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Context Professionalism lapses have high stakes for learners and educators. Problems with professionalism, unless appropriately and effectively remediated, may portend serious problems in practice. Yet, remediation for unprofessional behaviour is particularly challenging-and understudied. Increasingly, educators are turning to reflective writing as a remediation strategy in residency, yet little is known about what educators expect reflective writing to accomplish, how they choose reflective writing tasks, why they use reflective writing, or how they evaluate whether a learner has met expectations. We aimed to understand why and how postgraduate medical educators use reflective writing as an educational intervention to remediate professionalism. Method In this constructivist grounded theory study, we interviewed 13 medical education professionals with experience using reflective writing to remediate professionalism across five Canadian medical schools. Data collection and analysis occurred iteratively using constant comparison to identify themes and to understand the relationships among them. Results Medical educators reported using reflective writing as a learning tool to develop insight and as an assessment tool to unearth evidence of insight. The goal of learning may compete with the goal of assessment, creating tension that leads to uncertainty about the sincerity, quality, and effectiveness of reflective writing as well as concerns about learner safety. Educators reported uncertainty about whether learners write to pass or to introspect and about how to judge the effectiveness of reflective writing as a learning tool. They expressed concern about creating a safe environment for learners-one that enables the genuine reflection required for insight development-while meeting requirements of remediation. Conclusions Educators express ambivalence about using reflective writing to remediate professionalism in residency. Understanding the potential and pitfalls of reflective writing may inform more tailored and effective approaches to remediate professionalism.

Although cardiovascular magnetic resonance (CMR) imaging measurements are known to differ between sexes and according to age in the normal heart, little is known about measurement variability in patients with congenital heart disease (CHD). Yet, aggregate CMR data, without adjustment for sex or age

Background: Beta-blockers (BBs) are commonly prescribed to manage arrhythmias in children and adolescents without any standardised approach to establish BB adequacy. We invited all Canadian pediatric cardiologists to participate in an anonymous survey to understand practice variation in the assessment of BB adequacy in school-aged children and adolescents with arrhythmia or the potential for arrhythmia. Methods: An electronic survey approved by the Institutional Ethics Board was distributed by e-mail to 96 Canadian pediatric cardiologists who had been active in practice for at least 1 year. Incomplete surveys were excluded. Results: Forty-one cardiologists (43%) responded to all questions in the survey. Thirteen cardiologists (32%) reported always assessing BB adequacy, 17 (41%) did so only for specific arrhythmias, and 11 (27%) reported never performing such an assessment. A total of 19 cardiologists (46%) and 18 cardiologists (44%) reported using Holter monitoring and exercise testing, respectively, to assess beta receptor blockade adequacy. Thirteen cardiologists (32%) considered BB therapy adequate if Holter demonstrated a 20% decrease in heart rate (HR) from baseline, and 10 respondents (24%) defined adequate BB therapy using exercise testing as a 20% decrease in maximal HR or blood pressure from baseline. Conclusion: Despite wide variation in practice, Holter monitoring and exercise testing are commonly used methods to measure the adequacy of BB therapy. There are no standard criteria, but the majority (56%) reported using a 20% decrease in HR or blood pressure from the pretreatment state as a criterion for adequate BB therapy in children and adolescents with arrhythmia or the potential for arrhythmia. Résumé: Contexte: Les bêtabloquants (BB) sont couramment prescrits pour traiter les arythmies chez l’enfant et l’adolescent, en l’absence de méthode standardisée permettant d’établir le caractère adéquat du traitement par ces agents. Nous avons invité tous les cardiologues pédiatriques canadiens à participer anonymement à une enquête visant à rendre compte de la variabilité observée en pratique dans l’évaluation du caractère adéquat du traitement par BB chez l’enfant d’âge scolaire ou l’adolescent atteints d’arythmie ou susceptibles de l’être. Méthodologie: Un questionnaire électronique approuvé par le comité d’éthique en établissement a été distribué par courriel à 96 cardiologues pédiatriques canadiens en exercice depuis au moins un an. Les questionnaires partiellement remplis ont été exclus. Résultats: Parmi les participants, 41 cardiologues (43 %) ont répondu à toutes les questions de l’enquête. D’après les réponses obtenues, 13 cardiologues (32 %) évaluent toujours le caractère adéquat du traitement par BB, 17 (41 %) l’évaluent uniquement en présence de certains types d’arythmies et 11 (27 %) ne l’évaluent jamais. Au total, parmi les cardiologues interrogés, 19 (46 %) et 18 (44 %) affirment avoir recours respectivement à l’électrocardiographie (ECG) ambulatoire et à l’épreuve d’effort pour évaluer le caractère adéquat du blocage des récepteurs bêta. Ainsi, 13 cardiologues (32 %) jugent que le traitement par BB est adéquat si l’enregistrement ECG ambulatoire démontre une diminution de 20 % de la fréquence cardiaque (FC) maximale par rapport à la valeur de référence, et 10 cardiologues (24 %) estiment qu’il en est de même si le résultat obtenu à l’épreuve d’effort démontre une diminution de 20 % de la FC maximale ou de la pression artérielle par rapport aux valeurs de référence. Conclusion: Malgré de grandes variations observées en pratique, l’ECG ambulatoire et l’épreuve d’effort sont des méthodes couramment utilisées pour mesurer le caractère adéquat du traitement par BB. Il n’existe pas de critères standard, mais la majorité (56 %) des répondants ont indiqué qu’une diminution de 20 % de la FC ou de la pression artérielle par rapport aux valeurs de référence obtenues avant le traitement constitue un critère du caractère adéquat du traitement par BB chez l’enfant ou l’adolescent atteints d’arythmie ou susceptibles de l’être.

