Sean D. Smith MD , Eiad Habib MBBS , Tala B. Shahin MD , Amani Elshaer MBBS , Claire Yee PhD , Elaina A. Blickenstaff PhD , Francois Marcotte MD , Alexander Egbe MD , Heidi M. Connolly MD , Joseph Dearani MD , Hartzell V. Schaff MD , David S. Majdalany MD
{"title":"Long-term Outcomes in Anomalous Left Coronary Artery From the Pulmonary Artery Syndrome: A Multicenter Half-Century Experience","authors":"Sean D. Smith MD , Eiad Habib MBBS , Tala B. Shahin MD , Amani Elshaer MBBS , Claire Yee PhD , Elaina A. Blickenstaff PhD , Francois Marcotte MD , Alexander Egbe MD , Heidi M. Connolly MD , Joseph Dearani MD , Hartzell V. Schaff MD , David S. Majdalany MD","doi":"10.1016/j.cjcpc.2025.07.005","DOIUrl":"10.1016/j.cjcpc.2025.07.005","url":null,"abstract":"<div><h3>Background</h3><div>Anomalous left coronary artery from the pulmonary artery (ALCAPA) syndrome is a congenital anomaly that presents in infancy or rarely in adulthood and generally requires surgical correction. Data on long-term sequelae are minimal.</div></div><div><h3>Methods</h3><div>All patients with ALCAPA seen within our hospital system from 1965 to 2022 were reviewed. Patients with other structural heart diseases were excluded.</div></div><div><h3>Results</h3><div>Thirty-one patients were identified, with 9 patients (29.0%) being diagnosed as adults. The cohort was divided into pediatric (<18 years) and adult (≥18 years) subgroups. Heart failure was the most common presentation in pediatric patients (36.4%), whereas dyspnea and chest pain were more common in adults (44.4% and 33.3%, respectively). All patients underwent surgical repair. Overall survival was 93.4% at 1 year and 5 years (95% confidence interval [CI]: 85-100) and 83.6% at 10 years (95% CI: 69.8-100). The need for reintervention was 0% at 1 year, 15.8% at 5 years (95% CI: 0-31), and 29.8% at 10 years (95% CI: 3.4-49). Preoperative ejection fraction (left ventricular ejection fraction [LVEF]) was 51% for pediatric patients and 43% for adults; follow-up LVEF was 61% among pediatric patients and 55% among adults. Significant mitral valve regurgitation (MR) was noted on the preoperative echocardiogram in 30.8% of pediatric patients and 42.9% of adults. The prevalence of MR decreased to 11.8% in pediatric patients, but it was still 40% in adults at long-term follow-up.</div></div><div><h3>Conclusion</h3><div>ALCAPA has variable presentations in childhood and adulthood. Overall survival after surgery is excellent. LVEF improved throughout the cohort and MR improved in pediatric patients. Reintervention rates increased over time.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 2","pages":"Pages 57-64"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147727131","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}
Animesh Tandon MD, MS , Paige Ewing MPH , Mark Abboud BA , Colin K. Drummond PhD, MBA , Abigail C. Demianczyk PhD , Sabahat Hizlan BA, BS , Bradley S. Marino MD, MPP, MSCE, MBA , Rashmi Rao MD , Jennifer N. Avari Silva MD , MaryBeth Mercer MPH
{"title":"Use of and Experience With Wearable Biosensors in Congenital Heart Disease: A Survey Study","authors":"Animesh Tandon MD, MS , Paige Ewing MPH , Mark Abboud BA , Colin K. Drummond PhD, MBA , Abigail C. Demianczyk PhD , Sabahat Hizlan BA, BS , Bradley S. Marino MD, MPP, MSCE, MBA , Rashmi Rao MD , Jennifer N. Avari Silva MD , MaryBeth Mercer MPH","doi":"10.1016/j.cjcpc.2025.10.002","DOIUrl":"10.1016/j.cjcpc.2025.10.002","url":null,"abstract":"<div><div>Wearable biosensors (“wearables”) are increasingly used by consumers to monitor their health, but their use in congenital heart disease is understudied. This survey study aimed to define wearable use in congenital heart disease. We found that patients and parents tended to buy wearables because they expected good utility, that they were generally happy with the experience of using the wearables, and that those who did not purchase wearables did not expect to get useful data. These findings suggest that utility may be a key driver of patient uptake of wearables.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 2","pages":"Pages 75-79"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147727135","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":"Partial Anomalous Pulmonary Venous Connection in Turner Syndrome: The Challenge of the Right Follow-up","authors":"Estelle Tenisch MD , Tobias Rutz MD","doi":"10.1016/j.cjcpc.2025.10.009","DOIUrl":"10.1016/j.cjcpc.2025.10.009","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 2","pages":"Page 81"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147727061","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}
