Jessica D. Jenkins BN, MN, NP , Kayla Poku BSc , Connor Hass , Michelle A.L. Kotelko BEd, BA , David Campbell MD, BSc , Michelle Keir MD, MSc
{"title":"Advance Care Planning for Adults With Moderate-to-Severe Congenital Heart Disease: Readiness Trial","authors":"Jessica D. Jenkins BN, MN, NP , Kayla Poku BSc , Connor Hass , Michelle A.L. Kotelko BEd, BA , David Campbell MD, BSc , Michelle Keir MD, MSc","doi":"10.1016/j.cjcpc.2024.09.001","DOIUrl":"10.1016/j.cjcpc.2024.09.001","url":null,"abstract":"<div><h3>Background</h3><div>Adults with congenital heart disease (CHD) are growing in number and living longer with complex lesions; however, many are at risk of death in midlife. Conversations about advance care planning (ACP) in this population have been found to be infrequent and not part of regular outpatient care. The intent of this study was to explore readiness of patients with adult CHD (ACHD) to discuss ACP and assess the impact of interventions to support ACP conversations.</div></div><div><h3>Methods</h3><div>We conducted a prospective, randomized, single-centre trial of adults with moderate to severe CHD. Simply, the control group was asked if they were ready to discuss their wishes if they were to become seriously ill. The intervention group was provided 2 additional resources with the readiness question, the modified Lyon Family-Centered ACP survey and a patient-partner created ACHD-ACP video.</div></div><div><h3>Results</h3><div>A total of 111 responses (control [n = 59] and intervention group [n = 52]) were collected for analysis. Women represented 59.0% of the participants. The mean age was 39.6 years (standard deviation = 14.8 years). No significant difference was found between the control and experimental groups’ readiness (94.9% and 90.4%, respectively). Most participants (92.8%) responded positively towards initiating conversations related to end-of-life and ACP discussions.</div></div><div><h3>Conclusions</h3><div>We found that adults with CHD are ready to have ACP conversations as part of their outpatient care. Patient preferences and values should guide ACP conversations; further research is needed to determine whether the modified Lyon Family-Centered ACP survey and ACHD-ACP video are helpful adjuncts for ACP in outpatient clinics.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"3 6","pages":"Pages 256-264"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143176084","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}
Adriana H. Tremoulet MD, MAS , Nagib Dahdah MD, MBA, FRCPC
{"title":"Fostering Global Collaboration Around Kawasaki Disease. Reflections From the 14th International Kawasaki Disease Symposium","authors":"Adriana H. Tremoulet MD, MAS , Nagib Dahdah MD, MBA, FRCPC","doi":"10.1016/j.cjcpc.2024.10.004","DOIUrl":"10.1016/j.cjcpc.2024.10.004","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"3 6","pages":"Pages 237-240"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143176081","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}
Henri Juhani Pyykkönen MSc , Otto Rahkonen MD, PhD , Heikki Tikkanen MD, PhD , Karim Khanji MSc , Päivi Piirilä MD, PhD , Olli Pitkänen-Argillander MD, PhD
{"title":"Retrograde Flow to Aortic Root Predicts Inferior Cardiopulmonary Performance and Restrictive Lung Physiology in Fontan Circulation","authors":"Henri Juhani Pyykkönen MSc , Otto Rahkonen MD, PhD , Heikki Tikkanen MD, PhD , Karim Khanji MSc , Päivi Piirilä MD, PhD , Olli Pitkänen-Argillander MD, PhD","doi":"10.1016/j.cjcpc.2024.08.002","DOIUrl":"10.1016/j.cjcpc.2024.08.002","url":null,"abstract":"<div><h3>Background</h3><div>Cardiac output in Fontan circulation depends on systemic venous pressure, pulmonary vascular resistance, and ventricular function. Because myocardial function is dependent on coronary perfusion, we studied whether retrograde flow to aortic root in the mitral/aortic atresia subgroup of hypoplastic left heart syndrome (HLHS) affects cardiopulmonary performance.</div></div><div><h3>Methods</h3><div>We studied 26 stable Fontan patients (14.4 ± 2.4 years) with right (RV, n = 17) and left (LV, n = 9) systemic ventricle morphology. All RV patients had HLHS and were subdivided according to postnatal flow to the hypoplastic ascending aorta being antegrade (HLHS-A) or retrograde (HLHS-R) due to valve atresia. Physical activity was assessed by questionnaire (LASERI, a questionnaire for Finnish children regarding physical activity), cardiopulmonary exercise test (1-minute ramp protocol), body composition (Biacorpus RX 4000), and muscle fitness (EUROFIT). These data were correlated with the postnatal aorta size and current branch pulmonary artery size index (McGoon index).