Advancing Wearable Biosensors for Congenital Heart Disease: Patient and Clinician Perspectives: A Science Advisory From the American Heart Association.

Wearable biosensors (wearables) enable continual, noninvasive physiologic and behavioral monitoring at home for those with pediatric or congenital heart disease. Wearables allow patients to access their personal data and monitor their health. Despite substantial technologic advances in recent years, issues with hardware design, data analysis, and integration into the clinical workflow prevent wearables from reaching their potential in high-risk congenital heart disease populations. This science advisory reviews the use of wearables in patients with congenital heart disease, how to improve these technologies for clinicians and patients, and ethical and regulatory considerations. Challenges related to the use of wearables are common to every clinical setting, but specific topics for consideration in congenital heart disease are highlighted.

Evaluation of conventional troponin I testing for the detection of myocardial dysfunction in children.

Objectives: Troponin is a marker of myocardial injury but is not well studied in children. Our primary objective was to ascertain the sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of conventional troponin I for the detection of acute myocardial dysfunction in previously healthy children. Our secondary objective was to identify clinical predictors of myocardial dysfunction in the setting of elevated troponin. Study Design: This was a retrospective chart review in a single, paediatric, tertiary care centre of troponin tests performed in all admitted children over a 4-year period. Demographics, symptoms, signs, chest x-ray, ECG, and echocardiogram abnormalities were documented. Myocardial dysfunction was presumed to be absent when the patient had a normal cardiac assessment, with or without echocardiography, and did not re-present. Results: From January 2014 through December 2017, 566 patients had troponin tested as a screen for myocardial injury. Troponin was positive in 38 of 566 cases (6.7%). Myocardial dysfunction was detected in 9 of 566 cases (1.6%). Troponin was elevated in six of nine cases of myocardial dysfunction. The sensitivity of conventional troponin I for detecting acute myocardial dysfunction was 66% (95% confidence interval [CI] 30 to 93%). The specificity was 94% (95% CI 92 to 96%). PPV was 16% (95% CI 6 to 31%) and NPV 99% (95% CI 98 to 100%). An abnormal ECG was more prevalent in patients with a true positive versus a false-positive troponin result (P=0.03). Conclusion: Troponin testing identified few cases of myocardial dysfunction. We found the test to have only 66% sensitivity. Troponin testing as a screen for myocardial injury in children has limited utility.

Ambulatory blood pressure and carotid intima media thickness in children with type 1 diabetes.

BACKGROUND/OBJECTIVE: Blood pressure abnormalities may play an important role in macrovascular damage in type 1 diabetes. Little is known about blood pressure abnormalities and macrovascular damage in children with type 1 diabetes. METHODS: Children with type 1 diabetes (n = 57) for a short (3 months-2 years; n = 24) or long duration (≥5 years; n = 33) and a group of control children without diabetes (n = 29) completed 24-h ambulatory blood pressure monitoring (ABPM). Carotid intima media thickness (cIMT), a subclinical indicator of atherosclerosis, was assessed by carotid ultrasound. RESULTS: ABPM abnormalities were more prevalent (57% vs 24%, respectively), and daytime, nighttime and 24-h systolic, diastolic, and mean arterial blood pressure indices were higher in children with type 1 diabetes compared to control children. The odds estimate of an ABPM abnormality was 6.68 (95% confidence interval: 1.95, 22.9; P = .003) in children with type 1 diabetes compared to controls after adjusting for age, sex, and BMI standardized for age and sex (zBMI). An interaction between ABPM and zBMI on cIMT was observed. In children with type 1 diabetes and ABPM abnormalities, every 1 SD increase in zBMI was associated with a 0.030 mm increase in cIMT (95% confidence interval: 0.002, 0.041; P = .031). This was not observed in control children with ABPM abnormalities or in children with normal ABPM, regardless of type 1 diabetes status. CONCLUSIONS: Children with type 1 diabetes have a high prevalence of ABPM abnormalities independent of disease duration and this is related to early indicators of cardiovascular damage.

The relative incidence of cardiogenic and septic shock in neonates.