Mohamed Al Nasef FRCPCH , Bandar Al Shehri MD , Khalid Al Naji MD , Ahmed Al Zahrani MD , Merna Atiyah MD , Omar Abdulaziz Almohizy MD , Saria Mansour Ahmed MD , Khalid Al Najashi MD
{"title":"The Initial Middle Eastern Experience With the Alterra Adaptive Pre-Stent: Single-Center Outcomes From Saudi Arabia","authors":"Mohamed Al Nasef FRCPCH , Bandar Al Shehri MD , Khalid Al Naji MD , Ahmed Al Zahrani MD , Merna Atiyah MD , Omar Abdulaziz Almohizy MD , Saria Mansour Ahmed MD , Khalid Al Najashi MD","doi":"10.1016/j.cjcpc.2025.09.003","DOIUrl":"10.1016/j.cjcpc.2025.09.003","url":null,"abstract":"<div><h3>Background</h3><div>The Alterra Adaptive Pre-Stent, used with the Edwards SAPIEN 3 valve, offers a novel transcatheter option for patients with severely dilated right ventricular outflow tracts (RVOTs) previously unsuitable for conventional transcatheter pulmonary valve replacement (TPVR). No prior experience with this system has been reported from the Middle East.</div></div><div><h3>Methods</h3><div>We report the first Middle Eastern experience with the Alterra Adaptive Pre-Stent from a high-volume congenital heart center. A retrospective, single-center analysis was performed at Prince Sultan Cardiac Center (Riyadh, Saudi Arabia). Between April and July 2025, a total of 10 patients with native (after balloon valvuloplasty) or surgically patched RVOTs underwent TPVR using the Alterra Pre-Stent and Edwards SAPIEN 3 valve. Patient selection, procedural data, and early clinical outcomes were reviewed.</div></div><div><h3>Results</h3><div>All 10 patients (mean age: 30 ± 7.1 years; range: 15-40 years) underwent successful implantation. The mean weight was 66.4 ± 22.7 kg (range: 35-103 kg), and 30% (n = 3) were male. Procedural success was 100%, with accurate device positioning and no significant residual gradient or pulmonary regurgitation. No major complications—including valve embolization, coronary compression, or surgical conversion—occurred. At a median follow-up of 31 days (range: 10-43 days), all patients remained clinically stable with improved functional status.</div></div><div><h3>Conclusions</h3><div>This is the first reported experience of the Alterra Adaptive Pre-Stent in the Middle East. Early results demonstrate that the device is safe, technically feasible, and effective in selected patients with complex RVOT anatomies, potentially broadening TPVR applicability in the region.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 2","pages":"Pages 107-114"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147727132","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}
Jodie Beuth MBBS , Sara Thorne MBBS, MD , Brigitte Mueller PhD , David Barron MD , Adrienne Kovacs PhD , Rafael Alonso-Gonzalez MD , Heather Ross MD , Erwin Oechslin MD , Loretta TS. Ho MD , Jane Heggie MD
{"title":"Socioeconomic Barriers for Newly Transferred Young Adults With Congenital Heart Disease","authors":"Jodie Beuth MBBS , Sara Thorne MBBS, MD , Brigitte Mueller PhD , David Barron MD , Adrienne Kovacs PhD , Rafael Alonso-Gonzalez MD , Heather Ross MD , Erwin Oechslin MD , Loretta TS. Ho MD , Jane Heggie MD","doi":"10.1016/j.cjcpc.2025.08.005","DOIUrl":"10.1016/j.cjcpc.2025.08.005","url":null,"abstract":"<div><h3>Background</h3><div>This study aimed to ascertain socioeconomic factors affecting successful transfer to adult congenital cardiology and cardiac surgical services in Ontario.