Background:Transitioning from paediatric to adult congenital heart disease (CHD) care is a high-risk time for being lost to follow-up. Existing CHD transition programmes have not included patients, caregivers, and health care providers as partners in their development. This study aimed to develop recommendations for a CHD transition programme driven by lived and clinical experiences. Methods:We used a multilevel participatory process that engaged adult and paediatric people living with CHD, their caregivers, and CHD health care providers as members of the research team. We also consulted members of these stakeholder groups through a series of 3 virtual workshops that culminated in the generation of recommendations for the essential components of a CHD transition programme. Results:The Transition Essentials recommendations inform what information, education, or support is required, who should provide it, and when and how it should be provided. Information, education, and support for self-management and knowledge are required for people living with CHD. Caregivers require information, education, and support to build capacity in people living with CHD and navigate their new role in their loved ones' life. The health care team should provide this information, education, and support with peer support options when people living with CHD are 15-22 years of age. This information, education, and support should be individualized, navigate limitations, build over time, have multimodal options, and be available virtually or in person. Conclusions:Engaging those with lived and clinical expertise to develop recommendations for the essential components of a CHD transition programme provides important insights missing from previous studies.

Less than 2% of cases of isolated TAPVR are diagnosed prenatally. The objective of this study was to develop a diagnostic algorithm from routine obstetrical ultrasound images at mid-pregnancy to improve prenatal diagnosis of TAPVR. This was a matched case-control study at a tertiary-care center (2005-2019). Cases of TAPVR were identified from a pediatric cardiology clinic repository, and controls matched 4:1 by gestational age, calendar date, and sex. Post-processing review of stored fetal ultrasound images was performed by 2 blinded and independent observers in a standardized fashion evaluating 9 parameters: left/right heart disproportion, abnormal distribution of great vessels, pulmonary vein entry into left atrium (LA), confluence behind LA, abnormal coronary sinus, absence of coumadin ridge, aortic diameter, distance between LA and aorta, and post-LA space index >1.27. Diagnostic algorithms were developed, and sensitivity/specificity calculated to evaluate performance. Inter-observer reliability was determined using a Bland-Altman plot. From the 105 pregnancies in the study, the most common ultrasound abnormality amongst cases of isolated TAPVR (n=21) was absence of pulmonary vein entry into the LA (42.9%). Using a diagnostic algorithm with decision-branching stratifying on absence of pulmonary veins, followed by absence of the Coumadin ridge, cases of TAPVR could be correctly identified prenatally with high specificity (90.5%; 95% CI 82.1-95.8%) and sensitivity of 61.9% (95% CI=38.4%-81.9%). If a diagnostic algorithm was developed using presence of any 3 abnormal markers of 7 dichotomous options, the specificity improved to 94.1% (95% CI=86.7-98.1%) but the sensitivity was poorer at 23.8% (95% CI=8.2-47.2%). Standardized diagnostic approaches can be highly specific for prenatal detection of TAPVR, but screening algorithms that are sufficiently sensitive for screening in the general population are still needed.

BackgroundCongenital heart disease is the most common congenital birth defect and presents with differing degrees of complexity. Patients require lifelong specialized care. The transfer from paediatric to adult care is a time of risk that may result in lapses or loss of care. A successful transfer from paediatric to adult care is integral for improved patient outcomes.MethodsIn this retrospective study, we used the paediatric cardiology database and the electronic records at the adult congenital heart disease (ACHD) clinic to identify referrals and successful transfer between 2008 and 2017. Successful transfer was defined as a patient referred to the ACHD clinic who was seen in the clinic and has ongoing follow-up. We also sought to identify predictors of a successful transfer.ResultsA total of 555 patients were referred to the ACHD clinic (2008-2017). Of all patients referred, 62% had a successful transfer and an ongoing specialist care. The remaining 38% either did not show for first appointments or missed 3 consecutive visits. Independent predictors of a successful transfer were the presence of moderate or complex ACHD, residing within the city limits, older age at the time of referral, and a more recent year of referral.ConclusionsOver one-third of patients did not achieve successful transfer, namely attendance at first clinic visit plus early retention in care. We were able to identify several variables that predict successful transfer. Further research is required to identify interventions that can be implemented to reduce lapses in patient care.

