Clinical utility of endomyocardial biopsies in the diagnosis of arrhythmogenic right ventricular cardiomyopathy in children.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Understanding, teaching and assessing the elements of the CanMEDS Professional Role: canadian program directors' views.

BACKGROUND: 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. OBJECTIVE: 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. METHODS: A two-step sequential mixed method design using a survey and semi-structured interviews with Canadian program directors. RESULTS: 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. CONCLUSION: 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.

Adherence to clinical practice guidelines for pulmonary valve intervention after tetralogy of Fallot repair: A nationwide cohort study.

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.

Patient-Reported Outcomes After Tetralogy of Fallot Repair.

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.

Some Things Change, Some Things Stay the Same: Trends in Canadian Education in Paediatric Cardiology and the Cardiac Sciences.

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.

La formation en cardiologie pédiatrique a évolué parallèlement aux soins cliniques. L'accès à de nouvelles technologies dans la formation a eu une incidence particulièrement marquée. L'intelligence artificielle, les outils d'apprentissage faisant appel aux réalités virtuelles, augmentées et mixtes, et les ressorts ludiques sont tout autant d'outils qui n'étaient pas à la disposition des médecins en formation dans le passé. Un nouveau modèle de formation est également en place dans certaines écoles de médecine, où l'approche par compétences n'est plus seulement réservée aux résidents. Cette méthode d'enseignement permet d'établir une relation plus transparente entre la formation et le contrat social qui lie les médecins et la société. Elle permet une meilleure responsabilisation, tant du côté des enseignants que des apprenants, en ce qui concerne les compétences nécessaires pour bien outiller les spécialistes. Toutefois, ce modèle s'accompagne de certains défis puisqu'il engage davantage de responsabilités pour les médecins en exercice et les établissements de formation. En dépit de ces changements, certains éléments sont tout aussi importants que par le passé, notamment la présence de bons enseignants cliniques pour assurer un apprentissage efficace. Malheureusement, les apprenants continuent de vivre de grandes difficultés dans notre système de formation, et il s'agit peut-être du plus grand défi auquel font face les programmes canadiens de formation médicale. Apprendre à devenir de meilleurs enseignants et de meilleurs défenseurs des apprenants est un objectif important pour toutes les personnes qui s'emploient à former de nouveaux médecins au Canada.

Using Smartphone Wireless ECG Monitoring to Provide Symptom-Rhythm Correlation in the Paediatric Population.

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.

Les enfants présentent fréquemment des symptômes de palpitations. Ces symptômes peuvent se produire isolément ou en association avec d'autres manifestations comme la douleur thoracique, la présyncope ou la syncope. Habituellement, les symptômes sont épisodiques, d'où la difficulté d'établir une corrélation entre les symptômes et le rythme cardiaque, un exercice pourtant essentiel pour poser un diagnostic précis et assurer la prise en charge du patient. Nous avons passé en revue les données relatives à la surveillance électrocardiographique sans fil effectuée au moyen d'un téléphone intelligent via la technologie Kardia d'AliveCor afin d'établir une telle corrélation dans une série de cas pédiatriques issue d'un même centre de soins tertiaires canadien.

The impact of 2007 infective endocarditis prophylaxis guidelines on the practice of congenital heart disease specialists.

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.

Dexamethasone given to premature infants and cardiac diastolic function in early childhood.

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.

Left ventricular noncompaction and coronary artery fistula in an infant with deletion 22q11.2.

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.

Benefits of an international working exchange in pediatric cardiology.

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.

Follow-up of tetralogy of Fallot patients: tertiary centre versus satellite clinic.

BACKGROUND: Canadian 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. METHODS: We 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. RESULTS: Of 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. CONCLUSION: Tetralogy of Fallot patients followed at either satellite or tertiary clinics have similar subjective health status.

Practice Variation Among Canadian Pediatric Cardiologists in Medical Management of Dilated Ascending Aorta in Patients With Bicuspid Aortic Valve.

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.

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.

A Risk Management Strategy for Managing Critical Human Resource Changes in a Pediatric Heart Program.

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.

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.

Practices and Attitudes of Canadian Cardiologists Caring for Patients With Trisomy 18.

Trisomy 18 (T18) is a genetic disorder with cardiac lesions in up to 90% of patients. Cardiac surgery is not frequently offered because of the overall poor prognosis, although this has recently been challenged. Our study aimed to explore the practices and attitudes of Canadian pediatric cardiologists managing T18 patients. We administered a survey to pediatric cardiologists attending the Canadian Cardiovascular Congress, Canadian Pediatric Cardiology Association Business Meeting. There were 30 respondents. Most (67%) supported comfort care for affected patients with a heart lesion. None supported palliative surgery for those with complex heart lesions. Of 30 respondents, 16 (53%) counsel families prenatally, and none would present the option of a single ventricle surgical track for complex heart disease. In a hypothetical situation in which their own child was born with T18, 67% would choose comfort care with medical treatment of heart failure, and none would choose palliative surgery. Being a parent was associated with a higher likelihood of choosing termination (14 of 20 vs 6 of 9; P = 0.046) or comfort care (14 of 20 vs 6 of 9; P = 0.036). Qualitative data suggest support for comfort care, while recognizing the need for individualization and shared decision-making, within the context of institution-specific policies. Canadian pediatric cardiologists surveyed support comfort care and medical treatment but not surgical treatment for T18 patients with cardiac lesions. They place primacy on nonmaleficence, yet also recognize the emerging need for individualized shared decision-making in these cases.

Using the Readiness for Clerkship and Residency Surveys to Evaluate the Effectiveness of Four MD Programs: A Cross-Institutional Generalizability Study.

PURPOSE: 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. METHOD: 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. RESULTS: 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. CONCLUSIONS: 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.

Paediatricians' validation of learning objectives in paediatric cardiology.

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.

Trends in pediatric cardiology referrals, testing, and satisfaction at a Canadian tertiary centre.

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.

Human leukocyte antigen-DR and ABO mismatch are associated with accelerated homograft valve failure in children: implications for therapeutic interventions.

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 >or=2 grades or >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.

The Ross procedure for endocarditis in a 4-month-old infant.

Streptococcal endocarditis in an infant is rare. We report a case of acute aortic valve endocarditis with abscess and aorta-to-right atrial fistula formation. This 4-month-old infant with a structurally normal heart had been previously well. The child was successfully treated with the Ross procedure and remains well 13 months postoperatively.