</div></div><div><h3>Results</h3><div>Patients with HLHS-R seldom self-reported engagement in vigorous physical activity and had significantly lower cardiopulmonary performance (peak oxygen uptake [VO<sub>2</sub>peak]) than patients with LV morphology (<em>P</em> = 0.037), but not compared with patients with HLHS-A. Branch pulmonary artery size did not correlate with VO<sub>2</sub>peak. Patients with HLHS-R had most severe lung restrictions (forced vital capacity <em>z</em>-score –3 ± 0.9, <em>P</em> = 0.0073; forced expiratory volume in 1 second <em>z</em>-score –3.3 ± 1.1, <em>P</em> = 0.001).</div></div><div><h3>Conclusions</h3><div>Young Fontan patients with LV had better cardiopulmonary performance than patients with HLHS. Patients with HLHS-R were the least active and had the lowest VO<sub>2</sub>peak and most restrictive lungs. It is important to recognize postnatally single ventricle patients at high risk for inactivity to promote an active and healthy lifestyle.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"3 6","pages":"Pages 265-271"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143176085","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}
Matthew D. Elias MD , Federica Anselmi MD , Luisa B. Gámez-González MD , Fujito Numano MD, PhD , Rakesh Kumar Pilania MD, DM , Alan P. Wang MD , Nagib Dahdah MD, MBA , Adriana H. Tremoulet MD, MAS , Audrey Dionne MD
{"title":"Fourteenth International Kawasaki Disease Symposium: Learning From the Past, Looking to the Future","authors":"Matthew D. Elias MD , Federica Anselmi MD , Luisa B. Gámez-González MD , Fujito Numano MD, PhD , Rakesh Kumar Pilania MD, DM , Alan P. Wang MD , Nagib Dahdah MD, MBA , Adriana H. Tremoulet MD, MAS , Audrey Dionne MD","doi":"10.1016/j.cjcpc.2024.10.002","DOIUrl":"10.1016/j.cjcpc.2024.10.002","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"3 6","pages":"Pages 229-236"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143176082","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}
Alexander C. Egbe MD, MPH, MS, Malini Madhavan MBBS, Heidi M. Connolly MD, Ahmed E. Ali MD, Ahmed Younis MD, Abhishek Deshmukh MBBS
{"title":"Incidence, Correlates, and Prognostic Implications of New-Onset Atrial Fibrillation in Adults With Repaired Coarctation of Aorta","authors":"Alexander C. Egbe MD, MPH, MS, Malini Madhavan MBBS, Heidi M. Connolly MD, Ahmed E. Ali MD, Ahmed Younis MD, Abhishek Deshmukh MBBS","doi":"10.1016/j.cjcpc.2024.07.004","DOIUrl":"10.1016/j.cjcpc.2024.07.004","url":null,"abstract":"<div><h3>Background</h3><div>There are limited data about the incidence and outcomes of atrial fibrillation (AF) in adults with coarctation of aorta (COA). The purpose of this study was to determine the incidence, correlates, and prognostic implications of new-onset AF in adults with repaired COA.</div></div><div><h3>Methods</h3><div>A retrospective cohort study of adults with repaired COA without a prior history of atrial arrhythmias was performed. We reviewed rhythm data (electrocardiogram, Holter, and rhythm strip) obtained from baseline to the last clinical encounter. The correlates of AF and the relationship between AF and cardiovascular adverse events (heart failure hospitalization and/or all-cause mortality) were assessed using Cox regression.</div></div><div><h3>Results</h3><div>Of 782 patients (aged 32 [interquartile range: 21-43] years; 462 [59%] men), 42 (5.4%) developed new-onset AF. The incidence of new-onset AF was 9 per 1000 patient-years (0.9% per year), and the median age at onset of AF was 36 (interquartile range: 24-49) years. The correlates of new-onset AF were older age, hypertension, left atrial dysfunction, and left ventricular hypertrophy. Of 782 patients, 92 (12%) had cardiovascular adverse events. On multivariable analysis, new-onset AF was associated with cardiovascular adverse events (hazard ratio: 1.09, 95% confidence interval: 1.03-1.15), after adjustment for age, hypertension, and right and left ventricular structure and function.</div></div><div><h3>Conclusions</h3><div>Patients with COA were at risk for developing AF at a relatively young age (median age: 36 years), and AF was associated with cardiovascular adverse outcomes. There is a need to target the modifiable risk factors for AF to reduce the adverse outcomes associated with AF.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"3 6","pages":"Pages 247-252"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143176079","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}
Michael N. Gritti MD , Megan Werger MD (c) , Alison J. Howell MD , Conall T. Morgan MD