OBJECTIVE: To evaluate the relative incidence of cardiogenic and septic shock in term neonates and identify findings that help differentiate the two entities. STUDY DESIGN: We conducted a retrospective chart review of term neonates presenting to British Columbia Children's Hospital (BCCH) with decompensated shock of an undiagnosed etiology between January 1, 2008 and January 1, 2013. Charts were reviewed to determine the underlying diagnoses of all neonates meeting our inclusion criteria. Patients were categorized as having septic, cardiogenic, or other etiologies of shock. We then evaluated potential demographic, clinical, and biochemical parameters that could help differentiate between septic and cardiogenic shock. RESULTS: Cardiogenic shock was more common than septic shock (relative risk=1.53). A history of cyanosis was suggestive of cardiogenic shock (positive likelihood ratio, LR+=3.2 and negative likelihood ratio, LR-=0.4). Presence of a murmur or gallop (LR+=5.4, LR-=0.3), or decreased femoral pulses (LR+=5.1, LR-=0.5) on physical exam were also suggestive of cardiogenic shock as was cardiomegaly on chest x-ray (LR+=4.9, LR-=0.5). Notably, temperature instability (LR+=0.7, LR-=1.8) and white blood cell count elevation or depression (LR+=0.8, LR-=1.1) were all poor predictors of septic shock. CONCLUSION: Cardiogenic shock is a more common cause of decompensated shock than septic shock. A history of cyanosis, murmur or gallop, or decreased femoral pulses on exam and cardiomegaly on chest x-ray are useful indicators of cardiogenic shock. In evaluating the neonate with decompensated shock, early consideration for Cardiology consultation and interventions to treat the underlying condition is warranted.

Rivaroxaban, a direct Factor Xa inhibitor, versus acetylsalicylic acid as thromboprophylaxis in children post-Fontan procedure: Rationale and design of a prospective, randomized trial (the UNIVERSE study).

BACKGROUND: The Fontan procedure is the final step of the 3-stage palliative procedure commonly performed in children with single ventricle physiology. Thrombosis remains an important complication in children after this procedure. To date, guideline recommendations for the type and duration of thromboprophylaxis after Fontan surgery are mainly based on extrapolation of knowledge gained from adults at risk for thrombosis in other clinical settings. Warfarin is being used off-label, and because of its multiple interactions with other drugs and food, a new alternative is highly desirable. Rivaroxaban, a direct Factor Xa inhibitor with a predictable pharmacokinetic profile, is a candidate to address this medical need. STUDY DESIGN: The UNIVERSE study is a prospective, open-label, active-controlled, multicenter study in children 2 to 8 years of age who have single ventricle physiology and had the Fontan procedure within the 4 months preceding enrollment. This study consists of 2 parts. In Part A, rivaroxaban pharmacokinetics, pharmacodynamics, safety, and tolerability are assessed to validate the pediatric dosing selected. In Part B, safety and efficacy of rivaroxaban versus acetylsalicylic acid are evaluated for thromboprophylaxis in children post-Fontan procedure. Children in each part will receive study drug for 12 months. Part A has been completed with 12 children enrolled. Enrollment into Part B is currently ongoing. CONCLUSIONS: The UNIVERSE study aims to provide dosing, pharmacokinetics/pharmacodynamics, safety, and efficacy information on the use of rivaroxaban, an oral anticoagulant, versus acetylsalicylic acid, an antiplatelet agent, in children with single ventricle physiology after the Fontan procedure.

Use of sildenafil in an infant with persistent pulmonary hypertension secondary to lung and renal hypoplasia - a case report.