Background: Relocation, recruitment, or retirement of critical team members may lead to changes in the expertise pool that could threaten patient outcomes in a pediatric heart program. We developed a quality initiative aimed at risk management that uses risk-stratified case complexity and outcomes to guide a program during critical fluxes in the expert staff. The Ramp Down/Up protocol is a systematic, voluntary reduction in the complexity of cases performed, followed by a transparent and intentional escalation of case complexity. Methods: Institutional Ethics Review Board approval for this quality initiative was obtained. Patient/caregiver consent for quality data collection is obtained at the time of hospital admission. Every surgical patient having their index cardiac surgical procedure at the Izaak Walton Killam (IWK) from January 1, 2003, to December 2015 is included. The Ramp Down/Up protocol evolved to have to 4 critical elements: (1) a trigger and a reduction in case complexity; (2) an external/objective expert observer; (3) an escalation in case complexity; and (4) data (qualitative and quantitative) collection and analysis. Results: The Ramp Down/Up protocol was used 3 times over a 12-year period to address critical expert human resource challenges. The protocol was used for variable duration (3.5-9 months). Patient operative mortality was benchmarked to the Congenital Cardiac Surgery database, and outcomes were stable during and after protocol employment. Conclusions: A quality initiative aimed at risk management has allowed 1 pediatric heart team to ensure that patient outcomes were maintained during critical human resource changes. Résumé: Contexte: La relocalisation, le recrutement ou le départ à la retraite de membres critiques d’une équipe peuvent amener des changements dans le bassin d’expertises pouvant compromettre les résultats obtenus par les patients au sein d’un programme de pédiatrie cardiaque. Nous avons conçu une initiative relative à la qualité de la gestion des risques, qui évalue la complexité des cas et les résultats, stratifiés selon le risque, afin d’orienter le programme durant les flux critiques de personnel spécialisé. Le protocole de réduction/d’augmentation réalise une diminution méthodique et délibérée de la complexité des cas suivie d’une intensification transparente et intentionnelle de celle-ci. Méthodologie: Cette initiative sur la qualité a obtenu l’approbation du comité d’examen de l’éthique des établissements. Le consentement du patient/de l’aidant requis pour la collecte de données sur la qualité est obtenu au moment de l’admission à l’hôpital. L’initiative incluait tous les patients de chirurgie pour lesquels on disposait de l’indice de chirurgie cardiaque établi au centre Izaak Walton Killiam (IWK) entre le 1er janvier 2003 et décembre 2015. Le protocole de réduction/d’augmentation a évolué pour intégrer quatre éléments critiques : 1) un facteur déclencheur et une réduction de la complexité des cas; 2) un observateur externe/expert objectif; 3) une intensification de la complexité des cas; 4) la collecte et l’analyse des données (qualitatives et quantitatives). Résultats: Le protocole de réduction/d’augmentation a été utilisé à trois reprises sur une période de 12 ans et sur des durées variables (de 3,5 à 9 mois) dans le but de relever des défis majeurs liés aux ressources humaines spécialisées. Les taux de mortalité opératoire des patients ont été comparés à ceux de la base de données sur les chirurgies cardiaques congénitales, et les résultats se sont révélés stables durant et après l’emploi du protocole. Conclusions: Une initiative sur la qualité ciblant la gestion des risques a permis à une équipe de pédiatrie cardiaque de s’assurer que les patients continuaient d’obtenir des résultats similaires lors de changements critiques des ressources humaines.

Background: Medical therapy is often prescribed to reduce the rate of aortic dilatation and prevent aortic dissection in patients with bicuspid aortic valve (BAV) despite a lack of evidence. We conducted an anonymous survey to gain insight into Canadian clinical practice regarding medical therapy used to slow the progression of aortic dilatation in patients with BAV. Methods: A questionnaire was sent to 115 paediatric cardiologists and 18 adult congenital heart disease specialists in Canada. Results: Ninety of 113 paediatric cardiologists (80%) completing the questionnaire reported prescribing medication to reduce the rate of aortic dilatation. Some 75% (61/81) of them reported prescribing medications on the basis of aortic size z scores, and 78% (48/61) considered medication at a z score between ≥ 2 and < 5. The remaining 25% of responders (20/81) reported prescribing medications on the basis of absolute aortic diameter, and 80% (16/20) of them considered initiating medical therapy at an aortic diameter > 40 mm to < 50 mm. For practical purposes, however, 40% of respondents (45/113) would not or rarely consider medical therapy for this indication because of variation in the threshold for initiating treatment. Ten of 14 adult congenital heart disease specialists’ responses (71%), reported prescribing medications who were excluded because of missing data. Conclusion: The majority of Canadian paediatric cardiologists reported prescribing medications to slow the rate of aortic dilatation in patients with BAV. However, there is heterogeneity in the criteria to prescribe medical therapy. A multicenter randomized controlled trial is needed to establish the role of medical therapy in this patient population. Résumé: Introduction: En dépit du manque de données probantes, un traitement médical est souvent prescrit pour réduire le taux de dilatation aortique et prévenir la dissection aortique chez les patients ayant une bicuspidie aortique (BA). Nous avons mené une enquête anonyme pour avoir un aperçu de la pratique clinique au Canada en ce qui concerne le traitement médical utilisé pour ralentir la progression de la dilatation aortique chez les patients ayant une BA. Méthodes: Nous avons envoyé un questionnaire à 115 cardiologues en pédiatrie et à 18 spécialistes en cardiopathie congénitale de l’adulte du Canada. Résultats: Quatre-vingt-dix des 113 cardiologues en pédiatrie (80 %) ayant rempli le questionnaire ont signalé prescrire des médicaments pour réduire le taux de dilatation aortique. Certains d’entre eux, soit 75 % (61/81) ont signalé prescrire des médicaments en fonction du Z-score de la taille aortique, et 78 % (48/61) ont envisagé de prescrire les médicaments lorsque le Z-score était entre ≥ 2 et < 5. Les 25 % de répondants restants (20/81) ont signalé prescrire des médicaments en fonction du diamètre aortique absolu, et 80 % (16/20) d’entre eux ont envisagé de faire commencer le traitement médical lorsque le diamètre aortique était entre > 40 mm et < 50 mm. Concrètement, toutefois, 40 % des répondants (45/113) n’ont pas envisagé ou ont rarement envisagé de prescrire un traitement médical pour cette indication en raison de la variation dans le seuil de mise en place du traitement. Dix des 14 réponses (71 %) des spécialistes de la cardiopathie congénitale de l’adulte ont signalé prescrire des médicaments qui étaient exclus en raison de données manquantes. Conclusion: La majorité des cardiologues en pédiatrie du Canada ont signalé prescrire des médicaments pour ralentir le taux de dilatation aortique chez les patients ayant une BA. Toutefois, les critères utilisés pour prescrire un traitement médical sont hétérogènes. Un essai clinique multicentrique et à répartition aléatoire est nécessaire pour déterminer le rôle du traitement médical dans cette population de patients.

Background Quality improvement and patient safety (QIPS) have been assigned a higher profile in CanMEDS 2015, CanMEDS–Family Medicine 2017 and new accreditation standards, prompting an initiative at Dalhousie University to create a vision for integrating QIPS into postgraduate medical education. Objective The purpose of this study is to describe the implementation of a QIPS strategy across residency education at Dalhousie University. Methods A QIPS task force was formed, and a literature review and needs assessment survey were completed. A needs assessment survey was distributed to all Dalhousie residency programme directors. 12 programme directors were interviewed individually to collect additional feedback. The results were used to develop a ‘road map’ of recommendations with a graduated timeline. Results A task force report was released in February 2018. 46 recommendations were developed with a timeframe and responsible party identified for each. Implementation of the QIPS strategy is underway, and evaluation and challenges faced will be described. Conclusions We have developed a multiyear strategy that is available to provide guidance and support to all programmes in QIPS. The development and implementation of this QIPS framework may serve as a template for other institutions who seek to integrate these competencies into residency training.

