Camille Dufour, Louis-Olivier Roy MSc, Valérie Taillefer MD, Maxime Duclos BSc, Evelyne Raiche MD, Tiscar Cavallé-Garrido MD, Gitanjali Mansukhani MD FRACP, Richard Brown MD, FRCOG, Varsha Thakur MD, Alexandra Ferland-Dorval MD, Rami Al-Amir-Dach MD, Melinda Hall MD, Laurence Vaujois MD, Frédéric Dallaire MD, PhD, Canadian Congenital and Pediatric Cardiology Research Network
{"title":"Psychological Impact of Fetal Echocardiography Referral on Pregnant Women With a Normal Routine Morphologic Ultrasound","authors":"Camille Dufour, Louis-Olivier Roy MSc, Valérie Taillefer MD, Maxime Duclos BSc, Evelyne Raiche MD, Tiscar Cavallé-Garrido MD, Gitanjali Mansukhani MD FRACP, Richard Brown MD, FRCOG, Varsha Thakur MD, Alexandra Ferland-Dorval MD, Rami Al-Amir-Dach MD, Melinda Hall MD, Laurence Vaujois MD, Frédéric Dallaire MD, PhD, Canadian Congenital and Pediatric Cardiology Research Network","doi":"10.1016/j.cjcpc.2026.05.005","DOIUrl":"10.1016/j.cjcpc.2026.05.005","url":null,"abstract":"<div><h3>Background</h3><div>Fetal echocardiography (FE) is widely recommended in pregnancies at increased risk of congenital heart defects. Although FE is a safe diagnostic tool, its psychological impact on expectant pregnant women remains poorly understood.</div></div><div><h3>Methods</h3><div>We conducted a prospective observational study in 8 Canadian centers. Women aged ≥18 years with a normal second-trimester ultrasound between 18 and 26 weeks’ gestation referred for an FE were compared with women who were not. Anxiety was assessed using the Spielberger State-Trait Anxiety Inventory. The primary outcome was the difference between state and trait anxiety (net situational anxiety) after morphologic ultrasound but before FE (T0). Secondary outcomes included the proportion of participants with significant anxiety (State-Trait Anxiety Inventory ≥40) and anxiety trajectories after FE among referred participants.</div></div><div><h3>Results</h3><div>Eighty-four women were included, of whom 38 were referred for FE. At T0, women referred for FE showed slightly higher median net situational anxiety compared with controls. In multivariable analyses, FE referral was associated with a modest increase although estimates were imprecise. A higher proportion of referred women reached a state anxiety score ≥40, though this association was weak after adjustment. Among the 14 women who completed post-FE questionnaires, anxiety trajectories were heterogeneous, with both increases and decreases observed after FE.</div></div><div><h3>Conclusions</h3><div>Referral for FE after a normal second-trimester ultrasound is associated with a small and variable increase in maternal anxiety. These findings suggest that FE referral may not be psychologically neutral and highlight the importance of considering maternal psychological responses as referral criteria broaden.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 5","pages":"Pages 345-350"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149219080","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}
Rawan Amir MBBS, Jong Mi Ko MA, Shelby Kutty MD, PhD, Liesbet Van Bulck PhD, RN, Jamie L. Jackson PhD, Maryanne Caruana MD, PhD, Susan M. Jameson MHP, LPD, PA-C, Vaikom S. Mahadevan MD, Philip Moons PhD, RN, Ari Cedars MD, APPROACH-IS II consortium, International Society for Adults Congenital Heart Disease (ISACHD)
{"title":"Association Between a Novel Adult Congenital Heart Disease Patient-Reported Outcome Metric and New York Heart Association Functional Classification","authors":"Rawan Amir MBBS, Jong Mi Ko MA, Shelby Kutty MD, PhD, Liesbet Van Bulck PhD, RN, Jamie L. Jackson PhD, Maryanne Caruana MD, PhD, Susan M. Jameson MHP, LPD, PA-C, Vaikom S. Mahadevan MD, Philip Moons PhD, RN, Ari Cedars MD, APPROACH-IS II consortium, International Society for Adults Congenital Heart Disease (ISACHD)","doi":"10.1016/j.cjcpc.2026.04.002","DOIUrl":"10.1016/j.cjcpc.2026.04.002","url":null,"abstract":"<div><div>Patient-reported outcomes (PROs) are important for clinical care. As part of the Adults with Congenital Heart Disease International Study-II, we included an English language version of the novel adult congenital heart disease (ACHD)–specific PRO metric, the ACHD PRO, for distribution at English-speaking sites. We found that ACHD PRO scores correlate with New York Heart Association functional class, with an 11-point change in summary score reflecting a 1-category change in New York Heart Association class. These results provide further validation of the ACHD PRO as a tool and suggest clinically meaningful thresholds for differences in the ACHD PRO summary score.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 5","pages":"Pages 291-293"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149219031","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}