BACKGROUND: Premature preterm rupture of membranes (PPROM) is reported to be associated with high rates of neonatal mortality and morbidity. Sildenafil has been used in infants with persistent pulmonary hypertension of newborn (PPHN) due to congenital diaphragmatic hernia (CDH) and bronchopulmonary dysplasia (BPD). Recently, Sildenafil has been evaluated as an alternative or adjunctive pulmonary vasodilator. This case report illustrates the use of early sildenafil for PPHN and right ventricular dysfunction in an unusual setting of lung and renal hypoplasia. CASE PRESENTATION: A male infant was born at 37 weeks with a birth weight of 2840 g. Rupture of membranes developed at approximately 24 weeks of gestational age (GA). Bilateral small kidneys (< 2 standard deviations below average) were detected on ultrasound (US) examination at 30 weeks of gestation. The baby developed pneumothorax and pulmonary hypertensive crisis towards the end of the first day. An echocardiogram showed a dilated right ventricle, moderate right ventricular systolic dysfunction, hypoplastic pulmonary arteries and a large patent ductus arteriosus with bidirectional flow. The patient was sedated, paralyzed, and inhaled nitric oxide was administered to decrease the pulmonary resistance. In anticipation of persistent pulmonary hypertension due to the hypoplastic lungs and small calibre of pulmonary arteries, sildenafil was started on day of life (DOL) 5 at a dosage of 0.25 mg/kg/dose Q8H and gradually increased to 2 mg/kg/dose Q8H on DOL 9. The patient was finally extubated on DOL 7 and weaned off of non-invasive respiratory support on DOL 26. Sildenafil was gradually weaned beginning on DOL 21 and discontinued on DOL 48. Repeat echocardiogram assessment at 3 months showed complete resolution of PHT and right ventricular dilatation. CONCLUSIONS: We describe the early use of sildenafil in treating pulmonary hypertension associated with lung and renal hypoplasia in a non-CDH patient. Following this treatment the patient made a full recovery from right ventricular dysfunction.

Pulmonary artery wall thickness in children with Fontan physiology: an optical coherence tomography case control study.

INTRODUCTION: Failure of the Fontan circulation is not a well-understood clinical phenomena.For some patients, a gradual increase in pulmonary vascular resistance (PVR) and structural changes in the pulmonary artery may be an important causative factor. To further investigate this issue, we employed optical coherence tomography (OCT) to evaluate structural changes within the pulmonary arteries of Fontan patients and compared to those with a normal pulmonary circulation. MATERIALS AND METHODS: Pulmonary artery OCT was performed, without complications, in 12 Fontan and 11 control patients. Wall thickness and wall:vessel cross-sectional area (CSA) ratio were calculated after image acquisition, using digital planimetry. RESULTS: There was no difference in wall thickness between both groups. Median wall thickness for Fontan patients was 0.12 mm (IQR, 0.10-0.14) and for controls was 0.11 mm (IQR, 0.10-0.12; p = 0.62). Wall:vessel CSA ratio for Fontan patients was 0.13 (IQR, 0.12-0.16) and for controls was 0.13 (IQR, 0.11-0.15) (p = 0.73). There was no association between wall thickness and ventricle morphology, age at catheterisation, age at Fontan, years since Fontan completion, pulmonary artery pressure, and PVR. The vessel media was more readily visualised in control patients. DISCUSSION: OCT of the pulmonary arteries in Fontan patients is safe and feasible. Our OCT findings suggest that during childhood, pulmonary artery wall dimensions are normal in Fontan children with reassuring hemodynamics. Further evaluation of Fontan patients with abnormal hemodynamics and serial evaluation into adulthood are required to conclude on the utility of OCT for identifying early pulmonary artery structural changes.

Intimal thickening at coronary bifurcations in pediatric heart transplant recipients.

Heart transplant recipients are at increased risk for atherosclerosis and cardiac allograft vasculopathy, both initially presenting as intimal thickening. We aimed to determine the presence, extent, and anatomical characteristics of intimal thickness at coronary bifurcations in children using OCT. We measured the intimal thickness of coronary arteries in pediatric transplant recipients using OCT during routine cardiac catheterization. Intimal thickening was defined as (i) a percent change in contralateral intimal thickness greater than 50% when comparing the thickness at the bifurcation to the baseline thickness, and (ii) greater than 0.1 mm. We evaluated 153 unique coronary bifurcations in 31 children (58% boys, median 12.7 years). Intimal thickening was almost exclusively observed in the left coronary system (22 of 67 bifurcations) and rare in the right coronary system (2 of 86 bifurcations; P < .001). There was a positive association between the relative size of the side branch and contralateral intimal thickening at coronary bifurcations (P = .009). Intimal thickening at coronary bifurcations is already present in the left coronary system in many pediatric transplant recipients. The correlation between intimal thickening and side branch size suggests that low shear stress and oscillating shear stress may have an important role in the development of intimal thickening at coronary bifurcations.