Background: Researchers have shown that clinical educators feel insufficiently informed about how to teach and assess the CanMEDS roles. Thus, our objective was to examine the extent to which program directors utilize evidence-based tools and the medical education literature in teaching and assessing the CanMEDS roles.Methods: In 2016, the authors utilized an online questionnaire to survey 747 Canadian residency program directors (PD’s) of Royal College of Physicians and Surgeons of Canada (RCPSC) accredited programs.Results: Overall, 186 PD’s participated (24.9%). 36.6% did not know whether the teaching strategies they used were evidence-based and another third (31.9%) believed they were “not at all” or “to a small extent” evidence-based. Similarly, 31.8% did not know whether the assessment tools they used were evidence-based and another third (39.7%) believed they were “not at all” or “to a small extent” evidence-based. PD’s were aware of research on teaching strategies (62.4%) and assessment tools (51.9%), but felt they did not have sufficient time to review relevant literature (72.1% for teaching and 64.1% for assessment).Conclusions: Canadian PD’s reported low awareness of evidence-based tools for teaching and assessment, implying a potential knowledge translation gap in medical education research., Contexte : La recherche montre que les éducateurs en médecine se sentent insuffisamment informés sur la façon d’enseigner et d’évaluer les rôles CanMEDS. Notre objectif était donc d’examiner dans quelle mesure les directeurs de programmes utilisent des outils fondés sur les données probantes et la littérature en éducation médicale pour enseigner et évaluer les rôles CanMEDS.Méthodes : En 2016, les auteurs ont utilisé un questionnaire en ligne pour interroger 747 directeurs de programmes de résidence (DP) agréés par le Collège royal des médecins et chirurgiens du Canada (CRMCC).Résultats : Parmi les 186 (24,9 %) DP qui ont participé au sondage, 36,6 % ne savaient pas si les stratégies d’enseignement qu’ils utilisaient étaient fondées sur des données probantes et un tiers (31,9 %) estimaient qu’elles n’étaient « pas du tout » fondées sur des données probantes ou qu’elles ne l’étaient que « dans une faible mesure ». De surcroît, 31,8 % ne savaient pas si les outils d’évaluation qu’ils utilisaient étaient fondés sur des données probantes et un tiers (39,7 %) estimait qu’ils l’étaient « dans une faible mesure » ou qu’ils ne l’étaient « pas du tout ». Les DP ont déclaré qu’ils étaient au courant de l’existence d’études sur les stratégies d’enseignement (62,4 %) et sur les outils d’évaluation (51,9 %), mais qu’ils manquaient de temps pour se familiariser avec la documentation pertinente (72,1 % pour l’enseignement et 64,1 % pour l’évaluation).Conclusions : Les DP au Canada sont peu familiers avec les outils d’enseignement et d’évaluation fondés sur les données probantes, ce qui suggère un transfert des connaissances lacunaire de la recherche à l’éducation médicale.

Fetal compressive intrapericardial teratoma is a rare and life-threatening condition, qualifying as a high-acuity low-occurrence (HALO) event. To prepare for delivery and immediate neonatal management, specialists from pediatric cardiology, cardiac surgery, maternal-fetal-medicine, neonatology, cardiac anesthesia, critical care, clinical perfusion, obstetrical nursing, and operating room nursing convened. An in situ operating room simulation was used to identify and introduce key team members, derive and practice the anticipated clinical management algorithm, position human and equipment resources strategically, and ensure that each specialist team was familiar with the environment and available equipment. As rehearsed in the simulation, the cesarean delivery of the patient and neonatal cardiac surgery was uncomplicated and yielded a favourable clinical outcome. A patient-specific HALO simulation preparation (PSHSP) can facilitate positive clinical outcomes and improve health care team confidence in HALO scenarios such as the birth of newborns anticipated to have cardiorespiratory instabilty.

To compare the effect of umbilical cord milking (UCM) vs. early cord clamping (ECC) on cerebral blood flow (CBF). Preterm infants

Histomorphometry of endomyocardial biopsies is one component of arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnosis, although there is a need for stricter diagnostic criteria for this disease in pediatrics. The clinical utility of biopsy analysis as a component of ARVC diagnosis was evaluated in pediatric patients. Histomorphometric analysis of fibrofatty infiltrate was completed on pediatric right ventricular endomyocardial biopsy samples. Myocardial replacement by fat and fibrosis was quantified. ARVC diagnosis was established using the 2010 ARVC Task Force criteria, with the biopsy measures compared across various ARVC diagnoses (definite, borderline, possible, or no ARVC). Receiver-operating characteristic (ROC) curve analysis was also completed using biopsy measures. The greatest proportion of fat, fibrosis, and myocardial replacement was in the definite ARVC cohort, and was significantly larger than for the other diagnosis cohorts. ROC curve analysis (with the biopsy analysis removed from the diagnostic classification) produced cutoff values of 15 and 25% myocardial replacement, which is lower than current adult diagnosis criteria. We propose modifications in pediatric major and minor biopsy diagnosis criteria to allow for improved sensitivity. This study suggests that biopsy analysis in children is most significant for subjects with a more severe disease presentation.

PurposeThe authors previously developed and tested a reflective model for facilitating performance feedback for practice improvement, the R2C2 model. It consists of four phases: relationship building, exploring reactions, exploring content, and coaching. This research studied the use and effectiveness of the model across different residency programs and the factors that influenced its effectiveness and use.MethodFrom July 2014-October 2016, case study methodology was used to study R2C2 model use and the influence of context on use within and across five cases. Five residency programs (family medicine, psychiatry, internal medicine, surgery, and anesthesia) from three countries (Canada, the United States, and the Netherlands) were recruited. Data collection included audiotaped site assessment interviews, feedback sessions, and debriefing interviews with residents and supervisors, and completed learning change plans (LCPs). Content, thematic, template, and cross-case analysis were conducted.ResultsAn average of nine resident-supervisor dyads per site were recruited. The R2C2 feedback model, used with an LCP, was reported to be effective in engaging residents in a reflective, goal-oriented discussion about performance data, supporting coaching, and enabling collaborative development of a change plan. Use varied across cases, influenced by six general factors: supervisor characteristics, resident characteristics, qualities of the resident-supervisor relationship, assessment approaches, program culture and context, and supports provided by the authors.ConclusionsThe R2C2 model was reported to be effective in fostering a productive, reflective feedback conversation focused on resident development and in facilitating collaborative development of a change plan. Factors contributing to successful use were identified.

ObjectiveTo investigate whether umbilical cord milking (UCM) at birth improves systemic blood flow and short-term outcomes, as compared with immediate cord clamping (ICC).DesignRandomised clinical trial.SettingSingle tertiary care centre.PatientsInfants born to eligible women presenting in preterm labour between 24 and 31 weeks’ gestation.InterventionsUCM three times at birth or ICC.Outcome measuresPrimary outcome included systemic blood flow as represented by echo-derived superior vena cava(SVC) flow at 4–6 hours after birth. The echocardiographer and interpreter were blinded to the randomisation. Secondary outcomes included cardiac output, neonatal morbidities and mortality. Analysis was by intention to treat.ResultsA total of 73 infants were randomised (37 to UCM and 36 to ICC). Mean (SD) gestational age was 27 (2) weeks and mean (SD) birth weight was 1040 (283) g. Haemoglobin on admission was higher in the UCM than in the ICC group (16.1 vs 15.0 g/L), p=0.049 (mean difference 1.1, 95% CI 0.003 to 2.2). No statistically significant differences were found between groups in SVC flow at 4–6 hours (88.9±37.8 and 107.3±60.1 mL/kg/min), p=0.13 (mean difference −18.4, 95% CI −41.7 to 5.0 mL/kg/min) or at 10–12 hours of age (102.5±41.8 and 90.6±28.4 mL/kg/min), p=0.17 (mean difference 12.0, 95% CI −4.7 to 28.7 mL/kg/min), cardiac output or neonatal morbidities.ConclusionsCord milking was not shown to improve functional cardiac outcomes, neonatal morbidity or mortality. More research is needed before routine cord milking can be recommended for very preterm infants.Trial registrationNCT01487187.

