Predictors of Developing Heart Failure in Adults with Congenital Heart Defects.

Background: The population of adults with congenital heart defects (ACHD) is growing. The leading cause of premature death in these patients is heart failure (HF). However, there is still limited information on the predictive factors for HF in ACHD patients. Objectives: This study re-examined a group of patients with repaired or palliated congenital heart defects (CHD) that were initially studied in 2003. A follow-up period of 15 years has allowed us to identify and evaluate predictors for the development of HF in ACHD. Methods: All patients with repaired or palliated CHD who participated in the initial study (n = 364) were invited for a follow-up examination. The effects of maximum oxygen uptake ( VO 2max ) during exercise stress testing, the cardiac biomarker N-terminal pro brain natriuretic peptide (NT-proBNP), and QRS complex on the development of HF during the follow-up period were investigated. Results: From May 2017 to April 2019, 249 of the initial 364 (68%) patients participated in the follow-up study. Of these, 21% were found to have mild CHD, 60% had moderate CHD, and 19% had complex CHD. Significant predictors for the development of HF were: NT-proBNP level > 1.7 times the upper normal limit, VO 2max < 73% of predicted values, and QRS complex duration > 120 ms. Combination of these three parameters resulted in the highest area-under-the-curve of 0.75, with a sensitivity of 75% and specificity of 63% for predicting the development of HF. Conclusions: In this cohort of ACHD patients, the combination of VO 2max% , NT-proBNP, and QRS duration was predictive of HF development over a 15-year follow-up period. Enhanced surveillance of these parameters in patients with ACHD may be beneficial for the prevention of HF and early intervention.

Impact of the 2022 American Heart Association pediatric ambulatory blood pressure monitoring statement on the diagnosis of hypertension.

BACKGROUND: The diagnosis of hypertension and hypertension-induced target organ injury by the 2022 American Heart Association (AHA) ambulatory blood pressure threshold as compared with 2014 AHA and 2016 European Society of Hypertension (ESH) thresholds has not been evaluated. METHODS: In a cross-sectional study (n = 291, aged 5-18 years, at a tertiary care outpatient clinic), we compared 2022 AHA with 2014 AHA and ESH thresholds (revised with 2018 adult ESH thresholds where applicable) to diagnose ambulatory hypertension (AH), and detect ambulatory arterial stiffness index (AASI) and left ventricular target organ injury (LVTOI). RESULTS: The 2022 AHA threshold diagnosed significantly more AH (53%) than the 2014 AHA (42%, p < 0.01) and ESH (36%, p < 0.001) thresholds. The 2022 AHA threshold demonstrated only a moderate agreement with the 2014 AHA (kappa (k) = 0.77) and ESH (k = 0.66) thresholds to diagnose AH. Adjusted logistic regression analysis found that only the 2022 AHA threshold predicted elevated AASI significantly (odds ratio 2.40, 95% CI 1.09, 5.25, p = 0.02; AUC 0.61, p < 0.01). In those with elevated AASI, more participants had AH by the 2022 AHA threshold (72%) than the 2014 AHA (46%, p = 0.02) and ESH (48%, p = 0.03) thresholds. AH defined by the 2022 AHA threshold continued to maintain higher odds, larger AUC, and higher sensitivity to identify LVTOI than the 2014 AHA and ESH thresholds; however, the difference did not reach a statistically significant level. CONCLUSIONS: AH defined by the 2022 AHA threshold diagnoses more children with hypertension and identifies more children with hypertension-induced target organ injury than the 2014 AHA and ESH thresholds. A higher resolution version of the Graphical abstract is available as Supplementary information.

Cardiac Biomarkers Aid in Differentiation of Kawasaki Disease from Multisystem Inflammatory Syndrome in Children Associated with COVID-19.

Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) associated with COVID-19 show clinical overlap and both lack definitive diagnostic testing, making differentiation challenging. We sought to determine how cardiac biomarkers might differentiate KD from MIS-C. The International Kawasaki Disease Registry enrolled contemporaneous KD and MIS-C pediatric patients from 42 sites from January 2020 through June 2022. The study population included 118 KD patients who met American Heart Association KD criteria and compared them to 946 MIS-C patients who met 2020 Centers for Disease Control and Prevention case definition. All included patients had at least one measurement of amino-terminal prohormone brain natriuretic peptide (NTproBNP) or cardiac troponin I (TnI), and echocardiography. Regression analyses were used to determine associations between cardiac biomarker levels, diagnosis, and cardiac involvement. Higher NTproBNP (≥ 1500 ng/L) and TnI (≥ 20 ng/L) at presentation were associated with MIS-C versus KD with specificity of 77 and 89%, respectively. Higher biomarker levels were associated with shock and intensive care unit admission; higher NTproBNP was associated with longer hospital length of stay. Lower left ventricular ejection fraction, more pronounced for MIS-C, was also associated with higher biomarker levels. Coronary artery involvement was not associated with either biomarker. Higher NTproBNP and TnI levels are suggestive of MIS-C versus KD and may be clinically useful in their differentiation. Consideration might be given to their inclusion in the routine evaluation of both conditions.

Variation in Pharmacologic Management of Patients with Kawasaki Disease with Coronary Artery Aneurysms.

OBJECTIVE: To evaluate practice variation in pharmacologic management in the International Kawasaki Disease Registry (IKDR). STUDY DESIGN: Practice variation in intravenous immunoglobulin (IVIG) therapy, anti-inflammatory agents, statins, beta-blockers, antiplatelet therapy, and anticoagulation was described. RESULTS: We included 1627 patients from 30 IKDR centers with maximum coronary artery aneurysm (CAA) z scores 2.5-4.99 in 848, 5.0-9.99 in 349, and ≥10.0 (large/giant) in 430 patients. All centers reported IVIG and acetylsalicylic acid (ASA) as primary therapy and use of additional IVIG or steroids as needed. In 23 out of 30 centers, (77%) infliximab was also used; 11 of these 23 centers reported using it in <10% of their patients, and 3 centers used it in >20% of patients. Nonsteroidal anti-inflammatory agents were used in >10% of patients in only nine centers. Beta-blocker (8.8%, all patients) and abciximab (3.6%, all patients) were mainly prescribed in patients with large/giant CAAs. Statins (2.7%, all patients) were mostly used in one center and only in patients with large/giant CAAs. ASA was the primary antiplatelet modality for 99% of patients, used in all centers. Clopidogrel (18%, all patients) was used in 24 centers, 11 of which used it in >50% of their patients with large/giant CAAs. CONCLUSIONS: In the IKDR, IVIG and ASA therapy as primary therapy is universal with common use of a second dose of IVIG for persistent fever. There is practice variation among centers for adjunctive therapies and anticoagulation strategies, likely reflecting ongoing knowledge gaps. Randomized controlled trials nested in a high-quality collaborative registry may be an efficient strategy to reduce practice variation.

Comparison Between Currently Recommended Long-Term Medical Management of Coronary Artery Aneurysms After Kawasaki Disease and Actual Reported Management in the Last Two Decades.

In the 2017 American Heart Association (AHA) Kawasaki disease (KD) guidelines, risk levels (RLs) for long-term management are defined by both maximal and current coronary artery (CA) dimensions normalized as z-scores. We sought to determine the degree to which current recommended practice differs from past actual practice, highlighting areas for knowledge translation efforts. The International KD Registry (IKDR) included 1651 patients with CA aneurysms (z-score > 2.5) from 1999 to 2016. Patients were classified by AHA RL using maximum CA z-score (RL 3 = small, RL 4 = medium, RL 5 = large/giant) and subcategorized based on decreases over time. Medical management provided was compared to recommendations. Low-dose acetylsalicylic acid (ASA) use ranged from 86 (RL 3.1) to 95% (RL 5.1) for RLs where use was "indicated." Dual antiplatelet therapy (ASA + clopidogrel) use ranged from 16% for RL 5.2 to 9% for RL 5.4. Recommended anticoagulation (warfarin or low molecular weight heparin) use was 65% for RL 5.1, while 12% were on triple therapy (anticoagulation + dual antiplatelet). Optional statin use ranged from 2 to 8% depending on RL. Optional beta-blocker use was 2-25% for RL 5, and 0-5% for RLs 3 and 4 where it is not recommended. Generally, past practice was consistent with the latest AHA guidelines, taking into account the flexible wording of recommendations based on the limited evidence, as well as unmeasured patient-specific factors. In addition to strengthening the overall evidence base, knowledge translation efforts may be needed to address variation in thromboprophylaxis management.

Sapropterin reduces coronary artery malformation in offspring of pregestational diabetes mice.

