Quantitative Prediction of Right Ventricular Size and Function From the ECG.

BACKGROUND: Right ventricular ejection fraction (RVEF) and end-diastolic volume (RVEDV) are not readily assessed through traditional modalities. Deep learning-enabled ECG analysis for estimation of right ventricular (RV) size or function is unexplored. METHODS AND RESULTS: We trained a deep learning-ECG model to predict RV dilation (RVEDV >120 mL/m2), RV dysfunction (RVEF ≤40%), and numerical RVEDV and RVEF from a 12-lead ECG paired with reference-standard cardiac magnetic resonance imaging volumetric measurements in UK Biobank (UKBB; n=42 938). We fine-tuned in a multicenter health system (MSHoriginal [Mount Sinai Hospital]; n=3019) with prospective validation over 4 months (MSHvalidation; n=115). We evaluated performance with area under the receiver operating characteristic curve for categorical and mean absolute error for continuous measures overall and in key subgroups. We assessed the association of RVEF prediction with transplant-free survival with Cox proportional hazards models. The prevalence of RV dysfunction for UKBB/MSHoriginal/MSHvalidation cohorts was 1.0%/18.0%/15.7%, respectively. RV dysfunction model area under the receiver operating characteristic curve for UKBB/MSHoriginal/MSHvalidation cohorts was 0.86/0.81/0.77, respectively. The prevalence of RV dilation for UKBB/MSHoriginal/MSHvalidation cohorts was 1.6%/10.6%/4.3%. RV dilation model area under the receiver operating characteristic curve for UKBB/MSHoriginal/MSHvalidation cohorts was 0.91/0.81/0.92, respectively. MSHoriginal mean absolute error was RVEF=7.8% and RVEDV=17.6 mL/m2. The performance of the RVEF model was similar in key subgroups including with and without left ventricular dysfunction. Over a median follow-up of 2.3 years, predicted RVEF was associated with adjusted transplant-free survival (hazard ratio, 1.40 for each 10% decrease; P=0.031). CONCLUSIONS: Deep learning-ECG analysis can identify significant cardiac magnetic resonance imaging RV dysfunction and dilation with good performance. Predicted RVEF is associated with clinical outcome.

E-Health: A Game Changer in Fetal and Neonatal Cardiology?

Technological advancements have greatly impacted the healthcare industry, including the integration of e-health in pediatric cardiology. The use of telemedicine, mobile health applications, and electronic health records have demonstrated a significant potential to improve patient outcomes, reduce healthcare costs, and enhance the quality of care. Telemedicine provides a useful tool for remote clinics, follow-up visits, and monitoring for infants with congenital heart disease, while mobile health applications enhance patient and parents' education, medication compliance, and in some instances, remote monitoring of vital signs. Despite the benefits of e-health, there are potential limitations and challenges, such as issues related to availability, cost-effectiveness, data privacy and security, and the potential ethical, legal, and social implications of e-health interventions. In this review, we aim to highlight the current application and perspectives of e-health in the field of fetal and neonatal cardiology, including expert parents' opinions.

Neighborhood Childhood Opportunity, Race/Ethnicity, and Surgical Outcomes in Children With Congenital Heart Disease.

BACKGROUND: Racial and ethnic disparities in outcomes for children with congenital heart disease (CHD) coexist with disparities in educational, environmental, and economic opportunity. OBJECTIVES: We sought to determine the associations between childhood opportunity, race/ethnicity, and pediatric CHD surgery outcomes. METHODS: Pediatric Health Information System encounters aged <18 years from 2016 to 2022 with International Classification of Diseases-10th edition codes for CHD and cardiac surgery were linked to ZIP code-level Childhood Opportunity Index (COI), a score of neighborhood educational, environmental, and socioeconomic conditions. The associations of race/ethnicity and COI with in-hospital surgical death were modeled with generalized estimating equations and formal mediation analysis. Neonatal survival after discharge was modeled by Cox proportional hazards. RESULTS: Of 54,666 encounters at 47 centers, non-Hispanic Black (Black) (OR: 1.20; P = 0.01), Asian (OR: 1.75; P < 0.001), and Other (OR: 1.50; P < 0.001) groups had increased adjusted mortality vs non-Hispanic Whites. The lowest COI quintile had increased in-hospital mortality in unadjusted and partially adjusted models (OR: 1.29; P = 0.004), but not fully adjusted models (OR: 1.14; P = 0.13). COI partially mediated the effect of race/ethnicity on in-hospital mortality between 2.6% (P = 0.64) and 16.8% (P = 0.029), depending on model specification. In neonatal multivariable survival analysis (n = 13,987; median follow-up: 0.70 years), the lowest COI quintile had poorer survival (HR: 1.21; P = 0.04). CONCLUSIONS: Children in the lowest COI quintile are at risk for poor outcomes after CHD surgery. Disproportionally increased mortality in Black, Asian, and Other populations may be partially mediated by COI. Targeted investment in low COI neighborhoods may improve outcomes after hospital discharge. Identification of unmeasured factors to explain persistent risk attributed to race/ethnicity is an important area of future exploration.