In 2010, the Association of Faculties of Medicine of Canada, Collège des médecins du Québec, College of Family Physicians of Canada, and Royal College of Physicians and Surgeons of Canada launched the Future of Medical Education in Canada Postgraduate (FMEC PG) Project to examine postgraduate medical education (PGME) in Canada and make recommendations for improvement. One recommendation that emerged concerns the transitions learners experience across the undergraduate medical education-PGME-practice continuum. The FMEC PG, using a thorough process, developed projects to address these often-tumultuous transitions for the learner, aiming to provide support, tools, and standards for the learner's educational journey.With leadership by two senior academics and the Transitions Implementation Committee, three working groups helped implement these transitions projects, which addressed (1) the medical-school-to-residency transition, (2) career planning and the residency matching process, and (3) the residency-to-practice transition. Work products include the development of a learner education handover protocol and the establishment of pan-Canadian entrustable professional activities to be used nationally to help define expectations for new graduates entering residencies. A postmatch boot camp tool and a simulated night on-call tool were developed and are available to all medical schools. National standards are being promoted for career services counseling and best practices in residency selection. A practice management curriculum framework, mentorship resources, resiliency training for graduating residents, and the entry-level disciplines of residency are also being explored.Ultimately, with system-wide change and better integration of all players, transitions for Canada's learners will greatly improve.

Background Feedback is increasingly seen as a collaborative conversation between supervisors and learners, where learners are actively and reflectively engaged with feedback and use it to improve. Based on this, and through earlier research, we developed an evidence- and theory-informed, 4-phase model for facilitating feedback and practice improvement—the R2C2 model (relationship, reaction, content, coaching). Objective Our goal was to explore the utility and acceptability of the R2C2 model in residency education, specifically for engaging residents in their feedback and in using it to improve, as well as the factors influencing its use. Methods This qualitative study used the principles of design research. We recruited residents and their supervisors in 2 programs, internal medicine and pediatrics. We prepared supervisors to use the R2C2 model during their regular midrotation and/or end-of-rotation feedback sessions with participating residents to discuss their progress and assessment reports. We conducted debriefing interviews with supervisors and residents after each session. We analyzed transcripts as a team using template and content analysis. Results Of 61 residents, 7 residents (11%) participated with their supervisors (n = 5). Schedules and sensitivity to feedback prevented broader enrollment. Supervisors found the structured R2C2 format useful. Residents and supervisors reported that the coaching phase was novel and helpful, and that the R2C2 model engaged both groups in collaborative, reflective, goal-oriented feedback discussions. Conclusions Participants found that using the R2C2 model enabled meaningful feedback conversations, identification of goals for improvement, and development of strategies to meet those goals.

BACKGROUND: Umbilical cord milking at birth has been reported to have short-term benefits to preterm infants. Long-term neurodevelopmental outcomes need more exploration. OBJECTIVES: To compare the effects of cord milking (CM) vs. early cord clamping (ECC) at birth on neurodevelopmental outcomes at 36 months corrected age. DESIGN/METHODS: Preterm infants

We describe the management of a parturient woman with hypertrophic cardiomyopathy who developed a symptomatic accelerated idioventricular rhythm who required an urgent cesarean delivery at 32 weeks. Transthoracic echocardiography helped guide anesthetic management, including epidural dosing, fluid management, and phenylephrine infusion rates. This case demonstrates the application of transthoracic echocardiography to guide anesthetic management in a parturient woman at risk for cardiovascular compromise.

Background: Policies to guide remediation in postgraduate medical education exist in all Canadian medical schools. This study examines concordance between these policies and processes, and published “best practices” in remediation. Method: We conducted a literature review to identify best practices in the area of remediation. We then reviewed remediation policies from all 13 English medical schools in Canada other than our own and conducted interviews with key informants from each institution. Each policy and interview transcript pair was then reviewed for evidence of pre-defined “best practices.” Team members also noted additional potential policy or process enablers of successful remediation. Results: Most policies and processes aligned with some but not all published best practices. For instance, all participating schools tailored remediation strategies to individual resident needs, and a majority encouraged faculty-student relationships during remediation. Conversely, few required the teaching of goal-setting, strategic planning, self-monitoring, and self-awareness. In addition, we identified avoidance of automatic training extension and the use of an educational review board to support the remediation process as enablers for success. Discussion: Remediation policies and practices in Canada align well with published best practices in this area. Based on key informant opinions, flexibility to avoid training extension and use of an educational review board may also support optimal remediation outcomes.

Anecdotal and European evidence suggests that outpatient pediatric referrals and their diagnostic testing burden are increasing. We sought to characterize new pediatric cardiology referrals, testing performed, outcomes, and patient satisfaction in a Canadian academic hospital and how these had changed over time. Clinical data were extracted from new outpatient consultations to the IWK Children's Heart Centre between August 1, 2011 and August 17, 2012 and compared with similar local data collected in July-February 2002 using χ(2) testing. Predictors of significant differences were sought using regression analysis. Satisfaction data were collected from a validated patient questionnaire, and 620 new outpatients were evaluated. Organic disease was more likely in younger patients (odds ratio [OR], 2.7; 95% confidence interval [CI], 1.8-4.0) or in patients referred by pediatricians (OR, 2.3; 95% CI, 1.6-3.3). Odds of echocardiography being performed were significantly increased if patients were younger than 1 year (OR, 2.0; 95% CI, 1.3-3.0), were seen at outreach clinics (OR, 1.7; 95% CI, 1.2-2.3), or were referred by pediatricians (OR, 3.7; 95% CI, 2.6-5.3). Cardiologists differed significantly in ordering echocardiograms for referred patients (P = 0.002). The patients referred in the current era have significantly less organic disease than did those in 2002 (27% vs 37%; P = 0.007), but they underwent significantly more echocardiography (58% vs 38%; P < 0.001) and Holter monitoring (12% vs 4%; P = 0.001). Satisfaction results were high and unrelated to diagnostic testing. Pediatric cardiology referrals in Maritime Canada have increased in volume, consistent with changes seen at other centres. This, coupled with changing cardiac investigations, has increased testing burden. Individual cardiologists affected the odds of echocardiography being ordered. Satisfaction with services was high, with no predictors identified.

