CJC Pediatric and Congenital Heart Disease最新文献

{"title":"Advanced Imaging Technologies for Assessing Tetralogy of Fallot: Insights Into Flow Dynamics","authors":"Michal Schäfer MD, PhD ,&nbsp;Wadi Mawad MD","doi":"10.1016/j.cjcpc.2023.09.011","DOIUrl":"10.1016/j.cjcpc.2023.09.011","url":null,"abstract":"<div><p>Tetralogy of Fallot is the most common cyanotic congenital heart defect requiring surgical repair. Although surgical interventions have significantly reduced mortality, postrepair complications, such as pulmonary valve regurgitation and stenosis, may lead to adverse outcomes, including right ventricular dysfunction and increased risks of morbidity and mortality. This review explores the potential of advanced imaging technologies, including 4-dimensional–flow magnetic resonance imaging and high-frame-rate echocardiography, in providing valuable insights into blood flow dynamics and energy parameters. Quantitative measures, such as energy loss and vorticity, along with qualitative flow analysis, can provide additional insights into adverse haemodynamics at a potentially earlier and more reversible stage. Furthermore, personalized patient-specific information from these imaging modalities aids in guiding treatment decisions and monitoring postoperative interventions effectively. By characterizing flow patterns, these advanced imaging techniques hold great promise in improving the assessment and management of tetralogy of Fallot, providing tailored insights. However, further research and longitudinal studies are required to fully establish their clinical utility and potential impact on patient care.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"2 6","pages":"Pages 380-392"},"PeriodicalIF":0.0,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2772812923001379/pdfft?md5=3bfc0e3db3eb18d6801a6d1561e76a33&pid=1-s2.0-S2772812923001379-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134936403","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(23)00167-7","DOIUrl":"https://doi.org/10.1016/S2772-8129(23)00167-7","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"2 6","pages":"Page A1"},"PeriodicalIF":0.0,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2772812923001677/pdfft?md5=b94289389c5e762abf9a6a0f9cdbb166&pid=1-s2.0-S2772812923001677-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138501640","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":"Administrative Databases: Friend or Foe in Paediatric Cardiomyopathy","authors":"Jennifer Conway MD, MSc ,&nbsp;Olesya Barrett PhD ,&nbsp;Tara Pidborochynski MSc ,&nbsp;Katie Schroeder MN ,&nbsp;Chentel Cunningham MN, NP ,&nbsp;Aamir Jeewa MD ,&nbsp;Padma Kaul PhD","doi":"10.1016/j.cjcpc.2023.09.009","DOIUrl":"10.1016/j.cjcpc.2023.09.009","url":null,"abstract":"<div><h3>Background</h3><p>Cardiomyopathy (CM) is a rare childhood disease associated with morbidity and mortality. Limited data exist on paediatric CM in Canada. Given the rare nature, single-centre studies are not sufficiently powered to address important questions. Therefore, administrative health data may serve as a resource for the study of childhood CM. The goal of this study was to validate the accuracy of International Classification of Diseases (ICD)-based algorithms to identify paediatric CM in health databases using a clinical registry as the gold standard.</p></div><div><h3>Methods</h3><p>The clinical registry was compiled from outpatient and inpatient records at the Stollery Children’s Hospital (January 1, 2013, to December 31, 2021). Patients were categorized as having CM or screened without CM. Data were linked to administrative health databases using the patient’s Unique Lifetime Identifier. Algorithms based on the presence of ICD, 10th Revision, codes for CM were then evaluated, and cross-tabulations against the clinical registry were generated. Accuracy, positive predictive value, negative predictive value, sensitivity, and specificity were calculated.</p></div><div><h3>Results</h3><p>The clinical registry had 90 patients with CM and 249 screened without CM. The algorithms ruled out CM (high negative predictive value) but had variability in the ability to diagnose CM positive predictive value. The algorithm that performed the best was based on a diagnosis of CM in a hospitalization or 2 ambulatory visits.