Laurence Watelle MD , Louis-Olivier Roy BSc , Jonathan Lauzon-Schnitka , Garrett Newell MD , Anne Dumas MD , Alexandre Nadeau BSc , Wei Ting Xiong MD , Kevin Rego , Camille Beaulieu , Emilie Groulx-Boivin , Marie-Ève Roy-Lacroix MD , Laurence Vaujois MD , Christian Drolet MD , Adrian Dancea MD , Nagib Dahdah MD , Jean-Luc Bigras MD , Frédéric Dallaire MD, PhD , Canadian Congenital and Pediatric Cardiology Research Network (CCPCRN) investigators
{"title":"The Quebec Congenital Heart Disease Registry: A Model of Prospective Databank to Facilitate Research in Congenital Cardiology","authors":"Laurence Watelle MD , Louis-Olivier Roy BSc , Jonathan Lauzon-Schnitka , Garrett Newell MD , Anne Dumas MD , Alexandre Nadeau BSc , Wei Ting Xiong MD , Kevin Rego , Camille Beaulieu , Emilie Groulx-Boivin , Marie-Ève Roy-Lacroix MD , Laurence Vaujois MD , Christian Drolet MD , Adrian Dancea MD , Nagib Dahdah MD , Jean-Luc Bigras MD , Frédéric Dallaire MD, PhD , Canadian Congenital and Pediatric Cardiology Research Network (CCPCRN) investigators","doi":"10.1016/j.cjcpc.2023.12.001","DOIUrl":"10.1016/j.cjcpc.2023.12.001","url":null,"abstract":"<div><h3>Background</h3><p>A national registry of congenital heart disease (CHD) would facilitate project initiation, decrease costs, increase statistical power, and avoid duplication. Establishing such registries poses numerous challenges, but the current Canadian research ecosystem in CHD is well positioned to meet them. We assessed the feasibility of building a province-wide CHD registry by automatically identifying people with CHD and extracting their native cardiac anatomy from multiple clinical data sources, without the need for manual data entry.</p></div><div><h3>Methods</h3><p>We designed a CHD registry of all fetuses and children with at least 1 echocardiographic report confirming CHD since 2000. We interfaced the registry with several clinical and echocardiography data sources from all paediatric cardiology programmes in Québec.</p></div><div><h3>Results</h3><p>We extracted 885,287 echocardiogram reports and 70,121 clinical records. We identified CHD in 43,452 children and 4682 fetuses. There were 1128 (2.3%) cases with files in multiple institutions, and patients with more complex CHD were 3 times more likely to be seen in more than 1 institution. So far, the registry has been used to build and link CHD cohorts for 7 distinct projects.</p></div><div><h3>Conclusions</h3><p>We demonstrated the feasibility of a baseline CHD registry in Québec without the need for manual data entry, in which other CHD research projects could be nested. This could serve as a blueprint to expand the registry and to develop an integrated approach where data gathered in caring for patients with CHD serve as data layers that incrementally contribute to a national cohort, for which data remain easily accessible and usable.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2772812923002002/pdfft?md5=9d71ff5140b663a1bec7627593e87167&pid=1-s2.0-S2772812923002002-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139022654","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}
Francesca de la Cruz MD , Carolina A. Escudero MD, MSc, FRCPC
{"title":"Prolonged QTc in the Setting of Eating Disorders","authors":"Francesca de la Cruz MD , Carolina A. Escudero MD, MSc, FRCPC","doi":"10.1016/j.cjcpc.2023.11.003","DOIUrl":"10.1016/j.cjcpc.2023.11.003","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2772812923001872/pdfft?md5=33d35f0d71e4cf6463374c6c0a113c63&pid=1-s2.0-S2772812923001872-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139303228","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":"Rethinking Paediatric Cardiology Training in Canada","authors":"Michael N. Gritti MD , Conall T. Morgan MD","doi":"10.1016/j.cjcpc.2023.11.002","DOIUrl":"10.1016/j.cjcpc.2023.11.002","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2772812923001860/pdfft?md5=8696cd414f5c8600b81a9ea860495207&pid=1-s2.0-S2772812923001860-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139295051","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":"Advances in Fetal Cardiac Imaging and Intervention","authors":"Nathaniel Barber MBBS, MSc, Lindsay Freud MD","doi":"10.1016/j.cjcpc.2023.10.012","DOIUrl":"10.1016/j.cjcpc.2023.10.012","url":null,"abstract":"<div><p>The field of fetal cardiology has evolved significantly in recent years. This review focuses on specific advances in fetal cardiac imaging and intervention that are increasingly used in clinical practice. On the imaging frontier, updated screening guidelines and artificial intelligence hold promise for improving prenatal detection of congenital heart disease. Advances in ultrasound technology and magnetic resonance imaging techniques have enabled greater diagnostic and prognostic accuracy of fetal heart disease from the first to third trimesters, and maternal hyperoxygenation can offer additional physiological insights. Fetal cardiac therapy has also seen great progress, with advances in transplacental pharmacologic treatments, infusions of enzyme replacement therapy, and fetal surgery for select rare and severe conditions.