</div></div><div><h3>Methods</h3><div>Patients with congenital heart disease (CHD) referred from a pediatric CHD program to an adult CHD (ACHD) center between January 1, 2004, and December 31, 2015, were identified. The prevalence of (1) failed transfer (FT), (2) lost to follow-up (LTFU), and (3) cardiac surgery (CS) during the extended study period of 2004-2018 was investigated. Socioeconomic variables associated with FT, LTFU, and CS were explored using Environics data associated with postal code at the time of transfer.</div></div><div><h3>Results</h3><div>A total of 2196 patients were referred from the pediatric to ACHD center between 2004 and 2015. Within this cohort, 11% had FT and 25% had LTFU; there was a 2% overlap between the FT and LTFU groups. A total of 106 patients (4.8%) underwent CS. Age at referral (odds ratio [OR]: 0.591, <em>P</em> < 0.001) and being unable to travel to work by car (OR: 0.986, <em>P</em> < 0.001) were both associated with FT, though the latter is not clinically relevant. Residential addresses with lower income (OR: 0.976, <em>P</em> = 0.016) were associated with LTFU. Factors associated with CS were higher household income (<em>P</em> < 0.001), access to a car for travel to work (<em>P</em> < 0.001), Canadian citizenship (<em>P</em> = 0.041), and French or English as the primary language in the home (<em>P</em> = 0.038).</div></div><div><h3>Conclusions</h3><div>Socioeconomic factors are associated with access to specialized ACHD services among young adults. Strategies to ensure equity in care should be explored.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 2","pages":"Pages 82-92"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147727137","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}
Louis-Olivier Roy MSc , Marc-Olivier Pouliot , Reena Clarkson BA , Andrew S. Mackie MD, SM , Isabelle Vonder Muhll MD , Judith Therrien MD , Kevin C. Harris MD, MHSc , Luc Mertens MD, PhD , Paul Khairy MD, PhD , Rachel Wald MD , Jasmine Grewal MD , Frédéric Dallaire MD, PhD
{"title":"The Canadian Fontan Connection (CANFON): Protocol for a National Lifespan Cohort of People Living With a Fontan Circulation","authors":"Louis-Olivier Roy MSc , Marc-Olivier Pouliot , Reena Clarkson BA , Andrew S. Mackie MD, SM , Isabelle Vonder Muhll MD , Judith Therrien MD , Kevin C. Harris MD, MHSc , Luc Mertens MD, PhD , Paul Khairy MD, PhD , Rachel Wald MD , Jasmine Grewal MD , Frédéric Dallaire MD, PhD","doi":"10.1016/j.cjcpc.2025.09.001","DOIUrl":"10.1016/j.cjcpc.2025.09.001","url":null,"abstract":"<div><div>The life expectancy of people living with a Fontan circulation has improved significantly over recent decades. However, this population remains at risk of multiorgan complications and premature mortality with advancing age. Despite growing clinical experience, key knowledge gaps remain in our understanding of Fontan-related morbidity, including the timing, predisposition and progression of complications, their cumulative burden, and their long-term impact on quality of life and functional outcomes. To address these challenges, we establish Canadian Fontan Connection (CANFON), a national, longitudinal cohort of individuals with single ventricle physiology who are either eligible for or living with a Fontan circulation. The design and implementation of CANFON were developed through engagement with Canadian congenital heart disease experts and alignment with international research priorities. Comprehensive clinical data are collected and banked from birth onward, providing an in-depth, lifelong view of each participant’s health. In parallel, participant-reported outcome measures are prospectively collected over a 5-year period. Recruitment occurs in 24 pediatric and adult congenital heart disease centers across Canada. The overarching objectives of CANFON are to (1) establish the health trajectories of Fontan physiology across the lifespan, (2) determine the prevalence and evolution of complications and modifying factors, (3) identify early predictors and risk factors for Fontan failure, and (4) assess the impact of complications on quality of life and functional status through participant-reported outcomes. Beyond its immediate goals, CANFON will serve as a unique, well-phenotyped, pan-Canadian research platform to support nested studies and accelerate progress in understanding and improving outcomes for individuals living with a Fontan circulation nationally and internationally.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 2","pages":"Pages 65-74"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147727134","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}