Pulmonary Complications in Patients With Fontan Circulation: JACC Review Topic of the Week.

The Fontan operation has resulted in significant improvement in survival of patients with single ventricle physiology. As a result, there is a growing population of individuals with Fontan physiology reaching adolescence and adulthood. Despite the improved survival, there are long-term morbidities associated with the Fontan operation. Pulmonary complications are common and may contribute to both circulatory and pulmonary insufficiency, leading ultimately to Fontan failure. These complications include restrictive lung disease, sleep abnormalities, plastic bronchitis, and cyanosis. Cyanosis post-Fontan procedure can be attributed to multiple causes including systemic to pulmonary venous collateral channels and pulmonary arteriovenous malformations. This review presents the unique cardiopulmonary interactions in the Fontan circulation. Understanding the cardiopulmonary interactions along with improved recognition and treatment of pulmonary abnormalities may improve the long-term outcomes in this growing patient population. Interventions focused on improving pulmonary function including inspiratory muscle training and endurance training have shown a promising effect post-Fontan procedure.

Quantitative prediction of right ventricular and size and function from the electrocardiogram.

Background: Right ventricular ejection fraction (RVEF) and end-diastolic volume (RVEDV) are not readily assessed through traditional modalities. Deep-learning enabled 12-lead electrocardiogram analysis (DL-ECG) for estimation of RV size or function is unexplored. Methods: We trained a DL-ECG model to predict RV dilation (RVEDV>120 mL/m2), RV dysfunction (RVEF≤40%), and numerical RVEDV/RVEF from 12-lead ECG paired with reference-standard cardiac MRI (cMRI) volumetric measurements in UK biobank (UKBB; n=42,938). We fine-tuned in a multi-center health system (MSHoriginal; n=3,019) with prospective validation over 4 months (MSHvalidation; n=115). We evaluated performance using area under the receiver operating curve (AUROC) for categorical and mean absolute error (MAE) for continuous measures overall and in key subgroups. We assessed association of RVEF prediction with transplant-free survival with Cox proportional hazards models. Results: Prevalence of RV dysfunction for UKBB/MSHoriginal/MSHvalidation cohorts was 1.0%/18.0%/15.7%, respectively. RV dysfunction model AUROC for UKBB/MSHoriginal/MSHvalidation cohorts was 0.86/0.81/0.77, respectively. Prevalence of RV dilation for UKBB/MSHoriginal/MSHvalidation cohorts was 1.6%/10.6%/4.3%. RV dilation model AUROC for UKBB/MSHoriginal/MSHvalidation cohorts 0.91/0.81/0.92, respectively. MSHoriginal MAE was RVEF=7.8% and RVEDV=17.6 ml/m2. Performance was similar in key subgroups including with and without left ventricular dysfunction. Over median follow-up of 2.3 years, predicted RVEF was independently associated with composite outcome (HR 1.37 for each 10% decrease, p=0.046). Conclusions: DL-ECG analysis can accurately identify significant RV dysfunction and dilation both overall and in key subgroups. Predicted RVEF is independently associated with clinical outcome.

Management of Heart Failure With Arrhythmia in Adults With Congenital Heart Disease: JACC State-of-the-Art Review.

Together, heart failure and arrhythmia represent the most important cardiovascular sources of morbidity and mortality among adults with congenital heart disease (ACHDs). Although traditionally conceptualized as operating within 2 distinct clinical silos, these scenarios frequently coexist within the same individual; consequently the mechanistic, therapeutic, and prognostic overlap between them demands increased recognition. In fact, given the near ubiquity of heart failure and arrhythmia among ACHDs, there is perhaps no other arena within cardiology where this critical intersection is more frequently observed. Optimal care for ACHDs therefore requires a heightened awareness of the relevant interactions as well as the pharmacologic and interventional resources that are increasingly available to the treating cardiologist. This review explores and highlights the overlap between these 2 fields to recommend a parallel, yet interactive, multidisciplinary approach to clinical management. Congenital heart disease categories are broken down into their archetypal subtypes to highlight subtleties of the pathophysiology, evaluation, and therapeutic approach.