</p></div><div><h3>Conclusions</h3><p>A combination of inpatient and outpatient databases can be used, with acceptable accuracy, to identify paediatric patients with CM. This finding allows for the use of the identified algorithm for the comprehensive study of paediatric CM in Canada.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"2 6","pages":"Pages 490-493"},"PeriodicalIF":0.0,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2772812923001355/pdfft?md5=be23751e35560cdbcf1a06505201e258&pid=1-s2.0-S2772812923001355-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135406730","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":"Uncovering the Genetic Basis of Congenital Heart Disease: Recent Advancements and Implications for Clinical Management","authors":"Karanjot Chhatwal BSc ,&nbsp;Jacob J. Smith BSc ,&nbsp;Harroop Bola BSc ,&nbsp;Abeer Zahid BSc ,&nbsp;Ashwin Venkatakrishnan BSc ,&nbsp;Thomas Brand PhD","doi":"10.1016/j.cjcpc.2023.10.008","DOIUrl":"10.1016/j.cjcpc.2023.10.008","url":null,"abstract":"<div><p>Congenital heart disease (CHD) is the most prevalent hereditary disorder, affecting approximately 1% of all live births. A reduction in morbidity and mortality has been achieved with advancements in surgical intervention, yet challenges in managing complications, extracardiac abnormalities, and comorbidities still exist. To address these, a more comprehensive understanding of the genetic basis underlying CHD is required to establish how certain variants are associated with the clinical outcomes. This will enable clinicians to provide personalized treatments by predicting the risk and prognosis, which might improve the therapeutic results and the patient’s quality of life. We review how advancements in genome sequencing are changing our understanding of the genetic basis of CHD, discuss experimental approaches to determine the significance of novel variants, and identify barriers to use this knowledge in the clinics. Next-generation sequencing technologies are unravelling the role of oligogenic inheritance, epigenetic modification, genetic mosaicism, and noncoding variants in controlling the expression of candidate CHD-associated genes. However, clinical risk prediction based on these factors remains challenging. Therefore, studies involving human-induced pluripotent stem cells and single-cell sequencing help create preclinical frameworks for determining the significance of novel genetic variants. Clinicians should be aware of the benefits and implications of the responsible use of genomics. To facilitate and accelerate the clinical integration of these novel technologies, clinicians should actively engage in the latest scientific and technical developments to provide better, more personalized management plans for patients.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"2 6","pages":"Pages 464-480"},"PeriodicalIF":0.0,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2772812923001598/pdfft?md5=6074644a991a2fc73be42646b2565526&pid=1-s2.0-S2772812923001598-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135963647","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":"The Impact of the COVID-19 Pandemic Restrictions on the Provision of Adult Congenital Heart Disease Care Across Canada: A National Survey","authors":"Ruochen Tony Mao MD ,&nbsp;Luc Beauchesne MD, FRCPC ,&nbsp;Ariane Marelli MD, FRCPC ,&nbsp;Candice Silversides MD, FRCPC ,&nbsp;Annie Dore MD, FRCPC ,&nbsp;Javier Ganame MD, PhD ,&nbsp;Michelle Keir MD, MSc, FRCPC ,&nbsp;Rafael Alonso-Gonzalez MD, MSc ,&nbsp;Isabelle Vonder Muhll BSc, MD, FRCPC ,&nbsp;Jasmine Grewal MD, FRCPC ,&nbsp;Anne Williams MD, FRCPC, FACC ,&nbsp;Payam Dehghani MD, FRCPC ,&nbsp;Samuel Siu MD, SM, MBA, FRCPC ,&nbsp;Amer Johri MD, FRCPC ,&nbsp;Elisabeth Bedard MD, FRCPC ,&nbsp;Judith Therrien MD, FRCPC ,&nbsp;Doug Hayami MDCM, FRCPC ,&nbsp;Catherine Kells MD, FRCPC, FACC ,&nbsp;Robin A. Ducas BSc, MD, FRCPC","doi":"10.1016/j.cjcpc.2023.09.002","DOIUrl":"https://doi.org/10.1016/j.cjcpc.2023.09.002","url":null,"abstract":"<div><h3>Background</h3><p>The COVID-19 pandemic significantly impacted health care access across Canada with the reduction in in-person evaluations. The aim of the study was to examine the effects of the COVID-19 pandemic on access to health care services among the Canadian population with adult congenital heart disease (ACHD).