Cylia Houchi MSc , Marie-Joëlle Marcil MSc , Paul Khairy MD, PhD , Lena Rivard MD , Marie-Pierre Dubé PhD , Marie-Alexandre Chaix MD, PhD , François-Pierre Mongeon MD, SM , Annie Dore MD , Blandine Mondésert MD, FCHRS , Reda Ibrahim MD , Judith Brouillette MD, PhD
{"title":"Mapping the Biopsychosocial and Lifestyle Portrait of Adults With Congenital Heart Disease: A Comparative Study Across Sex and Age","authors":"Cylia Houchi MSc , Marie-Joëlle Marcil MSc , Paul Khairy MD, PhD , Lena Rivard MD , Marie-Pierre Dubé PhD , Marie-Alexandre Chaix MD, PhD , François-Pierre Mongeon MD, SM , Annie Dore MD , Blandine Mondésert MD, FCHRS , Reda Ibrahim MD , Judith Brouillette MD, PhD","doi":"10.1016/j.cjcpc.2025.08.003","DOIUrl":"10.1016/j.cjcpc.2025.08.003","url":null,"abstract":"<div><h3>Background</h3><div>Mental health disorders are prevalent comorbidities in adults with congenital heart diseases (CHD). This study aimed to provide a biopsychosocial portrait of overall health in adults with CHD and compare by sex and age to a general population.</div></div><div><h3>Methods</h3><div>A cross-sectional study of 264 adults with CHD at the Montreal Heart Institute collected clinical data and self-reported lifestyle, sociodemographic, and psychological measures (the Hospital Anxiety and Depression Scale and the Worry and Anxiety Questionnaire). Descriptive and comparative statistics were used to compare with local population or stratified by age and sex.</div></div><div><h3>Results</h3><div>Anxiety scores were higher in females and younger adults with CHD, whereas depression scores show no significant differences. Although mean anxiety and depression scores in adults with CHD were similar to a Québec primary care population, subgroup comparisons show differences such as higher anxiety scores in younger adults with CHD. Fewer adults with CHD were sedentary or very active in comparison with controls, with more engaged in moderate activities. Moreover, they reported lower caffeine, alcohol, and cannabis consumption than the general population. Rates of smoking use or nonprescription medications were similar in both populations.</div></div><div><h3>Conclusions</h3><div>This study highlights key trends in the psychological and behavioral profiles of adults with CHD, including higher anxiety among young adults and differences in physical activity and alcohol consumption compared with the general population. These findings underline the importance of a holistic approach to management of this population.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 2","pages":"Pages 93-103"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147727129","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":"Partial Anomalous Pulmonary Venous Connection in Turner Syndrome: Small Patients, Big Implications","authors":"Ashish H. Shah MD, MD-Research","doi":"10.1016/j.cjcpc.2025.07.002","DOIUrl":"10.1016/j.cjcpc.2025.07.002","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 2","pages":"Page 80"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147727136","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":"Understanding the Biopsychosocial Landscape of Adults With Congenital Heart Disease","authors":"Seda Tierney MD","doi":"10.1016/j.cjcpc.2025.09.005","DOIUrl":"10.1016/j.cjcpc.2025.09.005","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 2","pages":"Pages 104-106"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147727130","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}