Mohamed Ellabbad MBBCh, Ahmed Bahnasy MBBCh, Meena Bai MBBS, Sara Aboelmaaty MBBCh, Ahmed E. Ali MBBCh, William R. Miranda MD, Heidi M. Connolly MD, Alexander C. Egbe MD, MPH, MS
{"title":"Prognostic Implication of Heart Failure With Reduced and Midrange Ejection Fraction in Adults With Congenital Heart Disease","authors":"Mohamed Ellabbad MBBCh, Ahmed Bahnasy MBBCh, Meena Bai MBBS, Sara Aboelmaaty MBBCh, Ahmed E. Ali MBBCh, William R. Miranda MD, Heidi M. Connolly MD, Alexander C. Egbe MD, MPH, MS","doi":"10.1016/j.cjcpc.2026.02.006","DOIUrl":"10.1016/j.cjcpc.2026.02.006","url":null,"abstract":"<div><h3>Background</h3><div>In the acquired heart disease population, heart failure (HF) is broadly classified based on left ventricular (LV) ejection fraction (EF) as HF with reduced EF (HFrEF), HF with midrange EF (HFmrEF), and HF with preserved EF (HFpEF). The prognostic role of this LVEF-based HF classification has not been evaluated in the adult congenital heart disease (CHD) population because of anatomic and physiological heterogeneity within this population. The current study aimed to address this knowledge gap, by assessing the prevalence, predictors (correlates), and prognostic implications of HFrEF in adults with CHD.</div></div><div><h3>Methods</h3><div>This is a retrospective cohort study of adults with CHD and morphologic LV as the systemic ventricle. Patients were classified as HFrEF (LVEF ≤40%), HFmrEF (LVEF 41%-49%), and pEF (LVEF ≥50%).</div></div><div><h3>Results</h3><div>Of 7981 patients, 7203 (90%) had pEF, 451 (6%) had HFmrEF, and 327 (4%) had HFrEF. The correlates of HFrEF were older age, male sex, pacemaker implantation, multiple prior sternotomies, and acquired comorbidities such as hypertension, coronary artery disease, diabetes, and atrial fibrillation. The 15-year mortality risk was 18%, 36%, and 67% in the pEF, HFmrEF, and HFrEF groups, respectively (<em>P</em> < 0.001). Both HFmrEF (adjusted hazard ratio: 1.64 [95% confidence interval: 1.29; 2.09], <em>P</em> < 0.001) and HFrEF (adjusted hazard ratio: 3.33 [95% confidence interval: 2.72; 4.27], <em>P</em> < 0.001) were associated with mortality on multivariable analysis.</div></div><div><h3>Conclusions</h3><div>Given the elevated risk of mortality in patients with LV systolic dysfunction (both mrEF and rEF) and the emerging data suggesting the efficacy of HF therapy in patients with CHD, there is an urgent need to implement HF therapy in this population.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 4","pages":"Pages 258-266"},"PeriodicalIF":0.0,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148776090","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}
Jessica N. Zvonkovic PhD, Charlene M.T. Robertson MD, Erfan Ghasemi MSc, Irina A. Dinu PhD, Ari R. Joffe MD, Complex Pediatric Therapies Follow-Up Program
{"title":"Executive Functioning in Kindergarten-Aged Children After Complex Cardiac Surgery in Early Infancy","authors":"Jessica N. Zvonkovic PhD, Charlene M.T. Robertson MD, Erfan Ghasemi MSc, Irina A. Dinu PhD, Ari R. Joffe MD, Complex Pediatric Therapies Follow-Up Program","doi":"10.1016/j.cjcpc.2026.01.002","DOIUrl":"10.1016/j.cjcpc.2026.01.002","url":null,"abstract":"<div><h3>Background</h3><div>Executive function (EF) challenges occur in older children after complex cardiac surgery and can affect multiple aspects of daily life.</div></div><div><h3>Methods</h3><div>This is a prospective inception-cohort study of children after complex cardiac surgery (2009-2019) in early infancy assessed at age 4.5 years using the Behavior Rating Inventory of Executive Function Preschool Version. Independent predictors of clinically significant EF difficulties (T scores ≥65) were determined using multiple logistic regressions, reported as odds ratios (OR) with 95% confidence intervals (CI).