Familial Recurrence Patterns in Congenitally Corrected Transposition of the Great Arteries: An International Study.

BACKGROUND: Congenitally corrected transposition of the great arteries (ccTGA) is a rare disease of unknown cause. We aimed to better understand familial recurrence patterns. METHODS: An international, multicentre, retrospective cohort study was conducted in 29 tertiary hospitals in 6 countries between 1990 and 2018, entailing investigation of 1043 unrelated ccTGA probands. RESULTS: Laterality defects and atrioventricular block at diagnosis were observed in 29.9% and 9.3%, respectively. ccTGA was associated with primary ciliary dyskinesia in 11 patients. Parental consanguinity was noted in 3.4% cases. A congenital heart defect was diagnosed in 81 relatives from 69 families, 58% of them being first-degree relatives, including 28 siblings. The most prevalent defects in relatives were dextro-transposition of the great arteries (28.4%), laterality defects (13.6%), and ccTGA (11.1%); 36 new familial clusters were described, including 8 pedigrees with concordant familial aggregation of ccTGA, 19 pedigrees with familial co-segregation of ccTGA and dextro-transposition of the great arteries, and 9 familial co-segregation of ccTGA and laterality defects. In one family co-segregation of ccTGA, dextro-transposition of the great arteries and heterotaxy syndrome in 3 distinct relatives was found. In another family, twins both displayed ccTGA and primary ciliary dyskinesia. CONCLUSIONS: ccTGA is not always a sporadic congenital heart defect. Familial clusters as well as evidence of an association between ccTGA, dextro-transposition of the great arteries, laterality defects and in some cases primary ciliary dyskinesia, strongly suggest a common pathogenetic pathway involving laterality genes in the pathophysiology of ccTGA.

Antibiotic Use and Outcomes in Young Children Hospitalized With Uncomplicated Community-Acquired Pneumonia.

Background: We aimed to compare children aged 36 months or younger hospitalized with uncomplicated community-acquired pneumonia (CAP) who are not treated with antibiotics to those treated with antibiotics in terms of clinical features and outcome measures. Methods: Administrative data and medical record review were used to identify patients from 3 to 36 months of age hospitalized from 2011 to 2019 with uncomplicated CAP. Patients were considered treated if they received antibiotics for >2 inpatient days and/or at discharge, and not treated if they received ≤2 inpatient days and no antibiotics at discharge. Untreated patients were compared to treated patients based on demographic features, clinical and laboratory results, and outcomes of interest, including illness severity, length of stay, and 30-day hospital readmissions. Results: Three hundred twenty-two CAP cases were included; 266 (83%) received antibiotics for >48 hours and/or at discharge. Fifty-six patients received ≤2 inpatient days of antibiotics and no antibiotics at discharge; the majority received no inpatient antibiotics. There were no differences between the 2 groups in illness severity, length of stay, or hospital readmissions. The proportion of patients treated with antibiotics decreased from 88% (2011-2013) to 66% during the most recent years studied (2017-2019). Conclusions: There was no difference in outcome of uncomplicated CAP in previously healthy children <36 months of age between those treated and not treated with antibiotics. Additional tools are needed to facilitate identification of viral CAP in young children and decrease unnecessary antibiotic use.

Noninvasive Prediction of Congenital Cytomegalovirus Infection After Maternal Primary Infection.