Endothelial nitric oxide synthase (eNOS) and oxidative stress are critical to embryonic coronary artery development. Maternal diabetes increases oxidative stress and reduces eNOS activity in the fetal heart. Sapropterin (Kuvan®) is an orally active, synthetic form of tetrahydrobiopterin (BH4) and a co-factor for eNOS with antioxidant properties. The aim of the present study was to examine the effects of sapropterin on fetal coronary artery development during pregestational diabetes in mice. Diabetes was induced by streptozotocin to adult female C57BL/6 mice. Sapropterin (10 mg/kg/day) was orally administered to pregnant mice from E0.5 to E18.5. Fetal hearts were collected at E18.5 for coronary artery morphological analysis. Sapropterin treatment to diabetic dams reduced the incidence of coronary artery malformation in offspring from 50.0% to 20.6%. Decreases in coronary artery luminal diameter, volume and abundance in fetal hearts from diabetic mothers, were prevented by sapropterin treatment. Maternal diabetes reduced epicardial epithelial-to-mesenchymal transition (EMT) and expression of transcription and growth factors critical to coronary artery development including hypoxia-inducible factor 1a (Hif1a), Snail1, Slug, ß-catenin, retinaldehyde dehydrogenase 2 (Aldh1a2), basic fibroblast growth factor (bFGF) and vascular endothelial group factor receptor 2 (Vegfr2) in E12.5 hearts. Additionally, eNOS phosphorylation was lower while oxidative stress was higher in E12.5 hearts from maternal diabetes. Notably, these abnormalities were all restored to normal levels after sapropterin treatment. In conclusion, sapropterin treatment increases eNOS activity, lowers oxidative stress and reduces coronary artery malformation in offspring of pregestational diabetes. Sapropterin may have therapeutic potential in preventing coronary artery malformation in maternal diabetes.

Impact of Obesity on Noninvasive Cardiac Hemodynamic Measurement by Electrical Cardiometry in Adults With Aortic Stenosis.

OBJECTIVES: There are substantial potential benefits to noninvasive cardiac monitoring methods, such as electrical cardiometry (EC), over more invasive methods, including significantly reduced risk of complications, lower up-front and operational costs, ease of use, and continuous monitoring. To take advantage of these technologies, clinical equivalence to currently established methods must be determined. The authors sought to determine if the noninvasive measurement of cardiac index (CI) by EC was clinically equivalent to thermodilution (TD) in adult patients with aortic stenosis (AS). DESIGN: This is a cross-sectional study comparing measurement devices in a single patient group. SETTING: Single-center, university teaching hospital. PARTICIPANTS: The study included 52 adult patients with aortic stenosis undergoing right heart catheterization. INTERVENTIONS: Cardiac output (CO) was measured concurrently using EC with an ICON device and TD in 52 participants with AS. CI values were to determine the accuracy and precision of EC in reference to TD. Percentage error (PE) was used to assess their clinical equivalence. The participants were divided further into groups (normal and overweight/obese) based on body mass index and the analysis was repeated. MEASUREMENTS AND MAIN RESULTS: CO measurement made by EC in adult patients with obesity or overweight was reduced significantly relative to TD. This was not observed in normal-weight adult AS patients. EC provided clinically equivalent measurements to TD for measuring CI in normal-weight adult AS patients (PE = 25.0%), but not for those adult AS patients with overweight or obesity (PE = 42.3%). CONCLUSION: Overall, the ICON device produced lower CO and index measurements relative to TD in adult patients with AS. Overweight and obesity also significantly affected the relative precision and accuracy of the ICON electrical cardiometric device to measure CI in these patients.

Lack of access to paediatric cardiology services in the public health system in four major urban centres in Perú.

For one-third of the Peruvian population living in large cities outside the capital city Lima, there is no access to paediatric cardiology services provided by the public health care system. Children with suspected CHD living in these areas are referred to the adult cardiologist working at the regional hospitals for care and treatment. This is a consequence of the universal health care system and the heavily skewed distribution of the health care workforce towards the capital city of Lima. This imbalance has resulted in a severe shortage of paediatric cardiologists practicing outside of Lima and the adult cardiologists that remain are left to make up for this deficit.To gain a better understanding of the current model of care for children with suspected CHD within the public health care system in Peru, we surveyed adult cardiologists from four major urban centres that serve one-third of the Peruvian population outside of Lima. We determined that adult cardiologists spend a significant amount of time treating children, but lack the specialised training and equipment to provide model care. The cardiologists indicated that receiving additional training and appropriate equipment would help enable them to provide proper care for these children.