The importance of confidence for learning and performance makes learners' perceptions of readiness for the next level of training valuable indicators of curricular success. The "Readiness for Clerkship" (RfC) and "Readiness for Residency" (RfR) surveys have been shown to provide reliable ratings of the relative effectiveness of various aspects of training. This study examines the generalizability of those results.Surveys were administered at four medical schools approximately four months after the start of clerkship and eight months after the start of residency during 2013-2015. Collected data were anonymized. A total of 647 medical students and 483 residents participated.Reliabilities of G = 0.8 could be obtained with only 6 to 12 medical students and 8 to 15 residents. Within MD programs, no meaningful differences in item ratings were observed across cohorts. Residents in each school consistently rated themselves higher than clerks on the majority of Medical Expert and Communicator competencies common to both surveys. Similar strengths and weaknesses were identified across programs, but differences were observed on five clerkship items and one residency item.Across four MD programs, the RfC and RfR surveys provided reliable ratings of the relative effectiveness of aspects of training with small numbers of respondents. The capacity of these surveys to efficiently identify perceived strengths and weaknesses held by cohorts of learners may, thereby, facilitate program improvement.

Background Chronic hemodynamically relevant pulmonary regurgitation (PR) resulting in important right ventricular dilation and ventricular dysfunction is commonly seen after tetralogy of Fallot (TOF) repair. Late adverse clinical outcomes, including exercise intolerance, arrhythmias, heart failure and/or death accelerate in the third decade of life and are cause for considerable concern. Timing of pulmonary valve replacement (PVR) to address chronic PR is controversial, particularly in asymptomatic individuals, and effect of PVR on clinical measures has not been determined. Methods Canadian Outcomes Registry Late After Tetralogy of Fallot Repair (CORRELATE) is a prospective, multicentre, Canada-wide cohort study. Candidates will be included if they are ≥ 12 years of age, have had surgically repaired TOF resulting in moderate or severe PR, and are able to undergo cardiovascular magnetic resonance imaging. Enrollment of > 1000 individuals from 15 participating centres (Toronto, Montreal, Quebec City, Sherbrooke, Halifax, Calgary, Edmonton, and Vancouver) is anticipated. Clinical data, health-related quality of life metrics, and adverse outcomes will be entered into a web-based database. A central core lab will analyze all cardiovascular magnetic resonance studies (PR severity, right ventricular volumes, and ventricular function). Major adverse outcomes (sustained ventricular tachycardia and cardiovascular cause of death) will be centrally adjudicated. Results To the best of our knowledge, CORRELATE will be the first prospective pan-Canadian cohort study of congenital heart disease in children and adults. Conclusions CORRELATE will uniquely link clinical, imaging, and functional data in those with repaired TOF and important PR, thereby enabling critical evaluation of clinically relevant outcomes in those managed conservatively compared with those referred for PVR.

Background As overall mortality rates have fallen in pediatric cardiac surgical procedures, complication monitoring is becoming an increasingly important metric of patient outcome. Currently there is no standardized method available to monitor severity-adjusted complications in congenital cardiac surgical procedures. Methods Complications associated with pediatric cardiac surgical procedures were prospectively collected from consecutive cases in a single pediatric cardiac surgical unit from October 1, 2009 to September 31, 2011. Complications were accounted for by frequency and severity and then stratified by surgical complexity, using the Risk Adjustment for Congenital Heart Surgery (RACHS) method, giving an average morbidity burden per RACHS category. "Expected" morbidity burden for each RACHS category was derived from year 1 (2009–2010) data. Observed minus expected (O:E) plots were then generated for the entire series of complications from year 2 (2010–2011) data. Separate O:E plots were also created for 5 complication classes and monitored for increases. Results There were 181 index surgical procedures performed in 178 patients. Two hundred and seventeen complications occurred in 80 procedures. The frequency and severity of complications increased with surgical complexity. The overall O:E plot was flagged twice for unanticipated increases in severity-adjusted complications. When the class-specific O:E plots were monitored for increases, the overall flags were found to originate from increased rates of infections and cardiac/operative complications. Conclusions The O:E plot provides a simple and effective system to monitor complication rates over time based on severity-adjusted complication data. Grouping complications into classes allows us to identify specific subsets of complications that can be focused on to improve patient outcomes.

Objective Our objective was to establish baseline data and develop a tool to allow for systematic evaluation of pediatric cardiac surgical complications. As a first step, we examined the incidence and distribution of complications, risk stratified by case complexity in a single institution. With improving mortality rates for congenital heart surgery, the next frontier for improving patient outcomes is characterizing and reducing complications. Currently, no standardized approach is available to monitor the incidence and severity of all complications associated with a congenital cardiac surgery program. Methods Complications occurring in pediatric cardiac surgical patients (January 2006 to March 2009) were collected by database review applying standardized definitions. The surgical procedures were stratified by complexity to analyze the distribution of complications over the risk spectrum. Each complication was assigned a severity coefficient (1–3) used to calculate the combined effect of frequency and severity. The cumulative sum method was used to determined the trend of the adverse outcomes. Results Of 292 procedures, 84 (28.8%) were associated with a total of 150 complications. Of the 150 complications, 37 occurred in patients who died. The most common complications were arrhythmias (14.5%), cardiac (12.6%), and operative (12.6%). There was a linear relationship between the frequency and severity of complications and surgical complexity, as stratified using the Risk Adjustment for Congenital Heart Surgery category or Aristotle basic complexity levels (Spearman's coefficient = 1). Conclusions When examined in a systematic fashion, the risk of complications in pediatric cardiac surgical patients is considerable. Our data illustrate that it is possible to track complications over time in a consistent manner. The effect of complication monitoring on patient outcomes remains to be proved.

Objectives To determine if dexamethasone given to premature infants with bronchopulmonary dysplasia would result in cardiac diastolic dysfunction in early childhood, a topic unstudied in humans. Study design We compared seven children ages 3 to 8 years born at 26 weeks’ gestation and given dexamethasone for bronchopulmonary dysplasia with eight gestation-matched and age-matched control children using echocardiography to assess measures of systolic and diastolic function. All dexamethasone patients had resolved hypertrophic cardiomyopathy. Results Dexamethasone patients had the same normal τ and isovolumic relaxation time (24.9 ± 2.8 and 54.6 ± 6.3 ms) as control patients (22.1 ± 3.0 and 48.8 ± 6.7 ms). Peak A velocities were the same in dexamethasone patients as in control patients (59.5 ± 15 versus 49.4 ± 5.8 cm/s, P = .10), resulting in unchanged E:A ratios (1.89 ± 0.57 versus 2.15 ± 0.43, P = .22). Peak E velocity and E-wave deceleration times were not different. We found no significant differences in measures of systolic function (heart rate–corrected velocity of circumferential fiber shortening, wall stress, and ejection fraction). Left ventricular mass was the same between the groups confirming resolution of hypertrophic cardiomyopathy. Conclusions These data are consistent with normal myocardial relaxation, suggesting that long-term diastolic function is reassuringly normal in children who received dexamethasone as premature infants with resolution of hypertrophic cardiomyopathy.

