Nathan C. Denham BM, PhD , Robert D. Anderson MBBS, PhD , Raja Selvaraj MBBS, MD, DM , Andrew C.T. Ha MD , Krishnakumar Nair MD
{"title":"Use of the Local Activation Time Histogram “Trough” to Identify the Slow Conduction Channel in Complex Congenital Heart Disease Macro-re-entrant Arrhythmias","authors":"Nathan C. Denham BM, PhD , Robert D. Anderson MBBS, PhD , Raja Selvaraj MBBS, MD, DM , Andrew C.T. Ha MD , Krishnakumar Nair MD","doi":"10.1016/j.cjcpc.2023.12.004","DOIUrl":"10.1016/j.cjcpc.2023.12.004","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"3 2","pages":"Pages 79-82"},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2772812924000010/pdfft?md5=f563297a8567061816d883c563ce7d41&pid=1-s2.0-S2772812924000010-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139637664","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}
Mohit Singla MD, MBA , Pascal N. Tyrrell PhD , Manveer Khural MD , Gil J. Gross MD
{"title":"Increased Prevalence of the Electrocardiographic Early Repolarization Pattern in Young Patients With Vagally Mediated Syncope: A Case-Control Study","authors":"Mohit Singla MD, MBA , Pascal N. Tyrrell PhD , Manveer Khural MD , Gil J. Gross MD","doi":"10.1016/j.cjcpc.2023.10.013","DOIUrl":"10.1016/j.cjcpc.2023.10.013","url":null,"abstract":"<div><h3>Background</h3><p>Electrocardiographic early repolarization (EER) is linked with idiopathic ventricular fibrillation in adults. It is frequently seen in children, with poorly understood significance. Some evidence suggests that it could be a vagally mediated phenomenon. A retrospective case-control study was undertaken to test the hypothesis that EER is more common among children with typical vasovagal syncope (VVS) than among their peers with nonvagal syncope (NVS) or with no syncope.</p></div><div><h3>Methods</h3><p>Patients aged 4-18 years with syncope were identified by a single-centre database search followed by a review of history for features of VVS (n = 150) or NVS (n = 84). The first available electrocardiogram (ECG) for VVS or for NVS was retrieved. Age- and sex-matched children with no known syncope or heart disease were then identified (n = 216). ECGs were assessed separately for EER based on published criteria by 2 observers blinded to patients’ clinical status.</p></div><div><h3>Results</h3><p>Mean age was 12.3 ± 3.2 years, and heart rate was 74.2 ± 16.5 beats/min. EER was more prevalent in VVS (33.3%) than among patients with NVS (19.1%; odds ratio: 2.29; confidence interval: 1.32-5.50) or among those with no syncope (12.5%; odds ratio: 3.14; confidence interval: 1.81-5.46). Heart rates were significantly lower in VVS and NVS (heart rate: 70.1 ± 13.8 and 70.7 ± 12.4 beats/min, respectively) compared with children with no syncope (heart rate: 78.2 ± 18.0 beats/min), both <em>P</em> < 0.001.</p></div><div><h3>Conclusions</h3><p>EER is more common in paediatric patients with VVS than those with NVS or without syncope, consistent with a possible vagal contribution to the ECG finding.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"3 2","pages":"Pages 74-78"},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2772812923001653/pdfft?md5=890aa631d4ed1af635c0b5204d972a8f&pid=1-s2.0-S2772812923001653-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135615598","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 , Alison J. Howell MD , Pablo Perez Lopez MD , Wei Hui MD , Luc L. Mertens MD, PhD , Vitor C. Guerra MD, PhD
{"title":"Impact of Genotype-Phenotype Interactions on Cardiovascular Function in Paediatric Loeys-Dietz Syndrome","authors":"Nairy Khodabakhshian PhD , Alison J. Howell MD , Pablo Perez Lopez MD , Wei Hui MD , Luc L. Mertens MD, PhD , Vitor C. Guerra MD, PhD","doi":"10.1016/j.cjcpc.2023.12.003","DOIUrl":"https://doi.org/10.1016/j.cjcpc.2023.12.003","url":null,"abstract":"<div><h3>Background</h3><p>The relationship between genotype and phenotypical vascular and cardiac properties in paediatric Loeys-Dietz syndrome (LDS) patients are not well characterized. This study explores the phenotypical differences in aortic properties and cardiac structural and functional parameters between paediatric LDS patients with <em>TGFBR1</em> and <em>TGFBR2</em> mutations.</p></div><div><h3>Methods</h3><p>We included 32 LDS patients with either <em>TGFBR1</em> (n = 17) or <em>TGFBR2</em> (n = 15) mutations. Echocardiographic data included aortic dimensions, distensibility, strain, and stiffness at the level of the annulus, sinuses of Valsalva, sinotubular junction, ascending aorta, and descending aorta. Parameters for left ventricular size and function were also recorded.</p></div><div><h3>Results</h3><p>Demographics were similar between the groups. Patients with <em>TGFBR2</em> were more likely to have undergone aortic surgery (47% vs 12%, <em>P</em> = 0.057) and use angiotensin receptor blockers (93% vs 47%, <em>P</em> = 0.015). Aortic <em>z</em> scores were significantly larger in the <em>TGFBR2</em> group at the level of the aortic valve annulus (<em>P</em> = 0.007), sinuses of Valsalva (<em>P</em> = 0.001), sinotubular junction (<em>P</em> = 0.001), and ascending aorta (<em>P</em> = 0.054). Patients with <em>TGFBR2</em> also had significantly lower aortic distensibility and strain coupled with higher stiffness index at the level of the annulus, sinotubular junction, and ascending aorta. Parameters for the descending aorta, cardiac morphology, and cardiac function were similar between the groups.</p></div><div><h3>Conclusions</h3><p>Paediatric LDS patients with <em>TGFBR2</em> present with more severe cardiovascular phenotypes than patients with <em>TGFBR1</em> with larger aortic dimensions and increased aortic stiffness. Our findings suggest that genotypes should be taken into consideration in the clinical management of paediatric LDS patients.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"3 2","pages":"Pages 47-54"},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2772812923002026/pdfft?md5=3651d91d1771023d5bed1cde008ad648&pid=1-s2.0-S2772812923002026-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140645881","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":"Tetralogy of Fallot in Low- and Middle-Income Countries","authors":"Nabil Dib MD , Vincent Chauvette MD, PhD(c) , Momar Sokhna Diop MD , Ismail Bouhout MD , Mehdi Hadid MD , Christophe Vô MD , Paul Khairy MD, PhD , Nancy Poirier MD","doi":"10.1016/j.cjcpc.2023.12.002","DOIUrl":"10.1016/j.cjcpc.2023.12.002","url":null,"abstract":"<div><p>Low- and middle-incomes countries (LMICs) have limited resources for the diagnosis and treatment of congenital heart diseases such as tetralogy of Fallot. This is in part due to lack of infrastructures, financial means, and expertise. As a result, patients undergo surgery much later than in high-income countries. This delay in treatment results in right ventricular dysfunction, cardiac arrhythmias, and poor psychomotor development—complications that are all related to chronic hypoxia. There are limited data and a few small studies of patients treated for tetralogy of Fallot in LMICs, and, therefore, the aim of this review is to analyse and summarize the surgical outcomes of this LMIC population.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"3 2","pages":"Pages 67-73"},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2772812923002014/pdfft?md5=c5488d524219a63258cc64d51aef73e7&pid=1-s2.0-S2772812923002014-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139017103","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}
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":"3 2","pages":"Pages 57-66"},"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":"3 1","pages":"Pages 22-23"},"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}