Does obesity affect the non-invasive measurement of cardiac output performed by electrical cardiometry in children and adolescents?

Electrical cardiometry (EC) is a non-invasive and inexpensive method for hemodynamic assessment and monitoring. However, its feasibility for widespread clinical use, especially for the obese population, has yet to be determined. In this study, we evaluated the agreement and reliability of EC compared to transthoracic Doppler echocardiography (TTE) in normal, overweight, and obese children and adolescents. We measured stroke volume (SV) and cardiac output (CO) of 131 participants using EC and TTE simultaneously. We further divided these participants according to BMI percentiles for subanalyses: <85% normal weight (n = 41), between 85 and 95% overweight (n = 7), and >95% obese (n = 83). Due to small sample size of the overweight group, we combined overweight and obese groups (OW+OB) with no significant change in results (SV and CO) before and after combining groups. There were strong correlations between EC and TTE measurements of SV (r = 0.869 and r = 0.846; p < 0.0001) and CO (r = 0.831 and r = 0.815; p < 0.0001) in normal and OW+OB groups, respectively. Bias and percentage error for CO measurements were 0.240 and 29.7%, and 0.042 and 29.5% in the normal and OW+OB groups, respectively. Indexed values for SV were lower in the OW+OB group than in the normal weight group when measured by EC (p < 0.0001) but no differences were seen when measured by TTE (p = 0.096). In all weight groups, there were strong correlations and good agreement between EC and TTE. However, EC may underestimate hemodynamic measurements in obese participants due to fat tissue.

Implementation of clinical research trials using web-based and mobile devices: challenges and solutions.

BACKGROUND: With the increasing implementation of web-based, mobile health interventions in clinical trials, it is crucial for researchers to address the security and privacy concerns of patient information according to high ethical standards. The full process of meeting these standards is often made more complicated due to the use of internet-based technology and smartphones for treatment, telecommunication, and data collection; however, this process is not well-documented in the literature. RESULTS: The Smart Heart Trial is a single-arm feasibility study that is currently assessing the effects of a web-based, mobile lifestyle intervention for overweight and obese children and youth with congenital heart disease in Southwestern Ontario. Participants receive telephone counseling regarding nutrition and fitness; and complete goal-setting activities on a web-based application. This paper provides a detailed overview of the challenges the study faced in meeting the high standards of our Research Ethics Board, specifically regarding patient privacy. CONCLUSION: We outline our solutions, successes, limitations, and lessons learned to inform future similar studies; and model much needed transparency in ensuring high quality security and protection of patient privacy when using web-based and mobile devices for telecommunication and data collection in clinical research.

Small coronary artery fistulae in childhood: a 6-year experience of 31 cases in a tertiary paediatric cardiac centre.

BACKGROUND: This study describes the incidence and course of children with small coronary artery fistulae over a period of 6 years who presented at a paediatric tertiary-care centre. Materials and methods Age at diagnosis, mode of presentation, location (origin and drainage), and association with a cardiac defect were documented and analysed. All patients obtained an electrocardiogram, and older patients were further evaluated with an exercise treadmill test. RESULTS: A total of 31 patients were diagnosed with coronary artery fistula via transthoracic echocardiogram and comprised 0.43% of our entire patient group. Mean age was 6.14 years (standard deviation 5.4); 16 patients (52%) had associated cardiac defects. In the remaining 15 patients, the coronary artery fistula was discovered incidentally during diagnostic work-up for heart murmur or chest pain. Among all, 26 patients (84%) had left-sided and five patients (16%) had right-sided coronary artery fistulae. All right coronary artery fistula patients had associated cardiac defects; this was true for 42% of the patients with left coronary artery fistulae. None of the patients required any intervention due to the fistula, and spontaneous closure occurred in 12 patients (39%). CONCLUSION: Small coronary artery fistulae in children are frequently an incidental finding, and many will close spontaneously. Our data are supportive of a conservative, observant approach in asymptomatic patients with small coronary artery fistula in the paediatric population.

The Health Initiative Program for Kids (HIP Kids): effects of a 1-year multidisciplinary lifestyle intervention on adiposity and quality of life in obese children and adolescents--a longitudinal pilot intervention study.