BackgroundCanadian Cardiovascular Society consensus guidelines recommend that tetralogy of Fallot patients be seen by a congenital cardiologist every 2 years. In Atlantic Canada, tetralogy of Fallot patients are followed up at either tertiary or satellite clinics, which are held in the community and attended by paediatric cardiologists. The effectiveness of satellite clinics in congenital cardiac disease follow-up is unproven. Our objective was to compare patient-reported quality of life measures to determine whether these were impacted by the site of follow-up.MethodsWe included patients with tetralogy of Fallot undergoing surgical repair at the Izaak Walton Killam Health Centre from 1 November, 1972 to 31 May, 2002. Quality of life surveys, SF-10 or SF-36v2, were administered to consenting patients. We analysed the subjective health status by patient age and site of follow-up.ResultsOf the 184 eligible patients, 72 were lost to follow-up. Of the locatable patients, 61% completed the questionnaires. In all, 90% (101 out of 112) were followed up at recommended intervals. Of the 112 (68%) patients, 76 were followed up at a tertiary clinic. These patients were older, with a mean age of 18.4 years versus 14.7 years, and scored higher on the SF-36 physical component summary (52.6 versus 45.7, p = 0.02) compared with satellite clinic patients. The SF-36 mental component summary scores were similar for patients regardless of the site of follow-up. SF-10 physical and psychosocial scores were similar regardless of the site of follow-up.ConclusionTetralogy of Fallot patients followed at either satellite or tertiary clinics have similar subjective health status.

This report describes a 1-year exchange between members of two pediatric cardiology centers: one in Canada and one in Australia. Five cardiologists participated in sequence, fully engaging in the activities of the host department. The motivation of the exchange was broadly educational including clinical experience, shared expertise, teaching, and research collaboration. Structured debriefing confirmed the value of the exchange. In addition to the experience of working in a different medical system, eight research papers were developed, with two research projects ongoing as well as subsequent exchanges of nursing and technical personnel. Interchange between two academic departments can add strength to both and allow development of new skills and research activity.

Il est demontre que le traitement pharmacologique par des stimulants reduit les symptomes associes au trouble de deficit de l’attention avec hyperactivite (TDAH), et ce traitement fait partie des recommandations therapeutiques de l’American Academy of Pediatrics pour les enfants presentant ce trouble (1,2). Cependant, de recentes decisions en matiere de reglementation sur l’homologation et l’etiquetage des medicaments contre le TDAH (3,4), ainsi qu’un recent document de principes de l’American Heart Association (5), une correction et une reponse (clarification) de l’American Academy of Pediatrics (6,7), ont souleve des questions sur l’evaluation et la therapie du TDAH que preconisent les praticiens canadiens. La pratique canadienne actuelle dans le domaine de la pharmacotherapie du TDAH et du depistage cardiovasculaire est mixte, et il y a peu de consensus quant a l’evaluation pertinente des enfants avant d’instaurer la pharmacotherapie du TDAH (8). Les avis sont encore plus partages au sujet de l’innocuite du traitement des enfants ayant subi une refection ou une palliation de leur cardiopathie congenitale (CPC) et qui prennent des medicaments contre le TDAH (8). Afin d’orienter les medecins canadiens qui soignent les enfants ayant un TDAH et de clarifier la situation, la Societe canadienne de pediatrie, la Societe canadienne de cardiologie et l’Academie canadienne de psychiatrie de l’enfant et de l’adolescent ont prepare conjointement le present document de principes. Il contient des recommandations consensuelles sur l’evaluation du risque cardiaque chez les enfants ayant un TDAH a qui on envisage d’administrer des stimulants. Il ne s’attarde pas au diagnostic de TDAH, ni aux bienfaits therapeutiques des stimulants. De meme, il n’aborde pas le bien-fonde ou les risques relatifs d’un medicament par rapport a un autre au sein de cette population de patients. Enfin, les medicaments non stimulants utilises dans le traitement du TDAH, y compris l’atomoxetine, les antidepresseurs et les agonistes alpha-adrenergiques, ne font pas l’objet d’une analyse particuliere.

Objectives To describe a program for passive immunization against respiratory syncytial virus infection in infants with hemodynamically significant congenital heart disease (CHD) in accordance with the Canadian Paediatric Society recommendations. Methods A provincial coordinating committee composed of specialists in pediatrics, cardiology, infectious diseases, neonatology and public health collaborated to develop and implement a program to identify children with hemodynamically significant heart disease and offer respiratory syncytial virus prophylaxis. Database records for all children younger than two years of age who were seen by the provincial pediatric cardiology referral service were reviewed. Children with hemodynamically significant heart disease, as determined by a clinical assessment and echocardiography, were considered to be eligible for monthly palivizumab prophylaxis for five winter months. All physicians in the province were notified that approval of the provincial cardiology service was required for prophylaxis to be administered. Immunization rates were compared with projected rates based on the expected population-based immunization rates in infants with CHD in other provinces. Results 401 children with any cardiac diagnoses were identified, representing 545 potential patient-seasons of prophylaxis over two years in a birth cohort of 20,173 and 19,227 children, in each respective season (13.8 patient-seasons per 1000 births). Of these, 21 children were eligible for palivizumab prophylaxis according to the Canadian Paediatric Society criteria. All eligible children were immunized, and no ineligible children received the immunization. A review of palivizumab use in other provinces revealed highly variable rates of prophylaxis. Conclusions The use of palivizumab for children with CHD can be optimized through a provincial model, in which children requiring prophylaxis are prospectively identified and reviewed by pediatric cardiologists – and to whom evidence-based guidelines developed by a multidisciplinary team – are applied. Such a model ensures that all patients requiring prophylaxis receive the appropriate immunization and avoids the immunization of low-risk children with CHD, in whom the benefits of palivizumab have not been proven.

OBJECTIVE. The objective of this study was to assess whether computer-stored digital sound recordings can be used to distinguish innocent from pathologic systolic murmurs.METHODS. Recordings of 55 children aged 1 month to 19 years were made remotely with the use of a digital stethoscope and were e-mailed to a computer in our center for later assessment. Eight-second recordings were made by a physician in 2 to 4 locations on the chest. Three cardiologists who were blinded to the diagnosis reviewed the recordings independently using stethophones to assess the splitting of the second heart sound and whether murmurs were innocent or pathologic. Diagnoses were confirmed with echocardiography.RESULTS. Seventeen children had innocent murmurs and 38 had pathologic murmurs. For the 3 cardiologists, sensitivity was 0.87 to 1.0, specificity was 0.82 to 0.88, negative predictive value was 0.75 to 1.0, and positive predictive value was 0.93 to 0.95. Assessment of splitting of second heart sound was highly accurate.CONCLUSIONS. Digital recordings of children’s heart sounds allow reliable differentiation between innocent and pathologic murmurs. Use of this technology may allow remote diagnosis of childhood murmurs and avoid the expense and stress of travel to pediatric cardiology centers for some children. Cardiologists who use recordings should assess their diagnostic accuracy before clinical application.