Outcomes of Radiofrequency Perforation for Pulmonary Atresia and Intact Ventricular Septum: A Single-Centre Experience.

Percutaneous radiofrequency perforation (RFP) of the pulmonary valve is used as a primary therapy in neonates with pulmonary atresia and intact ventricular septum (PAIVS). We sought to determine the safety and efficacy of RFP for PAIVS in a single center and assess the pre-intervention anatomical parameters associated with a biventricular outcome. We retrospectively reviewed all cases of PAIVS treated with RFP at a single center from 1999 through 2012. We collected baseline imaging data, technical aspects of the procedure, adverse events and outcomes. RFP was attempted in 18 patients with 17 successful procedures. There was no mortality; one patient had an acute complication requiring surgical intervention. All were alive at the most recent follow-up (median 4.9 years; IQR = 2.0-6.8 years), 12/17 (71%) had a biventricular circulation, 2/17 (12%) had a 1½ ventricle repair, 2/17 (12%) had a univentricular repair and 1/17 was lost to follow-up. A biventricular outcome in patients with PAIVS was associated with the pre-intervention tricuspid valve/mitral valve (TV/MV) ratio and tricuspid valve (TV) z-score. The median TV/MV ratio for patients who underwent a biventricular repair and a non-biventricular repair was 0.82 (IQR = 0.71-0.90) and 0.59 (IQR = 0.39-0.76), P = 0.036, respectively. The median TV z-scores were -3.2 [(-4.9 to -2.6), and -6.8 (-9.7 to -4.8] P = 0.036 for the biventricular and non-biventricular groups, respectively. RFP is a safe primary therapy for PAIVS. With appropriate patient selection, RFP will often result in a biventricular circulation. Both the TV/MV and TV z-score were found to be a predictor of a biventricular outcome in our cohort.

Valvar aortico-ventricular tunnel: an insight into the development of the great arteries.

Aorto-left ventricular tunnel is a rare congenital heart lesion, with an incidence of <0.1% of all CHD. We present a unique case of a valvar aorto-left ventricular tunnel in a neonate, in belief that our findings may shed some light on the developmental origins of this lesion.

Hybrid stenting for left ventricular outflow tract obstruction in congenitally corrected transposition of the great arteries.

Congenitally corrected transposition of the great arteries is commonly associated with left ventricular outflow tract obstruction. We describe a case of congenitally corrected transposition of the great arteries and previous surgical ventricular septal defect repair with recurrent left ventricular outflow tract obstruction. The patient underwent a hybrid procedure to stent the left ventricular outflow tract, which was successful with no re-intervention through 3 years of follow-up.

Obesity and arterial stiffness in children: systematic review and meta-analysis.

OBJECTIVE: Childhood obesity is associated with risk factors for cardiovascular disease. Arterial stiffness is considered one of the earliest detectable measures of vascular damage. There is controversy in the literature regarding the effects of childhood obesity on arterial stiffness. The objective of this study is to systematically review the literature and to conduct a meta-analysis comparing measures of central arterial stiffness in children and adolescents with obesity to healthy body mass index controls. APPROACH AND RESULTS: Literature searches were conducted using databases (eg, MEDLINE, EMBASE) and citations cross-referenced. Studies assessing central pulse wave velocity or ß-stiffness index were included. A random effects meta-analysis of the standardized mean difference and 95% confidence intervals in arterial stiffness between children with obesity and control children was performed for each arterial stiffness measure. A total of 523 studies were identified. Fifteen case-control studies were included, with 2237 children/adolescents (1281 with obesity, 956 healthy body mass index controls) between 5 and 24 years of age. All studies measuring carotid and aortic ß-stiffness index and 10/12 studies measuring central pulse wave velocity reported greater arterial stiffness in children/adolescents with obesity compared with controls. A random effects meta-analysis was performed revealing a significant effect of obesity on pulse wave velocity (standardized mean difference=0.718; 95% confidence interval=0.291-1.415), carotid ß-stiffness index (0.862; 0.323-1.402), and aortic ß stiffness index (1.017; 0.419-1.615). CONCLUSION: These findings indicate that child/adolescent obesity is associated with greater arterial stiffness. However, further research is needed to address confounders, such as pubertal status, that may affect this relationship in children. In the future, these techniques may be useful in risk stratification and guiding clinical management of obese children to optimize cardiovascular outcomes.