BACKGROUND: Though recent data suggest that multidisciplinary outpatient interventions can have a positive effect on childhood obesity, it is still unclear which program components are most beneficial and how they affect quality of life (QoL). The aim of this study was to determine if a 1-year multidisciplinary, family-centered outpatient intervention based on social cognitive theory would be effective in (i) preventing further increases in BMI and BMI z-score, and (ii) improving QoL in obese children and adolescents. METHODS: Obese children and adolescents 8-17 years of age and their families participated in this 1-year longitudinal pilot intervention study. The intervention consisted of fifteen 90-minute educational sessions led by a dietitian, exercise specialist, and social worker. Anthropometric measures, body composition, and QoL (Pediatric Quality of Life Inventory 4.0), were assessed at baseline, 3 months, and 12 months. Laboratory values were measured at baseline and 12 months. The primary outcome measures were change in BMI and BMI z-score, secondary outcome measures included change in QoL and body composition. A paired sample t-test was used to assess within-group differences and 95% confidence intervals were reported for the mean differences. RESULTS: 42 obese children and adolescents (21 girls) completed the 1-year intervention (mean age 12.8 ± 3.14 years). Mean baseline BMI was 31.96 ± 5.94 kg/m(2) and BMI z-score was +2.19 ± 0.34. Baseline QoL (self-assessments and parental assessments) was impaired: mean baseline scores were 74.5 ± 16.5 and 63.7 ± 19.4 for physical functioning and 69.0 ± 14.9 and 64.0 ± 18.3 for emotional functioning, respectively. At 12 months, BMI z-score had decreased (-0.07 ± 0.11, 95% CI: -0.11 to -0.04). BMI (0.80 ± 1.57 kg/m(2), 95% CI 0.31 to 1.29) and fat-free mass (4.02 ± 6.27 kg, 95% CI 1.90 to 6.14) increased, but % body fat and waist circumference did not. Both the parent-reported physical (11.3 ± 19.2, 95% CI 4.7 to 17.9) and emotional (7.7 ± 15.7, 95% CI 2.3 to 13.0) functioning QoL scores and the children's self-reported physical (5.3 ± 17.1, 95% CI 0.5 to 11.1) and emotional (7.9 ± 14.3, 95% CI 3.2 to 12.7) functioning scores significantly improved. CONCLUSIONS: Following a 1-year intervention, the participants' BMI z-scores and QoL improved, while other adiposity-related measures of body composition remained unchanged. TRIAL REGISTRATION: UMIN Clinical Trials Registry UMIN000015622 .

Are the children and adolescents with congenital heart disease living in Southwestern Ontario really overweight and obese?

OBJECTIVE: To assess the prevalence of overweight and obesity in children with congenital heart disease and compare them with age-matched healthy children in Southwestern Ontario, Canada. METHODS: We compared the Center of Disease Control weight and body mass index z-scores of 1080 children, aged 2 to 18 years, who presented to our paediatric cardiology outpatient clinic from 2008 to 2010 for congenital heart disease with 1083 healthy controls. RESULTS: In all, 18.2% of the children with congenital heart disease and 20.8% of healthy children were identified to be either overweight or obese. Overall, the weight category distribution had been similar between the congenital heart disease and healthy control groups, as well as between the congenital heart disease subgroups. There was no difference in normal weight and overweight/obese categories between children with congenital heart disease and healthy children. The underweight category, however, showed a significantly higher prevalence in congenital heart disease compared with healthy children (6.8 and 4.5%, respectively, p = 0.03). CONCLUSION: The prevalence of overweight/obesity did not differ in children with congenital heart disease compared with age-matched healthy children; however, it is still high (18.2%). Obesity may represent an additional risk factor for the long-term cardiovascular health of congenital heart disease patients aside from the underlying heart defect.

Nighttime blood pressure, systolic blood pressure variability, and left ventricular mass index in children with hypertension.