{"title":"Gender Distribution in Paediatric Cardiology Training Programs in Canada","authors":"Michael N. Gritti MD , Megan Werger MD (c) , Alison J. Howell MD , Conall T. Morgan MD","doi":"10.1016/j.cjcpc.2024.09.002","DOIUrl":"10.1016/j.cjcpc.2024.09.002","url":null,"abstract":"<div><h3>Background</h3><div>Despite female medical students being the majority, there are certain medical specialities that continue to have a prevalence of male trainees, such as adult cardiology. The purpose of this study is to examine gender distribution within Canadian paediatric cardiology training programs.</div></div><div><h3>Methods</h3><div>Both application and successful matches to core paediatric residency programs and paediatric cardiology programs were obtained through the Canadian Resident Matching Service. Analysis was performed to determine if the association between gender of paediatric residents and paediatric cardiology applicants/fellows was significant.</div></div><div><h3>Results</h3><div>Between 2016 and 2024, 12% (n = 3/26) of individuals who successfully matched into a Canadian paediatric cardiology training program identified as female and 88% (n = 23/26) identified as male. Between 2013 and 2023, 78% (n = 947/1220) of individuals who successfully matched into a Canadian paediatrics residency identified as female and 22% (n = 273/1220) identified as male. There was a statistically significant difference between the gender of paediatric residents and the gender of paediatric cardiology residents (<em>P</em> < 0.0001). There was no significant association between applicant gender and match outcome within paediatric cardiology training programs (<em>P</em> < 0.23).</div></div><div><h3>Conclusions</h3><div>There is a gender discrepancy within Canadian paediatric cardiology training programs with a predominance of male trainees in the recent era. It appears that female under-representation in the field is due to the low number of female applicants. Although limited by sample size, there was no clear association between applicant gender and success of admission to a paediatric cardiology training program.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"3 6","pages":"Pages 241-246"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143176080","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}
Wisam Abozaid MD , Samantha Wong , Marc W. Deyell MD, FRCPC , Shubhayan Sanatani MD, FRCPC, FHRS , Sakethram Saravu Vijayashankar MD, MRCPCH
{"title":"Tachycardia-Induced Cardiomyopathy: A Case Series and a Literature Review","authors":"Wisam Abozaid MD , Samantha Wong , Marc W. Deyell MD, FRCPC , Shubhayan Sanatani MD, FRCPC, FHRS , Sakethram Saravu Vijayashankar MD, MRCPCH","doi":"10.1016/j.cjcpc.2024.10.007","DOIUrl":"10.1016/j.cjcpc.2024.10.007","url":null,"abstract":"<div><div>Tachycardia-induced cardiomyopathy (TIC), also known as arrhythmia-induced cardiomyopathy or tachycardiomyopathy, is a reversible form of heart failure characterized by persistent tachyarrhythmias and associated ventricular dysfunction. TIC is characterized by the reversal of myocardial damage with resolution of the arrhythmia. Early diagnosis of TIC is imperative, as the treatment course is distinct from cardiomyopathy of other or unknown causes. However, distinguishing TIC from tachycardia secondary to increased catecholamines due to congestive heart failure can be very challenging. There are relatively few paediatric reports, and herein we present a case series of 48 paediatric patients with TIC from literature (2014-2024). We also present 4 illustrative cases with TIC seen at our site (BC Children’s Hospital, Vancouver, Canada). The mean age in this case series was 6.98 ± 4.9 years. The majority of patients had ectopic atrial tachycardia (41.7%), followed by permanent junctional reciprocating tachycardia (20.8%), ventricular tachycardia (16.7%), and atrioventricular re-entrant tachycardia or atrioventricular nodal re-entrant tachycardia (10.4%). Pharmacologic treatment was the predominant therapy, but 70.8% of patients needed at least 1 ablation procedure. All patients demonstrated significant improvement in left ventricular ejection fraction after treatment, with most achieving at least near-normal ejection fractions in 80 days on average since presentation. In conclusion, TIC is overall a treatable condition with challenging diagnosis but generally has a favourable prognosis when diagnosed and treated appropriately. This article emphasizes the importance of considering TIC in the differential diagnosis of tachycardia in the context of reduced ventricular function, to recognize it and to enable targeted treatment initiation as soon as possible.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"3 6","pages":"Pages 272-284"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143176086","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}