</div></div><div><h3>Results</h3><div>EF outcomes were available for 318 of 581 (55%) eligible survivors; 20.4% showed clinically significant global EF difficulties. In model 1, independent predictors included birth year for the Inhibitory Self-Control Index, maternal years of education for the Emergent Metacognition Index and Global Executive Composite (GEC), and the number of medical specialists at age 4.5 years for GEC. In model 2, which excluded more distal variables, independent predictors for the Inhibitory Self-Control Index were birth year (OR: 1.16, 95% CI: 1.04, 1.31; <em>P</em> = 0.008) and life-saving support (OR: 2.59, 95% CI: 1.14, 5.74; <em>P</em> = 0.021); maternal education predicted the Emergent Metacognition Index (OR: 0.84, 95% CI: 0.74, 0.96; <em>P</em> = 0.011); and maternal education (OR: 0.82, 95% CI: 0.71, 0.93; <em>P</em> = 0.003) and life-saving support (OR: 2.41, 95% CI: 1.08, 5.18; <em>P</em> = 0.027) predicted GEC.</div></div><div><h3>Conclusions</h3><div>The findings highlighted the risk of emerging EF difficulties in young children, especially those needing life-saving support. A study is needed to better understand how home environmental factors influence EF outcomes. Given the broad developmental impact of EF, early intervention may offer widespread, cumulative benefits.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 4","pages":"Pages 193-204"},"PeriodicalIF":0.0,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148776080","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}
Nairy Khodabakhshian PhD, Vitor C. Guerra MD, PhD, Wei Hui MD, Cheryl Fackoury RDCS, Barbara Cifra MD, Luc L. Mertens MD, PhD
{"title":"Vascular and Ventricular Responses Assessed by Exercise Stress Echocardiography in Pediatric Marfan Syndrome","authors":"Nairy Khodabakhshian PhD, Vitor C. Guerra MD, PhD, Wei Hui MD, Cheryl Fackoury RDCS, Barbara Cifra MD, Luc L. Mertens MD, PhD","doi":"10.1016/j.cjcpc.2026.02.004","DOIUrl":"10.1016/j.cjcpc.2026.02.004","url":null,"abstract":"<div><h3>Background</h3><div>Pediatric Marfan syndrome (MFS) is associated with changes in aortic stiffness, endothelial function, and ventricular performance. Resting measurements may not fully capture cardiovascular functional reserve. Exercise stress echocardiography can reveal subclinical impairments not evident at rest.</div></div><div><h3>Methods</h3><div>This study included 31 pediatric patients with and 31 age-matched controls. Resting and exercise echocardiography were performed to assess systolic and diastolic function, aortic dimensions, and vascular parameters.</div></div><div><h3>Results</h3><div>Patients with MFS had larger baseline height and body surface area values compared with controls. Significant baseline differences in aortic dimensions, diastolic function, endothelial function, and vascular stiffness were observed in patients with MFS. Despite these differences, patients with MFS demonstrated preserved systolic and diastolic reserve during exercise. Vascular responses to exercise were comparable to controls, reflecting increased stiffness and altered endothelial function but preserved adaptability.</div></div><div><h3>Conclusions</h3><div>Preserved cardiovascular reserve during exercise suggests that baseline abnormalities may not result in significant dysfunction, supporting safe participation in endurance activities for pediatric patients with MFS.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 4","pages":"Pages 216-230"},"PeriodicalIF":0.0,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148776082","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 Intolerance After Fontan: Converging Vascular, Muscle Mass, and Metabolic Pathways","authors":"Mavis C. Lui BS, Seda Tierney MD","doi":"10.1016/j.cjcpc.2026.01.003","DOIUrl":"10.1016/j.cjcpc.2026.01.003","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 4","pages":"Page 257"},"PeriodicalIF":0.0,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148776089","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}
Matthew S. Chapelski PhD, Leah J. Ferguson PhD, Amanda Froehlich Chow PhD, Charissa Pockett MD, Kristi D. Wright PhD, Corey R. Tomczak PhD, Marta C. Erlandson PhD
{"title":"Effect of a 6-Month Chronic Disease Management Program on the Physical Literacy of Children With Congenital Heart Disease: A CHAMPS Cohort Study∗","authors":"Matthew S. Chapelski PhD, Leah J. Ferguson PhD, Amanda Froehlich Chow PhD, Charissa Pockett MD, Kristi D. Wright PhD, Corey R. Tomczak PhD, Marta C. Erlandson PhD","doi":"10.1016/j.cjcpc.2025.12.002","DOIUrl":"10.1016/j.cjcpc.2025.12.002","url":null,"abstract":"<div><h3>Background</h3><div>Children with congenital heart disease (CHD) have sequelae related to their heart defect that can affect their lifelong health. Physical literacy development is one way to improve their health because it provides them the competence and confidence to engage in physical activity; however, it may be impaired in children with CHD. Therefore, we sought to determine the effect of a 6-month chronic disease management program on physical literacy development of children with CHD.