BACKGROUND: Nighttime blood pressure (BP) and systolic BP variability on ambulatory blood pressure monitoring (ABPM) have been strongly associated with target-organ damage in hypertensive adults. The clinical relevance of these variables in children with hypertension remains under-studied. METHODS: The study group included children aged 5-18 years old referred to the outpatient nephrology clinic for an elevated casual BP who underwent an ABPM and echocardiography (ECHO) study and did not have secondary hypertension. The interpretation of ABPM parameters and left ventricular mass index (LVMI) was based on normative references. RESULTS: Seventy-two children fulfilled the inclusion criteria. The association of various potential predictors including age, BMI z-score, casual BP z-score and ABPM parameters (BP z-score, BP load, nocturnal dipping and BP variability- within-subject standard deviation (SD) of BP) with LVMI was analyzed. On adjusted regression analysis, nighttime systolic BP load [standardized regression coefficient (ß) 0.23; p < 0.05] and daytime systolic BP variability (ß 0.37; p < 0.05) had significant association with LVMI. CONCLUSIONS: In children with primary hypertension, nighttime systolic BP load and daytime systolic BP variability had a stronger association with LVMI than casual BP and other ABPM parameters. Future longitudinal studies are needed to establish the causality among these variables.

The effect of bundle branch block on heart function and cardiac output in premature infant.

It is well known that hyperkalemia may cause arrhythmia, right bundle branch block, and cardiac conduction block. These dysrhythmias have the potential to affect cardiac function. We present a premature newborn with hyperkalemia and right bundle branch block causing left ventricular dyssynchrony with mitral regurgitation, which led to decreased pump function.

Non-invasive measurement of cardiac output in obese children and adolescents: comparison of electrical cardiometry and transthoracic Doppler echocardiography.

The objective of this study was to evaluate the reliability and accuracy of electrical cardiometry (EC) for the noninvasive determination of cardiac output (CO) in obese children and adolescents. We compared these results with those obtained by transthoracic echocardiography. Sixty-four participants underwent simultaneous measurement of CO. Cardiac output was measured by EC using the ICON(®) device. Simultaneously CO was determined by using transthoracic Doppler echocardiography from parasternal long-axis and apical view. The median age was 12.52 years (range 7.9-17.6 years) and 36 (56 %) were female. A strongly significant correlation was found between the COEC and COEcho measurements (p < 0.0001, r = 0.91). Significant correlations were also found between CO and age (r = 0.37, p = 0.002), weight (r = 0.57, p < 0.0001), height (0.60, p < 0.0001) and BMI (r = 0.42, p = 0.001). The mean difference between the two methods (COEC - COEcho) was 0.015 l min(-1). According to the Bland and Altman method, the upper and lower limits of agreement, defined as mean difference ±2 SD, were +1.21 and -0.91 l min(-1), respectively. Compared to the transthoracic Doppler echocardiography, Electrical Cardiometry provides accurate and reliable CO measurements in obese children and adolescents.

Left atrial appendage aneurysm in pediatrics: Case study and literature review.

Left atrial appendage aneurysm (LAAA) is a rare cardiac pathology that is often identified in adulthood. There are a myriad of presentations related to atrial appendage enlargement, but most are asymptomatic. Pediatric cases of LAAA are extremely rare. We report a case of an incidental giant LAAA found in a healthy 6-year-old boy. He was successfully treated with surgical resection. A review of the literature shows that the presentation of LAAA in pediatrics likely involves cardiac or respiratory symptoms but can also be incidental findings. Similar to adults, diagnosis requires cardiac imaging, with echocardiography being the mainstay. Surgical intervention is indicated in symptomatic and most asymptomatic patients to prevent complications. More research is warranted into the optimal timing of surgery and alternative surgical approaches for complex cases.

Deep Learning Algorithms to Detect Murmurs Associated With Structural Heart Disease.

Background The success of cardiac auscultation varies widely among medical professionals, which can lead to missed treatments for structural heart disease. Applying machine learning to cardiac auscultation could address this problem, but despite recent interest, few algorithms have been brought to clinical practice. We evaluated a novel suite of Food and Drug Administration-cleared algorithms trained via deep learning on >15 000 heart sound recordings. Methods and Results We validated the algorithms on a data set of 2375 recordings from 615 unique subjects. This data set was collected in real clinical environments using commercially available digital stethoscopes, annotated by board-certified cardiologists, and paired with echocardiograms as the gold standard. To model the algorithm in clinical practice, we compared its performance against 10 clinicians on a subset of the validation database. Our algorithm reliably detected structural murmurs with a sensitivity of 85.6% and specificity of 84.4%. When limiting the analysis to clearly audible murmurs in adults, performance improved to a sensitivity of 97.9% and specificity of 90.6%. The algorithm also reported timing within the cardiac cycle, differentiating between systolic and diastolic murmurs. Despite optimizing acoustics for the clinicians, the algorithm substantially outperformed the clinicians (average clinician accuracy, 77.9%; algorithm accuracy, 84.7%.) Conclusions The algorithms accurately identified murmurs associated with structural heart disease. Our results illustrate a marked contrast between the consistency of the algorithm and the substantial interobserver variability of clinicians. Our results suggest that adopting machine learning algorithms into clinical practice could improve the detection of structural heart disease to facilitate patient care.