Objectives: To describe the incidence and rate of dilatation of the ascending aorta in children with bicuspid aortic valve (BAV) and to determine factors that predict rapid aortic dilatation. Design: Retrospective cohort study. Setting: Regional tertiary care children’s hospital. Patients: All children aged 0–18 years seen at the authors’ institution between 1990 and 2003 with an “isolated” BAV. All patients had had more than one technically adequate echocardiogram, at least six months apart, with concomitant height and weight data. Interventions: Offline echocardiographic measurements of multiple levels of the aortic root were completed for each participant at each serial echocardiogram. These measurements were then compared with expected measurements derived from a normal local control population. Main outcome measures: Rate of change of the ascending aorta size over time, where aortic size is expressed as the number of standard deviations above or below the mean size expected for a given body surface area (z score). Results: 279 echocardiograms spanning a period of from 9 months to 13.3 years were analysed for 88 patients with BAV. The ascending aorta in the BAV group was larger than expected for body surface area at diagnosis and continued to increase in relative size at each of the four subsequent follow-up echocardiograms. Ascending aortic z score increased at an average rate of 0.4/year. A faster rate of increase in z score was predicted by both larger initial aortic valve gradient and non-use of β blockers. Conclusions: Children with BAV are at risk of having a dilated ascending aorta. This risk increases with longer follow up.

Background GrAS causes illness ranging from uncomplicated pharyngitis to life-threatening necrotizing fasciitis, toxic shock and rheumatic fever. Attempts to develop an M protein-based vaccine have been hindered by the fact that some M proteins elicit both protective antibodies and antibodies that cross-react with human tissues. New molecular techniques have allowed this obstacle to be largely overcome. Methods We performed a phase II trial of a 26-valent GrAS vaccine comprising four recombinant proteins containing N-terminal peptides from 26 M proteins (plus Spa = 27) of common pharyngitis, invasive, and/or rheumatogenic serotypes adsorbed to AlOH 3 . Subjects were screened for good health and underwent baseline cardiac auscultation, echocardiography, ECG, and screening for human tissue-cross-reacting antibodies. Subjects (mean age = 33.6, range 18.9–50.9) were randomized in a 70:20 ratio to receive either GrAS or control vaccine, Havrix™, intramuscularly at 0, 1 and 6 months, with clinical and laboratory follow-up for safety and assay of type-specific M antibodies by quantitative ELISA. Results No vaccine-associated serious adverse events (SAE) occurred. Most adverse events (AEs) were at the injection site and were mild and self-limited. Systemic AEs were uncommon and did not differ between the 2 groups. No subject developed clinical or laboratory evidence of rheumatogenicity or nephritogenicity, and no induction of human tissue-reactive antibodies was demonstrated. The vaccine was highly immunogenic, eliciting an antibody response to the majority of group A streptococcus serotypes associated with pharyngitis, acute rheumatic fever, and invasive disease in North America Conclusions The 26-valent GrAS vaccine was well-tolerated and immunogenic in healthy adults; studies in adolescents and children are warranted.

We sought to determine the potential additive usefulness of Doppler tissue imaging (DTI), strain (epsilon), and epsilon rate (SR) imaging in assessing systolic function in pediatric patients with Alström syndrome.We conducted a case-control study at a pediatric hospital with 5 patients (age 5-18 years) with Alström syndrome living in Atlantic Canada and 21 age- and sex-matched healthy control subjects. Standard echocardiographic examination was followed by DTI of the interventricular septum (IVS) and left ventricle (LV) lateral wall, longitudinal epsilon, and SR at the basal, mid, and apical segments of the LV lateral, inferior, and anterior walls, and the IVS. We also imaged radial epsilon and SR of the interventricular posterior wall.For patients versus control subjects, conventional ejection fraction (0.65 vs 0.72) and fractional shortening (0.30 vs 0.35) did not distinguish between groups. DTI-derived s-waves consistently demonstrated significant differences in systolic function (LV lateral wall 0.073 vs 0.100; IVS 0.064 vs 0.080; LV anterior wall 0.066 vs 0.096; LV inferior wall 0.069 vs 0.092 [P.05 in all positions]). Epsilon differences were observed in the movement of the mid-LV lateral (-10.4 vs -15.2, P = .035), basal LV anterior wall (-16.3 vs -22.9, P = .004), and the apical IVS (-6.3 vs -13.8, P = .015). SR at the midposition of the LV lateral wall (-0.7 vs -1.4, P = .000) also differed between the groups. Substantial diastolic function differences were also observed between patients and control subjects.Detection of systolic and diastolic function abnormalities in patients with Alström syndrome can potentially be enhanced by the use of DTI, epsilon, and SR imaging.

Background The current objectives for teaching paediatric cardiology to paediatric residents have not been validated and may not be relevant to current paediatric practice. Objectives To validate the cardiology component of the Royal College of Physician and Surgeons of Canada's objectives for training paediatricians. Methods A questionnaire was sent to practising paediatricians in Atlantic Canada. The questions were based on the Royal College of Physician and Surgeons of Canada's training objectives. The frequency of problems seen, confidence in assessment and management of problems, and reasons for referral were identified. Clinical vignettes were followed by short questions. The outpatient referrals were reviewed to validate the questionnaire responses. Results One hundred fifty-one questionnaires were mailed and the response rate was 60%. Murmurs were the most common problem encountered (92%). Syncope (9%), Kawasaki disease (8%) and chest pain (6%) were less frequently encountered. Paediatricians were confident in assessing and managing problems despite the low frequency of encounters. Less confidence was expressed regarding physical examination skills and interpretation of electrocardiograms. Uncertainty of the diagnosis was the most common reason for patient referral, with parental anxiety and medicolegal concerns accounting for 24% and 7% of referrals, respectively. Syncope with exercise was relatively poorly recognized as a worrisome symptom. Conclusions Most cardiology objectives for general paediatric training remain relevant and appropriate to clinical practice. Physical examination skills, electrocardiogram interpretation and the assessment of syncope need to be emphasized.

Objective This study examines the incidence and factors associated with the failure of homograft valves and identifies those factors that are modifiable. Methods From 1990 to 2001, 96 homograft valves were implanted in the right ventricular outflow tract of 83 children (mean age 5.1 ± 5.6 years). Clinical and blinded serial echocardiographic follow-up was performed on all 90 valves in the 77 survivors. Results Eighteen homograft valves were replaced as the result of pulmonary insufficiency (3), stenosis (9), or both (6). Freedom from reoperation was 71% at 9 years (95% confidence interval, 58%-84%). Forty-eight valves developed progressive pulmonary insufficiency of at least 2 grades, 26 valves developed transvalvular gradients of 50 mm Hg or greater, and 14 of these valves were also insufficient. The freedom from echocardiographic failure (progressive pulmonary insufficiency ≥2 grades or ≥50 mm Hg gradient) was only 27% at 5 years (95% confidence interval, 17%-37%). In a multivariate analysis (Cox regression), use of an aortic homograft ( P = .001) and short antibiotic preservation time ( P = .04) were associated with reoperation. Younger age ( P = .01), ABO mismatch ( P = .04), and diagnosis ( P = .005) were associated with echocardiographic failure. In the subanalysis of patients with human leukocyte antigen typing, age ( P = .002), aortic homograft ( P = .04), and human leukocyte antigen-DR mismatch ( P = .03) were associated with echocardiographic valve failure. Conclusion Many homografts rapidly become insufficient and require replacement. In our analysis of both reoperation and echocardiographic failure, several immunologic factors are consistently associated with homograft failure. Matching for human leukocyte antigen-DR, blood group, and avoiding short preservation times (thus minimizing antigenicity) offers the potential to extend the life of these valves.