Objectives. To determine the most common fetal ultrasound markers of total anomalous pulmonary venous return (TAPVR) during mid-trimester ultrasound using standardly obtained images and evaluate the performance of diagnostic algorithms for improving prenatal diagnosis. Methods. This was a matched case-control study at a regional referral centre (2005 to 2019). Cases of TAPVR were matched to controls 1 : 4 by date of birth and biologic sex. Postprocessing review of stored fetal ultrasound images was performed by two blinded and independent observers in a standardized fashion using nine sonographic markers: (i) left/right heart disproportion; (ii) abnormal distribution of great vessels; (iii) pulmonary vein entry into the left atrium (LA); (iv) confluence behind the LA; (v) abnormal coronary sinus; (vi) absence of the Coumadin ridge; (vii) aortic diameter; (viii) distance between LA and aorta; and (ix) post-LA space index >1.27. Descriptive and inferential statistics were used to present results and compare cases and controls. Diagnostic algorithms were compared by sensitivity/specificity. Results. 21 cases of isolated TAPVR were matched to 84 controls (n = 105). The most common ultrasound marker of TAPVR was absence of pulmonary vein entry into the LA (42.9%), followed by abnormal Coumadin ridge (38.1%). Cases of TAPVR had significantly larger post-LA spaces than controls ( p < 0.0001 ) and wider aortic diameters ( p = 0.006 ). A diagnostic algorithm stratifying on absence of pulmonary veins followed by an abnormal Coumadin ridge, can correctly identify cases of TAPVR with high specificity (90.5%) and moderate sensitivity (61.9%). Conversely, a diagnostic algorithm using the presence of any 3 abnormal markers had improved specificity (94.1%) but poorer sensitivity (23.8%). Conclusions. Using standardly obtained images from routine fetal ultrasound, improved prenatal detection of isolated TAPVR is possible. A standardized diagnostic approach can be highly specific for fetal TAPVR, however, algorithms that are sufficiently sensitive for screening in the general population are still needed.

Background: The 2014–2016 Ebola outbreak in West Africa recapitulated that nosocomial spread of Ebola virus could occur and that health care workers were at particular risk including notable cases in Europe and North America. These instances highlighted the need for centers to better prepare for potential Ebola virus cases; including understanding how the virus spreads and which interventions pose the greatest risk. Methods: We created a fully equipped intensive care unit (ICU), within a Biosafety Level 4 (BSL4) laboratory, and infected multiple sedated non-human primates (NHPs) with Ebola virus. While providing bedside care, we sampled blood, urine, and gastric residuals; as well as buccal, ocular, nasal, rectal, and skin swabs, to assess the risks associated with routine care. We also assessed the physical environment at end-point. Results: Although viral RNA was detectable in blood as early as three days post-infection, it was not detectable in the urine, gastric fluid, or swabs until late-stage disease. While droplet spread and fomite contamination were present on a few of the surfaces that were routinely touched while providing care in the ICU for the infected animal, these may have been abrogated through good routine hygiene practices. Conclusions: Overall this study has helped further our understanding of which procedures may pose the highest risk to healthcare providers and provides temporal evidence of this over the clinical course of disease.

We present the case of a 16-year-old patient with anomalous left coronary artery from the left pulmonary artery requiring percutaneous coronary intervention in infancy who presented with ventricular fibrillation arrest. A coronary angiogram revealed 40% narrowing of the stent relative to the remainder of the left main coronary artery. Optical coherence tomography was performed and revealed an area stenosis of 70% relative to the native left main coronary artery. The patient had outgrown the stent. (Level of Difficulty: Advanced.)

Objective To evaluate the impact of a targeted neonatal echocardiography (TNE) service on patient management in the neonatal intensive care units (NICU) in Winnipeg, Canada. Study design Retrospective cohort study of neonates who had TNEs from 2014 to 2019. The primary outcome was the change in clinical management based on TNE recommendation. Multivariate logistic regression analysis was used to identify predictors of the primary outcome. Results A total of 747 echos were performed on 307 neonates. Patent ductus arteriosus assessment was the most common indication for TNE followed by evaluation of pulmonary hypertension and systemic hemodynamics. TNE led to a change in clinical management following 492 (66%) echos. Mechanical ventilation [Odds ratio (OR) 2.4, 95% CI: 1.7–3.4, P < 0.001) and receiving inhaled nitric oxide (1.9, 95% CI: 1.3−3.0, P = 0.003) were the predictors for the change in clinical management following TNE. Conclusion TNE has enhanced patient care by altering the management of patients in the NICU.

Please note that four authors (Logan Banadyga, Alixandra Albietz, Brad Pickering, and Gary Wong) have been erroneously omitted from the author list in the published original article [1].

Introduction: There are currently no licensed treatments for Ebola Virus Disease, making supportive care the only viable option. While there is anecdotal evidence that this is effective, the paucity of experimental data to demonstrate the role of supportive care in EVD has limited its use. We hypothesized that supportive care would enhance survival in a non-human primate (NHP) model of EVD.

