CJC Pediatric and Congenital Heart Disease最新文献

{"title":"Uncorrected Dextro–Transposition of the Great Arteries With Intact Ventricular Septum and Atrial Septal Defect Diagnosed After Developing Heart Failure","authors":"Kohsaku Goto MD, PhD , Akihito Saito MD, PhD , Hiroyuki Tokiwa MD, PhD , Masahiko Umei MD, PhD , Katsura Soma MD, PhD , Imari Mimura MD, PhD , Ryo Inuzuka MD, PhD , Yasutaka Hirata MD, PhD , Koichiro Niwa MD, PhD , Norihiko Takeda MD, PhD , Atsushi Yao MD, PhD","doi":"10.1016/j.cjcpc.2025.06.003","DOIUrl":"10.1016/j.cjcpc.2025.06.003","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 1","pages":"Pages 51-54"},"PeriodicalIF":0.0,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147328814","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Frailty and Cognitive Function After the Age of 40 in Adults With Moderate or Severe Congenital Heart Disease","authors":"Sandra Skogby PhD, MSc, RN ,&nbsp;Christina Christersson MD, PhD ,&nbsp;Joanna Hlebowicz MD, PhD ,&nbsp;Zacharias Mandalenakis MD, PhD ,&nbsp;Eva Goossens PhD, MSc, RN ,&nbsp;Adrienne H. Kovacs PhD ,&nbsp;Liesbet Van Bulck RN, PhD ,&nbsp;Koen Luyckx PhD ,&nbsp;Philip Moons RN, PhD ,&nbsp;Camilla Sandberg RPT, PhD ,&nbsp;Bengt Johansson MD, PhD ,&nbsp;APPROACH-IS II consortium,&nbsp;International Society for Adult Congenital Heart Disease","doi":"10.1016/j.cjcpc.2025.07.003","DOIUrl":"10.1016/j.cjcpc.2025.07.003","url":null,"abstract":"<div><h3>Background</h3><div>Decades of progress in care and treatment for congenital heart disease (CHD) has gradually shifted the research focus from initial survival to long-term prognosis and the ageing of adults with CHD. Knowledge about the ageing adult with CHD will guide interventions to safeguard the quality of life across the life course. The present study compares the prevalence of frailty and cognitive dysfunction between adults with CHD and a control group.</div></div><div><h3>Methods</h3><div>Using a multicentre design, we compared adults with moderate or complex CHD aged ≥40 years, equally distributed across the age groups 40-49, 50-59, and &gt;60 years, with age- and sex-matched controls. We assessed frailty phenotypes using the Fried method and cognitive dysfunction using the Montreal Cognitive Assessment tool.</div></div><div><h3>Results</h3><div>In total, 156 adults with CHD (56.0 ± 10.4 years, 54.4% male) and 86 controls (55.6 ± 11.2 years, 55.8% male) were included in the study. Adults with CHD and controls did not differ in terms of mean score on the Montreal Cognitive Assessment (mean score 27.1 vs 26.9, <em>P</em> = 0.59). Similarly, there was no statistical difference in the prevalence of prefrailty/frailty between adults with CHD and controls (36.5% vs 29.0%, <em>P</em> = 0.26).</div></div><div><h3>Conclusions</h3><div>Prevalence rates of cognitive dysfunction and frailty were similar between adults with CHD and age-matched controls. As more patients, particularly those with complex heart lesions, reach older ages, the prevalence of cognitive impairment and frailty may change.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 1","pages":"Pages 40-48"},"PeriodicalIF":0.0,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147328689","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cardiac Autonomic Function and Atrial Arrhythmias in Adult Patients With a Univentricular Physiology After Fontan Palliation","authors":"Marieke Nederend MD ,&nbsp;Elizaveta Polyakova MSc ,&nbsp;Philippine Kiès MD, PhD ,&nbsp;Arie Maan PhD ,&nbsp;Sum-Che Man MD, PhD ,&nbsp;Marianne Bootsma MD, PhD ,&nbsp;Anastasia D. Egorova MD, PhD ,&nbsp;Monique R.M. Jongbloed MD, PhD","doi":"10.1016/j.cjcpc.2025.05.007","DOIUrl":"10.1016/j.cjcpc.2025.05.007","url":null,"abstract":"<div><h3>Background</h3><div>Cardiac arrhythmias are frequently encountered in patients with single ventricle physiology palliated by the Fontan circulation. Autonomic dysfunction, an imbalance between sympathetic and parasympathetic systems, is critical in arrhythmogenesis. The relationship between impaired autonomic function, cardiac morphology, ventricular function, and clinical outcomes in adult patients remains poorly defined. This study aims to evaluate cardiac autonomic function using heart rate variability and exercise testing and investigate autonomic factors associated with the prevalence of atrial arrhythmias in adult Fontan patients.</div></div><div><h3>Methods</h3><div>Consecutive adult Fontan patients were included in this single-centre, cross-sectional study. Cardiac autonomic function was assessed using 24-hour ambulatory electrocardiogram recordings and exercise testing. Factors associated with atrial arrhythmias were evaluated.