</p></div><div><h3>Methods</h3><p>All Canadian adult congenital heart affiliated centres were contacted and asked to collect data on outpatient clinic and procedural volumes for the 2019 and 2020 calendar years. A survey was sent detailing questions on clinic and procedural volumes and wait times before and after pandemic restrictions. Descriptive statistics were used with the Student <em>t</em>-test to compare groups.</p></div><div><h3>Results</h3><p>In 2019, there were 19,326 ACHD clinic visits across Canada and only 296 (1.5%) virtual clinic visits. However, during the first year of the pandemic, there were 20,532 clinic visits and 11,412 (56%) virtual visits (<em>P</em> &lt; 0.0001). There were no differences in procedural volumes (electrophysiology, cardiac surgery, and percutaneous intervention) between 2019 and 2020. The mean estimated wait times (months) before the pandemic vs the pandemic were as follows: nonurgent consult 5.4 ± 2.6 vs 6.6 ± 4.2 (<em>P</em> = 0.65), ACHD surgery 6.0 ± 3.5 vs 7.0 ± 4.6 (<em>P</em> = 0.47), electrophysiology procedures 6.3 ± 3.3 vs 5.7 ± 3.3 (<em>P</em> = 0.72), and percutaneous intervention 4.6 ± 3.9 vs 4.4 ± 2.3 (<em>P</em> = 0.74).</p></div><div><h3>Conclusions</h3><p>During the pandemic and restrictions of social distancing, the use of virtual clinic visits helped to maintain continuity in ACHD clinical care, with 56% of ACHD visits being virtual. The procedural volumes and wait times for consultation and percutaneous and surgical interventions were not delayed.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"2 5","pages":"Pages 247-252"},"PeriodicalIF":0.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67739332","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":"Multistakeholder Recommendations for Supporting Patients and Families Transitioning From Paediatric to Adult Congenital Heart Disease Care","authors":"Emily K. Hyde RN, MN ,&nbsp;Annette S.H. Schultz RN, PhD ,&nbsp;Robin Ducas MD, FRCPC ,&nbsp;Reeni Soni MD, FRCPC ,&nbsp;Holly Bekkering RN, MN ,&nbsp;Dawn Barker RN ,&nbsp;Andrea Klippenstein PhD(c) ,&nbsp;Mudra G. Dave BKin ,&nbsp;Chloe Frechette ,&nbsp;Joanne St. Goddard-Frechette ,&nbsp;Lori Lester ,&nbsp;Shelly Mclarty ,&nbsp;Anna M. Chudyk MSc, PhD","doi":"10.1016/j.cjcpc.2023.08.001","DOIUrl":"https://doi.org/10.1016/j.cjcpc.2023.08.001","url":null,"abstract":"<div><h3>Background</h3><p>Transitioning from paediatric to adult congenital heart disease (CHD) care is a high-risk time for being lost to follow-up. Existing CHD transition programmes have not included patients, caregivers, and health care providers as partners in their development. This study aimed to develop recommendations for a CHD transition programme driven by lived and clinical experiences.</p></div><div><h3>Methods</h3><p>We used a multilevel participatory process that engaged adult and paediatric people living with CHD, their caregivers, and CHD health care providers as members of the research team. We also consulted members of these stakeholder groups through a series of 3 virtual workshops that culminated in the generation of recommendations for the essential components of a CHD transition programme.</p></div><div><h3>Results</h3><p>The Transition Essentials recommendations inform what information, education, or support is required, who should provide it, and when and how it should be provided. Information, education, and support for self-management and knowledge are required for people living with CHD. Caregivers require information, education, and support to build capacity in people living with CHD and navigate their new role in their loved ones’ life. The health care team should provide this information, education, and support with peer support options when people living with CHD are 15-22 years of age. This information, education, and support should be individualized, navigate limitations, build over time, have multimodal options, and be available virtually or in person.