Peritoneal-pericardial communication in an adolescent on peritoneal dialysis.

BACKGROUND: Dialysate leakage into the pericardium is a rare but potentially life-threatening complication of peritoneal dialysis (PD). There has been one reported pediatric case of spontaneous peritoneo-pericardial fistula in a 2-year-old boy with tissue fragility due to malnutrition and two reported adult cases in PD patients with a history of previous cardiac surgery and/or pericardiocentesis. CASE-DIAGNOSIS/TREATMENT: We describe a 15-year-old girl with end-stage renal disease secondary to granulomatosis with polyangiitis, with recurrent pericardial effusions secondary to a peritoneo-pericardial fistula while on continuous cycling peritoneal dialysis (CCPD). She had previously presented with chylous pericardial effusion that required pericardiocentesis and subsequently developed recurrent pericardial effusions when she was commenced on CCPD 9 months later. Pericardial fluid chemistry revealed a sterile, serous fluid containing 15.1 mmol/L of glucose and <0.11 mmol/L of triglycerides. Peritoneal scintigraphy with Tc-99m labeled sulfur colloid injected intra-peritoneally confirmed the presence of a peritoneo-pericardial fistula. The pericardial effusions resolved upon switching the patient to hemodialysis (HD). CONCLUSIONS: Our case of recurrent pericardial effusions in a child on PD secondary to a peritoneo-pericardial fistula highlights the need for close follow-up in patients with a history of previous pericardiocentesis who are commenced on PD.

A prospective observational multicenter study of balloon angioplasty for the treatment of native and recurrent coarctation of the aorta.

OBJECTIVES: Balloon angioplasty (BA) is an important treatment option for coarctation of the aorta. The congenital cardiovascular interventional study consortium (CCISC) represents a multi-institutional and multi-national effort to prospectively investigate congenital cardiac interventions. A prospective observational analysis of the efficacy and safety of balloon aortic angioplasty was conducted. METHODS: Data were collected prospectively from 36 CCISC sites from 2004 to 2012. One hundred and thirty patients underwent BA for native (n = 76) and recurrent (n = 54) coarctation. Acute, short-term, and intermediate outcomes are described for BA performed in the setting of native and recurrent coarctation of the aorta. Outcome measures included residual upper to lower extremity blood pressure gradient (ULG), use of antihypertensive medications, aortic wall injury, reobstruction, and need for reintervention. RESULTS: There was no procedural mortality. Acutely in native and recurrent coarctation, BA achieved an ULG less than 15 mm Hg in 73-80% and to less than 10 mm Hg in 54-68% of patients, respectively. At intermediate follow-up, ULG further improved, particularly for those who underwent initial reintervention for recurrent coarctation. No significant differences in aortic wall complications were seen and intervention free survival was similar for both groups. Following angioplasty, there was no significant difference in aortic wall complications; however follow up integrated imaging decreased over time. CONCLUSIONS: BA is a safe and effective treatment for coarctation of the aorta acutely and at intermediate term. Although aortic injury occurred in patients with both native and recurrent coarctation, at intermediate follow-up, aneurysm was noted more often in those with initial intervention for native coarctation.

Optical coherence tomography for the evaluation of asymmetric cardiac allograft vasculopathy in a child.