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S277281292300163X/pdfft?md5=bb53271556f8fec00b87a9f7412bd776&pid=1-s2.0-S277281292300163X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135455817","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":"Information for Readers","authors":"","doi":"10.1016/S2772-8129(24)00004-6","DOIUrl":"https://doi.org/10.1016/S2772-8129(24)00004-6","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2772812924000046/pdfft?md5=6eeba3589c50c128889dd76e1e1ce5a9&pid=1-s2.0-S2772812924000046-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139941623","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":"QTc Interval Prolongation and Its Association With Electrolyte Abnormalities and Psychotropic Drug Use Among Patients With Eating Disorders","authors":"Myles Benayon BSc, MD , Lekhini Latchupatula BHSc, MD , Emily Kacer BSc , Maaz Shanjer MD , Ethan Weiss BSc, MD , Shawnee Amar BMSc Candidate , Noah Zweig BSc Candidate , Mehran Ghadim BSc , Robert Portman BSc , Narayanaswamy Balakrishnan BSc, MSc, PhD , Hon Yiu So BSc, MPhil, PhD , Sheri Findlay MD, FRCPC , Natasha Johnson MD, FRCPC, FAAP , Tapas Mondal MD, MRCPCH, FRCPC","doi":"10.1016/j.cjcpc.2023.09.010","DOIUrl":"10.1016/j.cjcpc.2023.09.010","url":null,"abstract":"<div><h3>Background</h3><p>Eating disorders (EDs) often develop during adolescence with high mortality rates. Sudden cardiac death in these patients has been associated with corrected QT (QTc) interval prolongation. The significance of extrinsic factors on QTc prolongation in populations with EDs remains controversial. This study assessed the relationship between QTc prolongation in paediatric patients with EDs and extrinsic factors, such as QTc-prolonging medications and electrolyte abnormalities to investigate whether an ED alone is associated with an increased prevalence of QTc prolongation.</p></div><div><h3>Methods</h3><p>Electrocardiograms, electrolytes, and psychopharmaceutical usage were retrospectively analysed from the charts of 264 paediatric patients with EDs. Descriptive statistics were used to assess QTc prolongation and its relationship with electrolyte abnormalities and psychopharmaceuticals.</p></div><div><h3>Results</h3><p>Of 264 patients, 227 had normal QTc intervals (<440 ms), whereas 37 had borderline prolonged (440-460 ms) or prolonged (>460 ms) intervals. The prevalence of QTc intervals exceeding 440 ms in patients with normal electrolytes and not using QTc-prolonging psychotropics mirrored that of the general population (<em>P</em> = 0.59). Of the 23 patients taking psychotropics, 8 had abnormal QTc intervals. The average QTc was greater for patients using QTc-prolonging psychotropics (<em>P</em> = 0.05) with a correlation between interval length and psychotropic usage (<em>P</em> < 0.01). Average potassium (<em>P</em> = 0.08), calcium (<em>P</em> = 0.18), and magnesium (<em>P</em> = 0.08) levels did not significantly differ between those with normal and abnormal QTc intervals.</p></div><div><h3>Conclusions</h3><p>This study suggests that EDs alone may not prolong QTc intervals in paediatric patients with EDs, but psychotropics appear to be a salient external factor in QTc prolongation.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2772812923001367/pdfft?md5=c94ad84c5227cc196c23295e9686bdc1&pid=1-s2.0-S2772812923001367-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138625386","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}
Ismalia De Sousa PhD(c), MSc, RN , Vanessa Dizonno MSc , Karen LeComte MSN, RN , Alexis Carr MBBS, MSci, MHSc , Yussef El Kalza HBA , Mikayla Shymka BSc , Roger H. Payette BSc , Leah Kuzmuk MSc , Emily M.E. Lyall MD, CCFP , Jasmine Grewal MD , Jonathan M.C. Smith MD, MHSc , Thalia S. Field MD, FRCPC, MHSc