</div></div><div><h3>Results</h3><div>Fifty-four patients (median age 22 years, 56% female) were included. Paroxysmal atrial arrhythmias were present in 26% of patients and associated with significantly lower heart rate reserve (60 vs 91 beats per minute [bpm], <em>P</em> = 0.004) and chronotropic index (0.46 vs 0.82, <em>P</em> = 0.006) during exercise testing. Independent of β-blocker use, the autonomic function parameters, standard deviation of normal-to-normal intervals and standard deviation of the average normal-to-normal intervals calculated of all 5-minute intervals, were reduced in patients with atrial arrhythmias. Ventricular arrhythmias were less frequently encountered (7%).</div></div><div><h3>Conclusions</h3><div>Atrial arrhythmias, affecting 26% of this cohort, were associated with reduced heart rate reserve and chronotropic index, partly due to β-blocker use, and decreased standard deviation of normal-to-normal intervals and standard deviation of the average normal-to-normal intervals calculated over 5-minute intervals. This suggests impaired autonomic regulation and adaptability in adult Fontan patients, potentially predisposing to late complications. Addressing autonomic dysfunction may help mitigate arrhythmic risks in this population.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 1","pages":"Pages 23-31"},"PeriodicalIF":0.0,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147329039","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"To Live Long and Prosper: The Final Frontier for Adults With Congenital Heart Disease","authors":"Marnina B. Stimmel PhD ,&nbsp;Ariela R. Orkaby MD, MPH ,&nbsp;Fred Ming-Chieh Wu MD","doi":"10.1016/j.cjcpc.2025.08.004","DOIUrl":"10.1016/j.cjcpc.2025.08.004","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 1","pages":"Pages 49-50"},"PeriodicalIF":0.0,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147328820","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cardiac MRI Diagnosis of Large Aortopulmonary Collateral and Pulmonary Sequestration Presenting as Left Chamber Dilation","authors":"Paul W. Warren MD ,&nbsp;Bradley B. Keller MD ,&nbsp;Aki A. Tanimoto MD ,&nbsp;Russel Hirsch MD ,&nbsp;David G. Lehenbauer MD ,&nbsp;Sean M. Lang MD","doi":"10.1016/j.cjcpc.2025.08.002","DOIUrl":"10.1016/j.cjcpc.2025.08.002","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 1","pages":"Pages 20-22"},"PeriodicalIF":0.0,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147328723","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Systemic Right Ventricle: Is Equal Fitness Truly Equal?","authors":"Ashish H. Shah MD, MD-Research (UK), FRCP (UK)","doi":"10.1016/j.cjcpc.2025.09.004","DOIUrl":"10.1016/j.cjcpc.2025.09.004","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 1","pages":"Page 55"},"PeriodicalIF":0.0,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147328837","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Cluster Randomized Trial of an mHealth Intervention for Adolescents With Congenital Heart Disease: Rationale and Design of the READYorNot CHD Study","authors":"Andrew S. Mackie MD, SM ,&nbsp;Adrienne H. Kovacs PhD ,&nbsp;Daniella San Martin-Feeney MSc ,&nbsp;Alicia Via-Dufresne Ley ,&nbsp;Brian W. McCrindle MD, MPH ,&nbsp;Kevin C. Harris MD ,&nbsp;Anne Fournier MD ,&nbsp;Alyssa Chappell BScN, RN ,&nbsp;Jody Gingrich BScN, RN ,&nbsp;Gina Dimitropoulos PhD ,&nbsp;Brooke Allemang PhD ,&nbsp;Navreet Gill RN, MN ,&nbsp;Sandra Aiello RN ,&nbsp;Martha Rolland RN, BA ,&nbsp;Sunita O’Shea MA ,&nbsp;Lea Legge BScN ,&nbsp;Jennifer A. Collins BA ,&nbsp;Susan Casey RN ,&nbsp;Fabiola Breault BScN ,&nbsp;Frederique Provencher BScN ,&nbsp;Ariane J. Marelli MD, MPH","doi":"10.1016/j.cjcpc.2025.06.001","DOIUrl":"10.1016/j.cjcpc.2025.06.001","url":null,"abstract":"<div><div>The population of adolescents with congenital heart disease (CHD) is growing exponentially and requires transition preparation for adult-oriented health care. Nurse-led transition programs are effective in improving CHD knowledge and self-management skills. However, many clinical programs lack the human resources needed to provide transition services. Mobile health applications have the potential to prepare transition-age youth for entering adult health care. However, there are no outcome data on the impact and effectiveness of CHD transition applications. Accordingly, in partnership with a Youth Advisory Council, we developed the MyREADY Transition CHD App (the App) designed to enhance youth CHD knowledge and self-management skills. The READYorNot