</div></div><div><h3>Methods</h3><div>Thirteen children with CHD participated in the program and were age and sex matched to 12 typically developing peers (TDPs) who served as the control population. All participants had their motor competence (PLAYfun), confidence (PLAYself), and parental perception of the child’s physical literacy (PLAYparent) measured before and after the program. A 2 × 2 between-within factorial multivariate analysis of covariance measured the effectiveness of the program while controlling for age, sex, height, weight, maturity, and self-reported physical activity.</div></div><div><h3>Results</h3><div>The 6-month program significantly improved the skip, gallop, and locomotor motor competence in children with CHD (<em>P <</em> 0.05), whereas the TDP group displayed no change (<em>P ></em> 0.05). At baseline, children with CHD had lower locomotor, object control, and total motor competence compared with TDPs (<em>P <</em> 0.05). At follow-up, children with CHD had lower balance and total motor competence compared with TDPs (<em>P <</em> 0.05). No change was found in parental perception of physical literacy or the children’s confidence for either group (<em>P ></em> 0.05).</div></div><div><h3>Conclusions</h3><div>Children with CHD had impaired motor competence at baseline, and although the program did result in an improvement in the locomotor domain, it was not able to mitigate all differences in physical literacy.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 4","pages":"Pages 205-215"},"PeriodicalIF":0.0,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148776081","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}
Michael E. Kim DO, MEd, Azadeh Assadi PhD, RN, MN-NP, Daniel Ehrmann MD, Will Dixon BS, Spencer Vecile MESc, Robert Greer MSc, Sebastian Goodfellow PhD, Anica Bulic MD
{"title":"Clinician Performance in Training Data Curation for an Arrhythmia Machine Learning Model: Is Anyone Qualified?","authors":"Michael E. Kim DO, MEd, Azadeh Assadi PhD, RN, MN-NP, Daniel Ehrmann MD, Will Dixon BS, Spencer Vecile MESc, Robert Greer MSc, Sebastian Goodfellow PhD, Anica Bulic MD","doi":"10.1016/j.cjcpc.2026.03.003","DOIUrl":"10.1016/j.cjcpc.2026.03.003","url":null,"abstract":"<div><h3>Background</h3><div>Arrhythmia identification in the intensive care unit (ICU) is important to prevent ICU morbidity and mortality. Timely arrhythmia detection relies on bedside providers’ telemetry interpretation. Machine learning models can function as clinical support tools to facilitate diagnoses. Machine learning model development requires well-curated training data. The differential performance between labelers of different roles and experience is currently unknown.</div></div><div><h3>Methods</h3><div>This was a prospective observational study with frontline providers. A total of 300 (200 original and 100 duplicate) telemetry tracings were labeled as sinus rhythm, second-/third-degree atrioventricular block, junctional ectopic tachycardia, ectopic atrial tachycardia, and reentrant supraventricular tachycardia. Inter-rater reliability was calculated against the ground truth label (read by an electrophysiologist, AB) as the primary performance measure (intrarater reliability for consistency using duplicate labels).</div></div><div><h3>Results</h3><div>A total of 11 participants completed the study: 1 cardiology fellow, 4 pediatric ICU fellows, 2 pediatric cardiac ICU fellows, 3 pediatric cardiac ICU nurse practitioners, and 1 pediatrics resident. The highest level of agreement was moderate (κ = 0.68, <em>P</em> < 0.001), with the majority poor to moderate. Performance varied by rhythm type (median κ): sinus (0.61), atrioventricular block (0.68), junctional (0.47), ectopic atrial tachycardia (0.25), and supraventricular tachycardia (0.49). There was good intrarater reliability (κ = 0.71 [median], <em>P</em> < 0.001).</div></div><div><h3>Conclusions</h3><div>Overall, frontline provider performance was suboptimal, especially for complex arrhythmia classes. These findings highlight the need for thoughtful consideration in labeler training and validate the need for a clinical decision support tool in arrhythmia detection.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 4","pages":"Pages 271-277"},"PeriodicalIF":0.0,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148776092","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}