{"title":"“We have dealt with so much. There’s more coming?”: Improving Knowledge About Brain Health in Adults Living With Congenital Heart Disease","authors":"Ismalia De Sousa PhD(c), MSc, RN , Vanessa Dizonno MSc , Karen LeComte MSN, RN , Alexis Carr MBBS, MSci, MHSc , Yussef El Kalza HBA , Mikayla Shymka BSc , Roger H. Payette BSc , Leah Kuzmuk MSc , Emily M.E. Lyall MD, CCFP , Jasmine Grewal MD , Jonathan M.C. Smith MD, MHSc , Thalia S. Field MD, FRCPC, MHSc","doi":"10.1016/j.cjcpc.2023.10.010","DOIUrl":"10.1016/j.cjcpc.2023.10.010","url":null,"abstract":"<div><h3>Background</h3><p>Significant advances in managing congenital heart disease (CHD) have occurred over the past few decades, resulting in a fast-growing adult patient population with distinct needs requiring urgent attention. Research has recently highlighted the prevalence of neurocognitive differences among adults living with CHD. Yet, there is a lack of knowledge about the perspectives of people living with CHD and family members/caregivers on brain health. We sought to explore their perspectives to guide future research and clinical endeavours.</p></div><div><h3>Methods</h3><p>Using the principles of integrated knowledge translation and qualitative interpretive description, we conducted 2 focus groups with 7 individuals with CHD and their family members as part of a virtual forum on brain health in CHD. Data analysis followed the principles of interpretive description.</p></div><div><h3>Results</h3><p>A lack of understanding about overall brain health and neurocognitive differences in adult CHD was identified. To increase overall knowledge about brain health, initiatives should (1) focus on the individual living with CHD, involving family members and peers; (2) use social media and health care encounters for knowledge exchange; and (3) ensure a “balancing act” in the information provided to avoid feelings of worry and uncertainty about the future while simultaneously empowering people living with CHD.</p></div><div><h3>Conclusions</h3><p>There is a pressing need for better education about brain health among individuals living with CHD. Our findings can guide clinicians in developing programmes of care and (re)design health services that address the brain-heart axis and neurocognitive differences in CHD.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2772812923001616/pdfft?md5=08471eabbea01c56b163fbc0b226843b&pid=1-s2.0-S2772812923001616-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136128237","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}
Ryaan EL-Andari MD , Rami Zibdawi BSc, MSc , Paula Holinski MD , John Koller MD , Chloe Joynt MD , Nee Khoo MBChB , Laurance Lequier MD , Hamdy El-Hakim MD , Mohammed Al Aklabi FRCSC, MD
{"title":"Congenital Tracheal Stenosis Patients Undergoing Modified Slide Tracheoplasty: Single-Centre Technique and Long-Term Morbidity and Mortality","authors":"Ryaan EL-Andari MD , Rami Zibdawi BSc, MSc , Paula Holinski MD , John Koller MD , Chloe Joynt MD , Nee Khoo MBChB , Laurance Lequier MD , Hamdy El-Hakim MD , Mohammed Al Aklabi FRCSC, MD","doi":"10.1016/j.cjcpc.2023.10.003","DOIUrl":"10.1016/j.cjcpc.2023.10.003","url":null,"abstract":"<div><h3>Background</h3><p>Congenital malformations of the trachea are rare but often life-threatening. Limited data have been published on the outcomes of tracheal reconstruction for congenital tracheal stenosis. We sought to describe the outcomes of patients undergoing tracheal reconstruction over 10 years at our centre.</p></div><div><h3>Methods</h3><p>All paediatric patients who underwent long-segment tracheal or bronchial reconstruction from January 1, 2012, to August 31, 2022, were included. The primary outcome was mortality, and secondary outcomes included reoperation and postoperative morbidity. Patients were followed up to 10 years.</p></div><div><h3>Results</h3><p>Thirty-three patients with ages ranging from 1 day to 12 years (mean 8.5 months) at the time of tracheoplasty or bronchoplasty were included, with 5 patients undergoing off-pump tracheal reconstruction. The most common preoperative comorbidities included patent ductus arteriosus (30.3%), atrial septal defect (27.3%), and prematurity (24.2%). There were no deaths postoperatively within the follow-up period. All patients experienced successful reconstruction with no patients requiring reoperation of the trachea. A total of 14 patients (42.4%) required postoperative balloon dilation, 3 (9.1%) required bronchial repair after tracheal repair, and 2 (6.1%) required bronchoscopic tracheal debridement.</p></div><div><h3>Conclusions</h3><p>This single-centre retrospective study provides a large cohort of congenital tracheal reconstruction patients with a survival rate of 100%, experiencing no mortality during follow-up. The majority of patients had preoperative comorbidities and concomitant congenital cardiac defects. Although tracheal reconstruction continues to be complex with significant postoperative morbidity and mortality, the results of our single-centre study demonstrate the continual advancement of this field and the evolving improvement of postoperative outcomes for these patients.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2772812923001549/pdfft?md5=372002f31b6b9ab6d7e4a17cde9d24d0&pid=1-s2.0-S2772812923001549-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135760555","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}