1 ± 7.4 mL/kg/min indexed). RHI was 1.45 ± 0.6; NOx 22.2 ± 17.4 μmol/L. The low-density lipoprotein cholesterol level was 65.8 ± 18.9 mg/dL. Length-adjusted leg lean mass, handgrip strength, and leg extension strength <em>z</em> scores were –1.02 ± 1.0, –0.76 ± 0.9, and –0.70 ± 1.0, respectively. The FMS score averaged 11.9 ± 3.0. Peak VO<sub>2</sub> correlated with RHI (<em>r</em> = 0.50), lnRHI (<em>r</em> = 0.51), FMS (<em>r</em> = 0.30) (all <em>P</em> < 0.001), and length-adjusted leg lean mass <em>z</em> score (<em>r</em> = 0.25, <em>P</em> = 0.003). Indexed and percent-predicted peak VO<sub>2</sub> also correlated with FMS (<em>r</em> = 0.31 and 0.30, respectively) and high-density lipoprotein (<em>r</em> = 0.24 and <em>r</em> = 0.25, respectively).</div></div><div><h3>Conclusion</h3><div>In youth with Fontan circulation, lower aerobic capacity was associated with lower endothelial function, leg lean mass, strength, and mobility, underscoring the interdependence of cardiovascular, vascular, and musculoskeletal health.</div></div><div><h3>Clinical Trial Registration</h3><div>NCT04195451.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 3","pages":"Pages 119-128"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13343420/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148414598","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Reihane Taheri MSc, RD, Olivia Weaver MSc, Jacqueline A. Krysa PhD, Donna F. Vine PhD, Michael Khoury MD, PhD, Spencer D. Proctor PhD
{"title":"Lipid Screening for Cardiovascular Risk in Youth: A Narrative Review of Current Guidelines and Potential Use of Nonfasting Measures","authors":"Reihane Taheri MSc, RD, Olivia Weaver MSc, Jacqueline A. Krysa PhD, Donna F. Vine PhD, Michael Khoury MD, PhD, Spencer D. Proctor PhD","doi":"10.1016/j.cjcpc.2025.10.006","DOIUrl":"10.1016/j.cjcpc.2025.10.006","url":null,"abstract":"<div><div>Early identification of dyslipidemia in children and youth allows for the prevention of cardiovascular diseases (CVD). Emerging evidence in adults suggests that nonfasting remnant cholesterol (RC) is a significant independent risk factor for CVD. Understanding the applicability of nonfasting RC in youth and children may provide greater options for routine and early identification of CVD risk. The purpose of this review is to provide an overview of guidelines for assessing dyslipidemia in practice and to explore whether nonfasting lipid biomarkers and/or RC have been incorporated for children and youth. The Medline database was used to identify clinical practice guidelines published between 2011 and August 2025 in English for children and/or youth aged 2-20 years. Of an initial 2537 citations, 41 met the inclusion criteria for data extraction. Although there is controversy regarding assessing fasting or nonfasting biomarkers in youth, definitions of normal ranges of lipoproteins were less controversial and are typically defined based on the National Heart, Lung, and Blood Institute recommendations. Most guidelines recommend measuring fasting lipid biomarkers (especially low-density lipoprotein cholesterol) in youth. In contrast, a select number of guidelines and statements recommend assessing nonfasting non–high-density lipoprotein cholesterol as the priority. Currently, no guidelines discuss measuring RC in youth. Evidence suggests that nonfasting non–high-density lipoprotein cholesterol is a reliable, practical biomarker for lipid screening in children and youth; however, most guidelines still suggest fasting measurement of low-density lipoprotein cholesterol. We note that nonfasting RC is an important knowledge gap not addressed in the current guidelines, and increasing awareness of nonfasting RC may be valuable.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 3","pages":"Pages 155-168"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13343429/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148414620","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}