Background Identification of individuals at risk for major adverse cardiovascular events is essential for contemporary management of patients with repaired tetralogy of Fallot. We sought to identify clinical and cardiovascular magnetic resonance imaging (CMR) predictors of adverse clinical outcomes in repaired tetralogy of Fallot. Methods Children and adults prospectively enrolled in the CORRELATE (Comprehensive Outcomes Registry Late After Tetralogy of Fallot Repair) registry followed in North American, European, and Asian centers were studied. All patients had at least moderate pulmonary regurgitation and CMR at enrollment. Time‐to‐event analyses were performed from CMR completion to primary outcome, defined as mortality, resuscitated sudden death, sustained ventricular arrhythmia, or heart failure admission. Principal component analysis was used to create distinct CMR scores that collectively captured 80% of the variance among 10 CMR measures (systolic function, biventricular volumes/mass, and biatrial areas). Results In 720 patients (55% male, median age 30.3±14 years, 78% adult) with mean follow‐up 5.7±1.8 years, the primary outcome occurred in 38 patients (5.2%) at a rate of 0.9/100 patient‐years. A well‐calibrated risk scoring system was created for prediction of the primary outcome at 5 years based on 5 predictors: age, diabetes, right ventricular systolic pressure, and 2 CMR principal component scores (predominantly reflecting atrial areas in the first principal component score and ventricular volumes in the second principal component score) (c‐statistic for the composite risk score 0.79 [95% Cl, 0.71–0.88]). Conclusions Clinical and imaging characteristics can contribute to risk prediction in repaired tetralogy of Fallot. Further study will be required to evaluate the utility of a risk scoring system for identification of individuals who may benefit from enhanced surveillance, intensified medical therapy, and/or optimally timed intervention.

Reflective writing (RW) is a popular tool in medical education, but it is being used in ways that fail to maximize its potential. Literature in the field focuses on why RW is used - that is to develop, assess, and remediate learner competencies - but less so on how to use it effectively. The emerging literature on how to integrate RW in medical education is haphazard, scattered and, at times, reductionist. We need a synthesis to translate this literature into cohesive strategies for medical educators using RW in a variety of contexts. These 12 tips offer guidelines for the principles and practices of using RW in medical education. This synthesis aims to support more strategic and meaningful integration of RW in medical education.

BACKGROUND Comprehensive assessment of tetralogy of Fallot (TOF) outcomes extends beyond morbidity and mortality to incorporate patient-reported outcomes (PROs), including quality of life (QOL) and health status (HS).OBJECTIVES This study explored PROs in adolescents and adults with TOF and delineated variables associated with PROs. METHODS This was a cross-sectional observational study within a larger prospective registry of adolescents and adults with repaired TOF and moderate or greater pulmonary regurgitation from North America, Europe, and Asia. Participants completed PROs, including a QOL linear analogue scale (QOL-LAS) and an HS visual analogue scale (HS-VAS). Scores were classified according to age cohorts: <18, 18 to 25, 26 to 40, and >40 years.RESULTS The study included 607 patients (46.3% female; median age 28.5 years). Median QOL-LAS scores (0-100) were similar across age cohorts (85, 80, 80, 80; P = 0.056). Median HS-VAS scores (0-100) were lowest for the oldest cohort (77) compared with the 3 younger cohorts (85, 80, 80) (P = 0.004). With advancing age, there were increased reports of poor mobility (P < 0.001) and pain or discomfort (P = 0.004); problems in these dimensions were reported by 19.1% and 37.2% of patients aged >40 years, respectively. Of factors associated with superior PROs on multivariable regression modeling (ie, being White, being nonsyndromic, having employment, and having better left ventricular function; P < 0.05), asymptomatic status (functional class I) was the variable associated with the greatest number of QOL and HS measures (P < 0.001). CONCLUSIONS Strategies to improve TOF outcomes should consider PROs alongside conventional clinical variables. Factors associated with poorer PROs represent opportunities to intervene to improve the lives of patients with TOF. (J Am Coll Cardiol 2023;81:1937-1950) (c) 2023 by the American College of Cardiology Foundation.

Abstract Background Kawasaki Disease (KD) is the leading cause of acquired heart disease in children in developed countries with a variable incidence worldwide. Previous studies reported an unexpectedly high incidence of KD in the Canadian Atlantic Provinces. The goals of our study were to validate this finding in the province of Nova Scotia and to carefully review patients’ characteristics and disease outcomes. Methods This was a retrospective review of all children < 16 years old from Nova Scotia diagnosed with KD between 2007–2018. Cases were identified using a combination of administrative and clinical databases. Clinical information was collected retrospectively by health record review using a standardized form. Results Between 2007–2018, 220 patients were diagnosed with KD; 61.4% and 23.2% met the criteria for complete and incomplete disease, respectively. The annual incidence was 29.6 per 100,000 children < 5 years. The male to female ratio was 1.3:1 and the median age was 3.6 years. All patients diagnosed with KD in the acute phase received intravenous immunoglobulin (IVIG); 23 (12%) were refractory to the first dose. Coronary artery aneurysms were found in 13 (6%) patients and one patient died with multiple giant aneurysms. Conclusion We have confirmed an incidence of KD in our population which is higher than that reported in Europe and other regions of North America despite our small Asian population. The comprehensive method to capture patients may have contributed to the detection of the higher incidence. The role of local environmental and genetic factors also deserves further study. Increased attention to regional differences in the epidemiology of KD may improve our understanding of this important childhood vasculitis.