</p></div><div><h3>Conclusions</h3><p>Engaging those with lived and clinical expertise to develop recommendations for the essential components of a CHD transition programme provides important insights missing from previous studies.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"2 5","pages":"Pages 225-236"},"PeriodicalIF":0.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67739348","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(23)00140-9","DOIUrl":"https://doi.org/10.1016/S2772-8129(23)00140-9","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"2 5","pages":"Page A1"},"PeriodicalIF":0.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67739352","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":"Home-Based Resistance Training Programme in Fontan Patients: It Is Feasible, Safe, and Efficient","authors":"Joanie Gagnon MD candidate, PHT ,&nbsp;Mickaël Martin MSc ,&nbsp;Louise Gosselin RN ,&nbsp;Philippe Chetaille MD, MSc","doi":"10.1016/j.cjcpc.2023.08.003","DOIUrl":"https://doi.org/10.1016/j.cjcpc.2023.08.003","url":null,"abstract":"<div><h3>Background</h3><p>Fontan physiology is characterized by a single functional systemic ventricle that reduces cardiac output adaptation and exercise capacity. Peripheral muscle pump is crucial for venous return, and resistance training of the peripheral muscles may be particularly beneficial for cardiac function in these patients. This study explores the feasibility of a training programme including safety and effectiveness, maximal exercise capacity, cardiorespiratory endurance, and quality of life in a Fontan population.</p></div><div><h3>Methods</h3><p>Twelve Fontan patients (18-32 years old) with New York Heart Association functional classes I and II were recruited. The intervention was 12 weeks of home-based exercise programme of 3 sessions per week supervised online by a physiotherapist. Pre- and postintervention evaluations assessed lean body mass, segmental forces with a hand-held dynamometer, maximal oxygen consumption (VO₂max), cardiovascular endurance, leg endurance with a 1-minute-sit-to-stand test, and quality of life with a 36-item Short-Form Survey.</p></div><div><h3>Results</h3><p>A total of 11 of 12 participants completed 35 of 36 workouts, exceeding our 80% threshold for completion. No adverse events were reported. Leg muscle strength increased significantly (knee extensors improved by 22.8 ± 22.8 N m [17.7%], <em>P</em> = 0.019). Despite no change in VO₂max, endurance time showed a significant improvement of 5.3 minutes (72%). Perceived physical condition evaluated with a self-perception scale improved significantly. There was no significant difference in the lean body mass and in quality of life.</p></div><div><h3>Conclusion</h3><p>In this small group of post-Fontan patients with mild functional impairments, we describe a structured, accessible, and feasible home-based resistance training programme that seems safe and show improvements in strength and perceived physical condition.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"2 5","pages":"Pages 240-246"},"PeriodicalIF":0.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67739333","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":"Successful Use of Prostaglandin Infusion in a Two-Month-Old With Coarctation of the Aorta and Ventricular Dysfunction","authors":"Stephanie Hosang MD ,&nbsp;Luis Altamirano-Diaz MD","doi":"10.1016/j.cjcpc.2023.05.005","DOIUrl":"https://doi.org/10.1016/j.cjcpc.2023.05.005","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"2 5","pages":"Pages 222-224"},"PeriodicalIF":0.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67739349","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":"Cascade Screening in Familial Hypercholesterolemia: Achieving Buy-In and Turning Patients Into Partners","authors":"Michael Khoury MD, FRCPC","doi":"10.1016/j.cjcpc.2023.06.002","DOIUrl":"https://doi.org/10.1016/j.cjcpc.2023.06.002","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"2 5","pages":"Pages 219-221"},"PeriodicalIF":0.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67739350","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}