We present an unusual case of CAV in a child with isolated disease in the LAD coronary artery. Initial progression of the disease appeared to have been halted by the use of sirolimus, but the assessment of disease in other vessels (particularly the RCA) was of particular importance in deciding whether or not to relist this patient for transplantation. Due to the known limitations of coronary angiography, we used OCT to assess for angiographically silent CAV. The normal intravascular appearance of the RCA by OCT was reassuring, and the child was not relisted for transplantation. OCT offers multiple advantages for the assessment of CAV in children.

Single-ventricle anatomy predicts delayed microstructural brain development.

BACKGROUND: Term newborns with congenital heart disease (CHD) show delayed brain development as early as the third trimester, especially in single-ventricle physiology (SVP). Mechanisms causing delayed brain development in CHD are uncertain but may include impaired fetal brain blood flow. Our objective was to determine if cardiac anatomy associated with obstruction to antegrade flow in the ascending aorta is predictive of delayed brain development as measured by diffusion tensor imaging and magnetic resonance spectroscopic (MRS) imaging. METHODS: Echocardiograms from 36 term newborns with SVP were reviewed for the presence of aortic atresia and the diameter of the ascending aorta. Quantitative magnetic resonance imaging parameters measuring brain microstructural (fractional anisotropy (FA) and average diffusivity (Dav)) or metabolic development (N-acetylaspartate (NAA) and lactate/choline (Lac/Cho)) were recorded. RESULTS: Increasing NAA/Cho and white matter (WM) FA, and decreasing Dav and Lac/Cho characterize normal brain development. Consistent with the hypothesis that delayed brain development is related to impaired brain perfusion, smaller ascending aortic diameter and aortic atresia were associated with higher Dav and lower WM FA. Echocardiogram variables were not associated with brain metabolic measures. CONCLUSIONS: These observations support the hypothesis that obstruction to fetal cerebral blood flow impairs brain microstructural development.

Variation in paediatric 24-h ambulatory blood pressure monitoring interpretation by Canadian and UK physicians.

Twenty-four-hour ambulatory blood pressure monitoring (ABPM) is widely accepted as a more accurate method for measurement of blood pressure (BP) compared to a single office-based measurement of BP. However, it is unclear how physicians interpret ABPM and make management decisions. This study's goal is to investigate variation in ABPM interpretation among paediatric nephrologists (Canada and UK) and paediatric cardiologists (Canada only) via an online survey. The survey content included baseline demographics, questions on the use and indications for ABPM, interpretation of results, and subsequent management decisions in various clinical scenarios. The survey was sent to 196 Canadian physicians, with 69 (35.2%) total responses. Thirty-five UK clinicians also completed the survey. Most respondents were >44 years old, were in practice for at least 11 years, and were university-based. There were substantial differences among clinicians in ABPM interpretation for isolated systolic, diastolic, and night-time hypertension. For example, only 53.1% of physicians would initiate or modify treatment in those with diastolic HTN in CKD. Further, even for the same abnormal ABPM parameter, the decision to start or alter treatment was influenced by the underlying medical condition. There is significant variation in clinical practice among physicians for interpretation and management of hypertension when using ABPM. Differences in guidelines among various jurisdictions, as well as knowledge gaps in the research on which guidelines are based, create ambiguity regarding ABPM interpretation and management decisions. A more protocolized approach and further insight into the reasoning behind the variation in physicians' interpretation may help to standardise practice.

Reduced Physical Activity During COVID-19 in Children With Congenital Heart Disease: A Longitudinal Analysis.