Objectives: To determine guideline adherence pertaining to pulmonary valve replacement (PVR) referral after tetralogy of Fallot (TOF) repair. Methods: Children and adults with cardiovascular magnetic resonance imaging scans and at least moderate pulmonary regurgitation were prospectively enrolled in the Comprehensive Outcomes Registry Late After TOF Repair (CORRELATE). Individuals with previous PVR were excluded. Patients were classified according to presence (+) versus absence (-) of PVR and presence (+) versus absence (-) of contemporaneous guideline satisfaction. A validated score (specific activity scale [SAS]) classified adult symptom status. Results: In total, 498 participants (57% male, mean age 32 +/- 14 years) were enrolled from 14 Canadian centers (2013-2020). Mean follow-up was 3.8 +/- 1.8 years. Guideline criteria for PVR were satisfied for the majority (n = 422/498, 85%), although referral for PVR occurred only in a minority (n = 167/498, 34%). At PVR referral, most were asymptomatic (75% in SAS class 1). One participant (0.6%) received PVR without meeting criteria (PVR+/indication-). The remainder (n = 75/498, 15%) did not meet criteria for and did not receive PVR (PVR-/indication-). Abnormal cardiovascular imaging was the most commonly cited indication for PVR (n = 61/123, 50%). The SAS class and ratio of right to left end-diastolic volumes were independent predictors of PVR in a multivariable analysis (hazard ratio, 3.33; 95% confidence interval, 1.92-5.8, P < .0001; hazard ratio, 2.78; 95% confidence interval, 2.18-3.55, P < .0001). Conclusions: Although a majority of patients met guideline criteria for PVR, only a minority were referred for intervention. Abnormal cardiovascular imaging was the most common indication for referral. Further research will be necessary to establish the longer-term clinical impact of varying PVR referral strategies.

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.

Pulmonary embolism in the neonate is a rare, life-threatening emergency. Risk factors for neonatal pulmonary embolism (PE) include sepsis, asphyxia, prematurity, and vascular catheterisation. We report the case of a preterm neonate with a massive saddle pulmonary thrombosis of unidentified etiology. Prompt diagnosis by cardiology allowed an emergent lifesaving open surgical thrombectomy, underscoring the importance of efficient multidisciplinary teamwork. Pediatric health-care professionals must be aware of this rare entity when initial oxygen desaturation management fails, even when obvious risk factors for PE are not apparent. We emphasise the importance of seamless multidisciplinary communication and proactive surgical consultation.

Umbilical cord milking improves postnatal adaptation and short-term outcomes of very preterm infants compared to early cord clamping. Little is known about the impact of umbilical cord milking on long-term neurodevelopmental outcomes. The objective of this study is to compare the effects of intact umbilical cord milking (UCM) vs. early cord clamping (ECC) at birth on neurodevelopmental outcomes at 36 months’ corrected age. Preterm infants < 31 weeks’ gestation who were randomized at birth to receive three time milking of their attached cord or ECC (< 10 s) were evaluated at 36 months’ corrected age. Neurodevelopmental outcomes were assessed by blinded examiners using Bayley Scales of Infant and Toddler Development (version III). Analysis was by intention to treat. Out of the 73 infants included in the original trial, 2 died and 65 (92

Education in paediatric cardiology has evolved along with clinical care. The availability and application of new technologies in education, in particular, have had a significant impact. Artificial intelligence; virtual, augmented, and mixed reality learning tools; and gamification of learning have all resulted in new opportunities for today's trainees compared with those of the past. A new training model is also being used. Though currently focused on residency education, competency-based medical education is also being applied to undergraduate education in some Canadian medical schools. Competency-based medical education offers a more transparent relationship between education and physicians' social contract with society. It provides greater accountability for programmes and learners to teach and learn the skills required to function as competent specialists. However, it has not come without challenges. Coincident with the application of this model for learners, there has been increased educational accountability for physicians in practice and for the institutions training them. Despite these changes, some things have remained the same. On the positive side, the importance of good clinical teachers to effective learning remains constant. Unfortunately, the mistreatment of learners within our education system also remains and is perhaps the most important challenge facing medical education in Canada today. Learning to be better teachers and learner advocates is an important goal for all of those involved in educating Canadian medical learners.

Children frequently present with symptoms of palpitations. These symptoms can occur in isolation or in association with other presentations such as chest pain, presyncope, or syncope. Usually, their symptoms are episodic in occurrence; therefore, establishment of symptom-rhythm correlation is challenging but critical for accurate diagnosis and management. We reviewed the use of smartphone-based wireless electrocardiogram monitoring with AliveCor Kardia, to establish symptom-rhythm correlation in a paediatric case series at a single Canadian tertiary care centre.

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.

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 cardi-ologists (46%) and 18 cardiologists (44%) reported using Holter monitoring and exercise testing, respectively, to assess beta receptor blockade adequacy. Thirteen cardiologists (32%) considered BB ther-apy 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 ade-quacy 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.

Objective To compare the effect of umbilical cord milking (UCM) vs. early cord clamping (ECC) on cerebral blood flow (CBF). Method Preterm infants <31 weeks’ gestation were randomized to receive UCM or ECC at birth. Blood flow velocities and resistive & pulsatility indices of middle and anterior cerebral arteries were measured at 4–6 and 10–12 h after birth as an estimate of CBF. Results Randomization allocated 37 infants to UCM and 36 to ECC. Maternal and antenatal variables were similar. There were no significant differences between groups in middle or anterior CBF velocities and resistive indices at either study time point. CBF variables were not correlated with mean blood pressure, systemic blood flow, or intraventricular hemorrhage. Conclusions In very preterm infants, UCM compared with ECC was not shown to change CBF indices during the first 12 h of age or correlate with other hemodynamic measures or with intraventricular hemorrhage. Trial registration ClinicalTrials.gov: NCT01487187.

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.

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.

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.