Physicians are required to maintain and sustain professional roles during their careers, making the Professional Role an important component of postgraduate education. Despite this, this role remains difficult to define, teach and assess.To (a) understand what program directors felt were key elements of the CanMEDS Professional Role and (b) identify the teaching and assessment methods they used.A two-step sequential mixed method design using a survey and semi-structured interviews with Canadian program directors.Forty-six program directors (48% response rate) completed the questionnaire and 10 participated in interviews. Participants rated integrity and honesty as the most important elements of the Role (96%) but most difficult to teach. There was a lack of congruence between elements perceived to be most important and most frequently taught. Role modeling was the most common way of informally teaching professionalism (98%). Assessments were most often through direct feedback from faculty (98%) and feedback from other health professionals and residents (61%). Portfolios (24%) were the least used form of assessment, but they allowed residents to reflect and stimulated self-assessment.Program directors believe elements of the Role are difficult to teach and assess. Providing faculty with skills for teaching/assessing the Role and evaluating effectiveness in changing attitudes/behaviors should be a priority in postgraduate programs.

Background: The next horizon for improving pediatric cardiac surgery outcomes is the standardization and systematic tracking of complications. Methods: IWK REB approval was obtained. The Multisocietal Database Committee short list of complications (52) were captured prospectively for all pediatric cardiac operations at the IWK Oct 1 2009-Sept 30 2010. Morbidity burden was calculated by multiplying a severity coefficient (1-3) by frequency of complication in each surgical complexity strata using RACHS categories. Death was included as a complication (severity coefficient= 5). Indexed morbidity was calculated for each RACHS category by dividing morbidity burden by number of procedures. Results: A total of 110 procedures were performed on 86 patients. 83 of the 86 index procedures were open and 22 of the patients were neonates. The procedural mortality rate was 3.6%. Forty one (41/86, 47.7%) of the index procedures had a total of 89 complications. Sixty-four patients were in RACHS category 2 or 3. The most common complications were pulmonary (22) arrhythmias (16), or operative (16), accounting for 61%(54/89) of total complications. The indexed morbidity was 0 for RACHS1, 1.55 for RACHS 2, 2.63 for RACHS 3, 1.81for RACHS 4, and 2.81 for RACHS5/6. The CUSUM plots the occurrence of any complication in each case versus morbidity burden, illustrating the effect of complication severity on the slope of the curve. Conclusions: A high rate of perioperative complications are recorded when tracked prospectively using standardized definitions. Adjusting complications for severity may identify areas for improving patient outcomes that are missed by simply recording their occurrence.

This report describes an infant presenting with deletion 22q11.2 in combination with left ventricular noncompaction and a coronary artery fistula. These two cardiac findings have rarely been reported in association with each other and have never been reported together in combination with deletion 22q11.2. The reported case demonstrates the expanding cardiac phenotype of individuals with deletion 22q11.2, suggesting that it may be appropriate to offer studies for the detection of deletion 22q11.2 to individuals with a wide range of structural cardiac defects.

Background The impact of the 2007 American Heart Association endocarditis prophylaxis guidelines on clinician practice has not been well established. Our objective was to evaluate how the American Heart Association endocarditis guidelines changed the practice of cardiologists who manage congenital heart disease and to ascertain the degree of practice variation among cardiologists. Methods A cross-sectional Web-based survey was e-mailed to Canadian (n = 134), Australian (n = 33), New Zealand (n = 9), and a random sample of American (n = 250) pediatric and adult congenital heart disease cardiologists in 2008. Nonrespondents received the survey 4 times by e-mail and once by regular post. Results The response rate was 55%. The lesions for which cardiologists were most evenly divided between recommending versus not recommending prophylaxis were “rheumatic mitral stenosis of moderate severity” (45% recommended prophylaxis) and “perimembranous ventricular septal defect (VSD) status post surgical patch closure with no residual shunt 3 months post-operatively” (54% recommended prophylaxis). The lesions for which the greatest proportion of cardiologists discontinued prophylaxis were “small muscular VSD, no previous endocarditis” (80% discontinued prophylaxis) and “small audible patent ductus arteriosus” (83% discontinued prophylaxis). Only 69% recommended prophylaxis for “VSD s/p surgical patch closure with small residual shunt” despite current guidelines recommending prophylaxis for this scenario. Twenty-eight percent of respondents felt that the new guidelines leave some patients at risk, and 6% would not recounsel any low-risk patients following these guidelines. Conclusions The 2007 guidelines have resulted in a substantial change in endocarditis prophylaxis. There remains considerable heterogeneity among cardiologists regarding the prophylaxis of certain cardiac lesions.

OBJECTIVE. In May 2006, Health Canada released a statement advising against the use of stimulant medications for patients with cardiac disease after isolated reports of sudden death. The objectives of this study were to determine whether the Health Canada advisory has had an impact on cardiovascular screening of all children with attention-deficit/hyperactivity disorder before stimulants and the medical treatment of children with attention-deficit/hyperactivity disorder and real or potential cardiac problems. METHODS. Two questionnaires were developed and mailed to a sample of noncardiologist physicians and pediatric cardiologists in Canada. The difference in the proportion of noncardiologist-responders who performed a “full screen” before and after the announcement of the Health Canada advisory was analyzed. Willingness to prescribe stimulant medications to children was assessed by using a series of clinical scenarios. RESULTS. A total of 2326 questionnaires were distributed, 717 (31%) of which were returned. The proportion who performed a full screen increased for both noncardiologists and cardiologists after the advisory. There was also a dramatic increase in the use of the “modified screen” by the noncardiologist group and the pediatrician subgroup. There was a considerable decrease in the proportion of noncardiologists who were willing to prescribe stimulant medications to children with potential or actual cardiac issues after the advisory. CONCLUSIONS. The Health Canada advisory has had an impact on clinical practice. It has resulted in an increase in the use of cardiovascular assessment tools before starting stimulants and a decrease in noncardiologists' willingness to prescribe stimulants to those with real or potential cardiac disease. These changes have occurred despite the lack of studies to address the cardiac risks of these medications. Because it will be difficult to assess the true cardiac risks of stimulants, consensus recommendations are needed to determine whether screening is required, what constitutes an appropriate screen, and which children can be treated cautiously with stimulant medications.

Kawasaki disease (KD) is an acute vasculitis that can result in cardiovascular sequelae. One of the most serious consequences of KD is the development of arterial aneurysms that can lead to thrombosis and ischemia. Commonly, these lesions, occurring early in the course of KD, are found in the coronary arteries. However, this location is not exclusive. This report presents a case of KD with atypical findings of a late-appearing brachiocephalic artery aneurysm. The authors suggest that for patients with complicated KD, surveillance should include a careful vascular survey to discover aneurysms distant from the heart.