BACKGROUND: Infants with heterotaxy syndrome (HS) have abnormal lateralization of organs along the right-left body axis. Intestinal rotation abnormalities (IRAs) are a potential source of morbidity and mortality. For this study, our objective was to prospectively observe a cohort of infants with HS and determine the incidence and natural history of IRA. METHODS: Infants <= 6 months of age with HS were enrolled in this prospective observational study. Exclusion criteria were other congenital abnormalities that necessitated abdominal surgery. HS was defined as any arrangement of organs that was not situs solitus or situs inversus along with associated congenital heart disease. The investigation for IRA was at the discretion of each participating center. RESULTS: Infants were recruited from January 2012 to December 2016. Thirty-eight infants from 7 institutions were included; 22 infants had right isomerism and 16 infants had left isomerism. Twenty-nine infants (76%) were evaluated for IRAs; 21 of 29 evaluations (72%) were abnormal. Eight infants were investigated because of symptoms, and 21 infants were evaluated routinely. The median age at symptom presentation was 46 days (range: 5-171 days). Seven infants had a Ladd procedure; 4 were prophylactic, with 3 as part of a combined procedure, and 3 were emergent. No child suffered acute midgut volvulus over a median follow-up of 1.6 years (range: 0.06-4.93 years). CONCLUSIONS: IRAs are common in infants with HS. Infants with symptoms presented by 6 months of age. There was no failure of expectant management resulting in midgut volvulus during a median follow-up of 1.6 years.

ObjectivesPrenatal diagnosis of complex congenital heart disease (CHD) during routine obstetric ultrasound (US) examinations improves postnatal outcomes, but sensitivity is low (<40%). Our objective was to improve our prenatal detection of complex CHD with implementation of a specific screening protocol.MethodsFrom January 2003 to December 2013, 506 consecutive confirmed cases of complex CHD in the province of Manitoba, Canada, were analyzed to compare the sensitivity and positive predictive value of prenatal US detection of complex CHD before and after the introduction of a novel prenatal screening protocol. The intervention was done in October 2004, emphasizing screening and not diagnosis of complex CHD. It involved education, practical scanning tips, a checklist, and feedback on cases. We also assessed the effect of the intervention in different screening settings: community hospitals, tertiary hospitals, and fetal assessment units.ResultsThe sensitivity for detecting complex CHD increased from 29.8% to 88.3% (P < .0001), while the positive predictive value remained high. The largest improvement in detection was found for US units in community hospitals (52.4% higher; P < .0001), followed by tertiary hospitals (39.9%; P = .0004), and fetal assessment units (7.2%; P = .16). Additionally, there was a significant decrease in the presentation of neonates in critical condition from before to after the implementation (24.3% to 13.1%, respectively; P = .0165).ConclusionsImplementing a focused routine prenatal screening protocol can vastly improve detection rates of critical cardiac abnormalities. The improvement in community hospitals was particularly important because early detection ensured that the birth was planned to take place in an appropriate facility. Our novel protocol can be performed by all sonographers, regardless of experience, equipment used, and hospital setting.

Objectives: Our objective was to compare patent ductus arteriosus (PDA) diameter, PDA score and brain-type natriuretic peptide (BNP) measurements at 48-72 hours of life, for prediction of neonatal morbidities. We hypothesized that use of a PDA score with BNP, may improve pre-symptomatic prediction of PDAs associated with adverse outcomes. Method: Infants < 31 weeks GA were prospectively studied by targeted neonatal echocardiogram (TNE) at 48-72 hours age, composite PDA score and serum BNP assay; the clinical team remained blinded. PDA was independently diagnosed by echocardiography at time of clinical suspicion (6 ± 2 days), and treated at discretion of the clinical team. Primary outcome was survival with one or more of adverse outcomes (intraventricular hemorrhage [IVH], bronchopulmonary dysplasia [BPD], retinopathy of prematurity [ROP], necrotizing enterocolitis [NEC]). Results: A PDA was present in 56 of 70 infants studied at 48-72 hours; 30 were eventually diagnosed with PDA but never required treatment, 19 required medical treatment, 7 surgical ligation. After adjustment for gestation, PDA diameter did not predict any adverse outcome, PDA score was associated with increased risk of any adverse outcome and high BNP was associated with IVH, BPD, or survival with any adverse outcome. Conclusions: Comprehensive PDA evaluation at 48-72 hours of age may predict the subsequent occurrence of adverse outcomes and may be useful to define the PDA treatment threshold.