Obesity and Outcomes of Kawasaki Disease and COVID-19-Related Multisystem Inflammatory Syndrome in Children.

Importance: Obesity may affect the clinical course of Kawasaki disease (KD) in children and multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19. Objective: To compare the prevalence of obesity and associations with clinical outcomes in patients with KD or MIS-C. Design, Setting, and Participants: In this cohort study, analysis of International Kawasaki Disease Registry (IKDR) data on contemporaneous patients was conducted between January 1, 2020, and July 31, 2022 (42 sites, 8 countries). Patients with MIS-C (defined by Centers for Disease Control and Prevention criteria) and patients with KD (defined by American Heart Association criteria) were included. Patients with KD who had evidence of a recent COVID-19 infection or missing or unknown COVID-19 status were excluded. Main Outcomes and Measures: Patient demographic characteristics, clinical features, disease course, and outcome variables were collected from the IKDR data set. Using body mass index (BMI)/weight z score percentile equivalents, patient weight was categorized as normal weight (BMI <85th percentile), overweight (BMI ≥85th to <95th percentile), and obese (BMI ≥95th percentile). The association between adiposity category and clinical features and outcomes was determined separately for KD and MIS-C patient groups. Results: Of 1767 children, 338 with KD (median age, 2.5 [IQR, 1.2-5.0] years; 60.4% male) and 1429 with MIS-C (median age, 8.7 [IQR, 5.3-12.4] years; 61.4% male) were contemporaneously included in the study. For patients with MIS-C vs KD, the prevalence of overweight (17.1% vs 11.5%) and obesity (23.7% vs 11.5%) was significantly higher (P < .001), with significantly higher adiposity z scores, even after adjustment for age, sex, and race and ethnicity. For patients with KD, apart from intensive care unit admission rate, adiposity category was not associated with laboratory test features or outcomes. For patients with MIS-C, higher adiposity category was associated with worse laboratory test values and outcomes, including a greater likelihood of shock, intensive care unit admission and inotrope requirement, and increased inflammatory markers, creatinine levels, and alanine aminotransferase levels. Adiposity category was not associated with coronary artery abnormalities for either MIS-C or KD. Conclusions and Relevance: In this international cohort study, obesity was more prevalent for patients with MIS-C vs KD, and associated with more severe presentation, laboratory test features, and outcomes. These findings suggest that obesity as a comorbid factor should be considered at the clinical presentation in children with MIS-C.

Aortic-Valve Sparing Repair and Homograft and Dacron Pulmonary Reconstruction Long-term After Arterial Switch Operation.

The arterial switch operation is the gold-standard treatment for dextro-transposition of the great arteries. Long-term follow-up data are beginning to reveal its natural history and associated late complications, including various reoperations for those complications. Given the unique anatomy and the increasing longevity of these patients, there is a need for effective surgical repair options to address aneurysmal and degenerative changes in both neoaortic and pulmonic roots. Thereby, we describe our technique and the novel considerations for prosthetic choice with reconstruction of both the neoaortic root and pulmonary artery, with satisfactory postoperative results.

La détransposition artérielle constitue le traitement de référence dans les cas de dextro-transposition des gros vaisseaux. De nouvelles données, issues du suivi à long terme, nous permettent de mieux comprendre l'évolution naturelle à la suite de cette intervention et les complications tardives qui y sont associées, y compris les diverses interventions à réaliser pour les corriger. Étant donné les caractéristiques anatomiques uniques de ces patients et l'augmentation de leur espérance de vie, il est nécessaire de proposer des options efficaces de réparations chirurgicales pour remédier aux changements anévrismaux et dégénératifs des racines néoaortique et pulmonaire. Ainsi, nous décrivons la technique que nous avons utilisée et les nouveaux éléments qui entrent en ligne de compte dans le choix d'une prothèse pour une reconstruction de la racine néoaortique et de l'artère pulmonaire, avec des résultats postopératoires jugés satisfaisants.