Background: Coronavirus disease 2019 (COVID-19) was associated with a reduction in physical activity in children with congenital heart disease (CHD) in early 2020. Given the increased cardiovascular risk of this population, optimizing cardiovascular health behaviour is important. The aim of the study is to determine how the ongoing COVID-19 pandemic has impacted longitudinal physical activity measures in children with CHD. Methods: As part of a prospective cohort study, children and adolescents aged 9-16 years old with moderate-to-complex CHD were recruited from British Columbia Children's Hospital and partnership clinics across British Columbia and the Yukon territory. Daily step counts were measured continuously over 3 years (2018-2021) with Fitbit Charge 2. School status during the COVID-19 pandemic was assessed with parent- or self-report survey. Results: A total of 102, 114, and 93 participants had valid Fitbit data during 2018, 2019, and 2020, respectively. There was a significant reduction in the annual mean step count for 2020 (8225 ± 4328 steps) compared with both 2018 (9416 ± 3770 steps) and 2019 (9533 ± 4114 steps) (P < 0.001). There was a loss of seasonal variation in physical activity, and reduced levels of physical activity persisted when most children resumed in-person schooling in September 2020. Conclusions: We demonstrated a significant decrease in physical activity and loss of seasonal patterns in children with CHD during 2020. These findings represent a worsening of the cardiovascular risk profile in children with CHD, who are already at an increased risk of adverse cardiovascular outcomes. Mitigation strategies are needed to optimize the cardiovascular health status of children with CHD as the pandemic persists.

Contexte: Au début de l'année 2020, la maladie à coronavirus 2019 (COVID-19) a été associée à une diminution de l'activité physique chez les enfants présentant une cardiopathie congénitale. Compte tenu du risque cardiovasculaire accru chez ces patients, il importe d'optimiser les comportements en matière de santé cardiovasculaire. Cette étude visait à évaluer les répercussions de la pandémie actuelle de COVID-19 sur les mesures longitudinales de l'activité physique auprès d'enfants présentant une cardiopathie congénitale. Méthodologie: Pour cette étude de cohorte prospective, des enfants et des adolescents de 9 à 16 ans atteints de cardiopathie congénitale modérée ou complexe ont été recrutés au British Columbia Children's Hospital ainsi que dans les cliniques partenaires de la Colombie-Britannique et du Yukon. Le nombre de pas quotidiens a été compté en continu pendant trois ans (2018-2021) à l'aide d'un appareil Fitbit Charge 2. Les enfants ou leurs parents ont indiqué, à l'aide d'un sondage, s'ils allaient à l'école ou non pendant la pandémie de COVID-19. Résultats: Des données de l'appareil Fitbit ont été obtenues chez un total de 102, 114 et 93 participants en 2018, 2019 et 2020, respectivement. Une nette réduction du nombre de pas annuel moyen en 2020 (8225 ± 4328 pas) a été constatée par rapport à 2018 (9416 ± 3770 pas) et à 2019 (9533 ± 4114 pas) (p < 0,001). Les variations saisonnières de l'activité physique se sont amoindries, et le faible volume d'activité physique a perduré même lorsque la majorité des enfants avaient repris l'école en présentiel, en septembre 2020. Conclusions: Une nette diminution de l'activité physique et un affaiblissement des variations saisonnières de l'activité physique ont été observés chez les enfants présentant une cardiopathie congénitale en 2020. Ces résultats signifient une aggravation du risque cardiovasculaire que courent ces patients, déjà exposés à un risque accru d'événements cardiovasculaires. Comme la pandémie persiste, des stratégies d'atténuation des risques sont nécessaires pour optimiser la santé cardiovasculaire des enfants présentant une cardiopathie congénitale.

Hepatic and Renal Consequences of Single-Ventricle Physiology Palliated With the Fontan Operation.

Over time, long-term survival has dramatically increased for patients with complex congenital heart disease who undergo the Fontan operation. With this increased survival, it has become apparent that such a circulation has important consequences for other organ systems, particularly the liver and kidney. The adverse milieu created by chronic venous hypertension, low cardiac output, and an inflammatory state contribute to the pathologic changes observed in the liver and kidneys over the long term in Fontan patients. The clinical importance of these hepatic and renal comorbidities have only recently begun to be recognized in the context of increasing life expectancy in this population. The objectives of this review are to provide an overview of the pathophysiology of the Fontan circulation and how liver and kidney disease evolve in this setting; to summarize the current evidence base as it relates to the diagnostic approach to liver and kidney disease in Fontan patients; and to discuss the therapeutic approaches to Fontan- associated liver and kidney disease. Given that this is a very active area of research in congenital heart disease, we have identified knowledge gaps and priority research areas to improve the care of Fontan patients. These include establishing the optimal diagnostic tests to detect and track liver and kidney disease change over time, determining which treatable risk factors contribute to the development of liver and kidney disease, and evaluating therapies to prevent or slow progression of liver and kidney disease.