Objective: The purpose of this study is to determine the incidence of persistent coronary artery sequelae due to KD between the First Nation/Metis (FN/M) and non First Nation children (n-FN) in the province of Manitoba and Northwestern Ontario, Canada. Methods: This is a 10-year (2000-2010) retrospective chart review study of all patients < 18 years of age identified with KD and persistent coronary artery aneurysms. The data was obtained from the Children’s Hospital of Winnipeg medical records and the Electronic Echocardiography data based at the Variety Heart Centre. Results: A total of 103 children and adolescents with KD and with at least 3 echocardiography studies were enrolled. Complete or incomplete presentation of KD was determined according to the fulfillment for the disease classic criteria on diagnosis. Age at diagnosis ranged between 2 months to 4 years (median 0.5 years) with 2 patients diagnosed during their early infancy (age< 90 days). The median follow up period was 4 years (1.3 to 6.3 years). Persistent coronary artery aneurysms were detected in 10 children (M/F ratio 3:1). Nine (9) of the studied subjects account for the FN/M children group and the remaining 1 is of Asian origin. We observed no n-FN children with permanent cardiac sequelae secondary to KD. Conclusion: There is a significantly higher occurrence of permanent coronary artery lesions due to KD among the FN/M children in the province of Manitoba and Northwestern Ontario. However, this study does not imply direct association of any risk factor, which might be related with our findings. Socio-environmental or even genetic factors might contribute to these findings. Nevertheless, these speculations are beyond the scope of this study. We suggest that a strategy of early, diagnosis and treatment of KD based on the latest AHA guidelines, when we deal with patients of these ethnic/racial groups, should be implemented.

Right sided Congenital Diaphragmatic Hernia (CDH) has been previously associated with venous or lymphatic obstruction of vessels by the herniated liver. We report an unusual case of direct cardiac atrial compression by the liver causing limitation of preload and resultant fetal non-immune hydrops fetalis.

Prenatal diagnosis of congenital heart disease (CHD) during routine obstetric sonography has been aptly named the sonographer’s Achilles heel. Although CHD occurs more commonly than any other major congenital abnormality, the detection rate remains low. The goal of this study was to improve the prenatal diagnosis of CHD during routine obstetric sonography through the development and implementation of a simple and effective screening protocol.

Pediatric heart failure (HF) is an important cause of morbidity and mortality in childhood. This article presents guidelines for the recognition, diagnosis, and early medical management of HF in infancy, childhood, and adolescence. The guidelines are intended to assist practitioners in office-based or emergency room practice, who encounter children with undiagnosed heart disease and symptoms of possible HF, rather than those who have already received surgical palliation. The guidelines have been developed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology, and are accompanied by practical Recommendations for their application in the clinical setting, supplemented by online material. This work does not include Recommendations for advanced management involving ventricular assist devices, or other device therapies.

BACKGROUND:Pediatric extracorporeal membrane oxygenation (ECMO) programs are sophisticated endeavors usually found only in high-volume cardiac surgical programs. Worldwide, many cardiology programs do not have on-site pediatric cardiac surgery expertise. Our single-center experience shows that an organized multidisciplinary rescue-ECMO program, in collaboration with an accepting facility, can achieve survival rates comparable to modern era on-site ECMO.METHODS:A retrospective review was conducted of all patients initiated on rescue-ECMO from 2004 to 2009 in a single academic pediatric hospital without a pediatric cardiac surgery program. All aspects of ECMO were formalized using Failure Mode Effects Analysis.RESULTS:Eight patients were initially cannulated for ECMO at our institution. Six were subsequently transported by air to the receiving facility 1,305 km away. Extracorporeal membrane oxygenation was initiated in 0.2% of our Pediatric Intensive Care Unit admissions and in 0.52% of all our pediatric cardiac patients. Mean age was 4.0 years (7 weeks to 15 years). Indications for ECMO initiations were cardiogenic shock (n = 5) and acute respiratory distress syndrome (n = 3). Six had veno-arterial- and two had veno-veno ECMO. Two patients were not transported (one death and one weaned locally). Six patients were successfully transported within 2 to 24 hours, with a survival to hospital discharge rate of 67% (four of six). Median total time on ECMO was 5.5 days. Complication rate was 50% (4/8).CONCLUSIONS:Our rescue-ECMO survival results were comparable to that of current published results from established pediatric ECMO programs. Air transport of ECMO patients can be performed safely using an organized multidisciplinary team approach.

Newly emerging health technologies are being developed to care for children with complex cardiac defects. Neurodevelopmental and childhood school-related outcomes are of great interest to parents of children receiving this care, care providers, and healthcare administrators. Since the 1970s, neonatal follow-up clinics have provided service, audit, and research for preterm infants as care for these at-risk children evolved. We have chosen to present for this issue the mechanism for longitudinal follow-up of survivors that we have developed for western Canada patterned after neonatal follow-up. Our program provides registration for young children receiving complex cardiac surgery, heart transplantation, ventricular assist device support, and extracorporeal life support among others. The program includes multidisciplinary assessments with appropriate neurodevelopmental intervention, active quality improvement evaluations, and outcomes research. Through this mechanism, consistently high (96%) follow-up over two years is maintained.