CHD study is a multicenter, cluster randomized noninferiority clinical trial that is evaluating the efficacy of the App plus limited nurse teaching (intervention), vs comprehensive nurse-only teaching (control) for 16- to 17-year-olds with moderate or complex CHD. Participants are being enrolled in clusters based on week of attendance in the pediatric cardiology clinic, with a 1:1 allocation between intervention vs control and target recruitment of 204 participants. The primary outcome is the change in Transition Readiness Assessment Questionnaire score from baseline to 18 months. Secondary outcomes are change in CHD knowledge score, self-efficacy, and time to first adult CHD appointment. Semistructured interviews will provide additional insights into the advantages and disadvantages of the App vs nurse-only teaching. This study will inform patients, pediatric cardiology programs, and policy makers in judging whether this mobile health intervention warrants widespread availability in clinical settings to improve transition outcomes of adolescents with CHD. Clinical Trial Registration: <span><span>NCT04463446</span><svg><path></path></svg></span>.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 1","pages":"Pages 1-11"},"PeriodicalIF":0.0,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147328975","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Outcomes of Cardiac Repair in Heterotaxy Syndrome Patients—Tertiary Center Experience","authors":"Mohammad A. Ebrahim MD ,&nbsp;Nada T. Alzayed MD ,&nbsp;Sakeena M. Alsahhaf MD ,&nbsp;Mohammad A. Almulla MD ,&nbsp;Karim M. Yassin MD ,&nbsp;Leina A. Shabaan BSc ,&nbsp;Moustafa A. Elsayed MD ,&nbsp;Vadim G. Lyubomudrov MD","doi":"10.1016/j.cjcpc.2025.06.005","DOIUrl":"10.1016/j.cjcpc.2025.06.005","url":null,"abstract":"<div><h3>Background</h3><div>This study aims to determine factors associated with poor outcomes and frequency of arrhythmia incidence in patients with heterotaxy syndrome (HS).</div></div><div><h3>Methods</h3><div>A retrospective cohort study in a single tertiary center was conducted and included patients with operated HS between 2011 and 2020. A total of 52 patients were included. Relevant factors associated with mortality that were considered included univentricular (UV) or biventricular (BV) physiology, prematurity, low weight/age at surgery, the presence of atrioventricular valve regurgitation, anomalous pulmonary veins drainage, and type of atrial isomerism.</div></div><div><h3>Results</h3><div>Thirty-three patients (63.4%) were diagnosed with left atrial isomerism (LAI), whereas the remaining 19 (36.5%) were diagnosed with right atrial isomerism (RAI). Thirty-eight patients (73%) underwent UV repair, whereas 14 patients (27%) had BV physiology. Patients were followed up for an average of 4.8 years. Lastly, 14 patients (27%) had died during the follow-up period. Notably, most patients with complete repair are among the LAI group, and high-grade heart block only occurred in patients with LAI. Moreover, patients with LAI were more likely to receive BV repair, whereas patients with RAI were more likely to undergo UV repair (<em>P</em> = 0.008). In addition, pulmonary venous anomalies occurred more frequently among the RAI group. Expectedly, nonsinus rhythm was frequently present among the cohort. Finally, the mortality rate was significantly higher among the RAI group (42% vs 18%, <em>P</em> = 0.06).</div></div><div><h3>Conclusion</h3><div>Managing HS patients with UV physiology still remains a challenge. Risk factors for mortality included lower initial operation age, and RAI approached significance. These data may assist with risk stratification and patient counseling.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 1","pages":"Pages 12-19"},"PeriodicalIF":0.0,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147328816","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Information for Readers","authors":"","doi":"10.1016/S2772-8129(26)00003-5","DOIUrl":"10.1016/S2772-8129(26)00003-5","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 1","pages":"Page A1"},"PeriodicalIF":0.0,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147413210","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pan-Canadian Registry, Pan-System Disease: Rethinking Fontan Pathobiology","authors":"Ashish H. Shah MD, MD-Research (UK)","doi":"10.1016/j.cjcpc.2025.10.008","DOIUrl":"10.1016/j.cjcpc.2025.10.008","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"4 6","pages":"Page 338"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145750136","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}