A Survey of Immunization Practices in Patients With Congenital Heart Disease.

Background: Congenital heart disease, the most common congenital anomaly, often presents in neonates. Because of perceived risks, health care providers may consider deferring immunizations in this population. We sought to understand the perceived risk of immunizations in those providing health care to children with particular heart conditions. Methods: A survey, which included 6 hypothetical scenarios assessing immunization recommendations, was distributed internationally to relevant health care providers, and responses were compared between the different scenarios. Results: Majority of responses (n = 142) were from paediatric cardiologists (n = 98; 69%) and nurse practitioners (n = 27; 19%) located in the United States (n = 77; 54%) or Canada (n = 53; 37%) working in academic teaching hospitals (n = 133; 93.7%). Most favoured vaccinations (n = 107; 75.4%) and less likely to proceed with the first immunization in infants with structural heart disease compared with channelopathy (risk ratio: 0.80, confidence interval: 0.73-0.87; P < 0.001). Only 40% would proceed with immunization as normal in an infant with manifest Brugada type I electrocardiogram. Special precautions after the immunization included longer duration of observation (19%) and administering prophylactic antipyretic medication (92%). Conclusions: Respondents were 20% more likely to defer immunizations in the presence of treatable structural heart disease as compared with channelopathy despite the lack of evidence supporting deferring immunizations in children with structural heart disease. Most were cautious in their response to the scenario involving Brugada syndrome, indicating awareness of the risk of haemodynamic instability in the event of a fever. The majority of respondents still strongly recommend immunizations in this population as the benefits outweigh the potential for adverse events.

Contexte: La cardiopathie congénitale ­ l'anomalie congénitale la plus courante ­ est souvent observée chez les nouveau-nés. En raison des risques perçus, les dispensateurs de soins de santé peuvent parfois envisager de reporter la vaccination chez ces patients. Notre but était de comprendre le risque perçu à l'égard de la vaccination par les dispensateurs de soins de santé traitant des enfants atteints de certaines cardiopathies. Méthodologie: Un sondage comprenant six scénarios hypothétiques visant à évaluer les recommandations de vaccination a été distribué à des dispensateurs de soins de santé pertinents dans différents pays, et leurs réponses pour les différents scénarios ont été comparées. Résultats: La majorité des répondants (n = 142) étaient des cardiologues pédiatriques (n = 98; 69 %) ou des infirmières praticiennes (n = 27; 19 %) des États-Unis (n = 77; 54 %) ou du Canada (n = 53; 37 %) travaillant dans des hôpitaux universitaires (n = 133; 93,7 %). La plupart d'entre eux étaient en faveur de la vaccination (n = 107; 75,4 %), bien que moins enclins à administrer un premier vaccin à des nourrissons présentant une cardiopathie structurelle comparativement à une canalopathie (rapport des risques : 0,80, intervalle de confiance : 0,73-0,87; p < 0,001). Or, seulement 40 % d'entre eux vaccineraient de façon normale un nourrisson présentant un syndrome de Brugada de type 1 à l'ECG. Les précautions particulières prises après la vaccination comprenaient une période d'observation plus longue (19 %) et l'administration d'un antipyrétique à des fins prophylactiques (92 %). Conclusions: À la lumière des réponses obtenues, la probabilité de report de la vaccination était 20 % plus élevée en présence d'une cardiopathie structurelle traitable comparativement à une canalopathie, malgré le manque de données probantes justifiant ce report chez les enfants atteints d'une cardiopathie structurelle. La plupart des répondants ont répondu de façon prudente au scénario du syndrome de Brugada en évoquant un risque d'instabilité hémodynamique en cas de fièvre. La majorité d'entre eux recommandent quand même fortement la vaccination chez ces patients, car les bienfaits escomptés l'emportent sur les risques d'effets indésirables.