OBJECTIVE: To develop and internally validate a noninvasive method for the prediction of congenital cytomegalovirus (CMV) infection after primary maternal CMV infection. METHODS: We conducted a secondary analysis of a multicenter randomized placebo-controlled trial of CMV hyperimmune globulin to prevent congenital infection. Women were eligible if they had primary CMV infection, defined as detectable plasma CMV-specific immunoglobulin (Ig)M and CMV-specific IgG with avidity less than 50% before 24 weeks of gestation or IgG seroconversion before 28 weeks, and were carrying a singleton fetus without ultrasonographic findings suggestive of CMV infection. Antibody assays were performed in a single reference laboratory. Congenital infection was defined as CMV detection in amniotic fluid, neonatal urine or saliva, or postmortem tissue. Using backward elimination, we developed logit models for prediction of congenital infection using factors known at randomization. The performance of the model was assessed using leave-one-out cross-validation (a method of internal validation). RESULTS: Of 399 women enrolled in the trial, 344 (86%) had informative data for this analysis. Congenital infection occurred in 68 pregnancies (20%). The best performing model included government-assisted insurance, IgM index 4.5 or higher, IgG avidity less than 32%, and whether CMV was detectable by polymerase chain reaction in maternal plasma at the time of randomization. Cross-validation showed an average area under the curve of 0.76 (95% CI 0.70-0.82), indicating moderate discriminatory ability. More parsimonious one-, two-, and three-factor models performed significantly less well than the four-factor model. Examples of prediction with the four-factor model: for a woman with government-assisted insurance, avidity less than 32%, IgM index 4.5 or higher, and detectable plasma CMV, probability of congenital infection was 0.69 (95% CI 0.53-0.82); for a woman with private insurance, avidity 32% or greater, IgM index less than 4.5, and undetectable plasma CMV, probability of infection was 0.03 (95% CI 0.02-0.07). CONCLUSION: We developed models to predict congenital CMV infection in the presence of primary maternal CMV infection and absence of ultrasonographic findings suggestive of congenital infection. These models may be useful for patient counseling and decision making.

A Trial of Hyperimmune Globulin to Prevent Congenital Cytomegalovirus Infection.

BACKGROUND: Primary cytomegalovirus (CMV) infection during pregnancy carries a risk of congenital infection and possible severe sequelae. There is no established intervention for preventing congenital CMV infection. METHODS: In this multicenter, double-blind trial, pregnant women with primary CMV infection diagnosed before 24 weeks' gestation were randomly assigned to receive a monthly infusion of CMV hyperimmune globulin (at a dose of 100 mg per kilogram of body weight) or matching placebo until delivery. The primary outcome was a composite of congenital CMV infection or fetal or neonatal death if CMV testing of the fetus or neonate was not performed. RESULTS: From 2012 to 2018, a total of 206,082 pregnant women were screened for primary CMV infection before 23 weeks of gestation; of the 712 participants (0.35%) who tested positive, 399 (56%) underwent randomization. The trial was stopped early for futility. Data on the primary outcome were available for 394 participants; a primary outcome event occurred in the fetus or neonate of 46 of 203 women (22.7%) in the group that received hyperimmune globulin and of 37 of 191 women (19.4%) in the placebo group (relative risk, 1.17; 95% confidence interval [CI] 0.80 to 1.72; P = 0.42). Death occurred in 4.9% of fetuses or neonates in the hyperimmune globulin group and in 2.6% in the placebo group (relative risk, 1.88; 95% CI, 0.66 to 5.41), preterm birth occurred in 12.2% and 8.3%, respectively (relative risk, 1.47; 95% CI, 0.81 to 2.67), and birth weight below the 5th percentile occurred in 10.3% and 5.4% (relative risk, 1.92; 95% CI, 0.92 to 3.99). One participant in the hyperimmune globulin group had a severe allergic reaction to the first infusion. Participants who received hyperimmune globulin had a higher incidence of headaches and shaking chills while receiving infusions than participants who received placebo. CONCLUSIONS: Among pregnant women, administration of CMV hyperimmune globulin starting before 24 weeks' gestation did not result in a lower incidence of a composite of congenital CMV infection or perinatal death than placebo. (Funded by the Eunice Kennedy Shriver National Institute of Child Health and Human Development and the National Center for Advancing Translational Sciences; ClinicalTrials.gov number, NCT01376778.).

Drip System for Admissions to Resident Teams: Impact on Workload and Education.

OBJECTIVES: Assigning patients to a call team every fourth day (bolus system) caused the maldistribution of patients among resident teams and required additional faculty effort for overflow patient care. We changed to a continuous daily rotation (drip system) and examined the effect on clinical workload among resident teams, resident education, and faculty utilization. METHODS: This is a retrospective study based on the daily records of 7 am team census, the attending physician schedules for a pediatric hospital medicine service with 5 teams, and the measures of resident education, including noon conference attendance, scores on in-service examinations, and duty hour violations. Data from the bolus system (May 2014-June 2015) were compared with the drip system (May 2016-June 2017). RESULTS: Data from 348 bolus days and 338 drip days were analyzed. There was a decrease in interteam variation from 6.2 to 3.9 patients (P < 0.001). There were fewer days with the following: large interteam variation (143 to 25, P < 0.001), days with resident teams at or above capacity (26 to 11, P = 0.01), resident teams below a minimum 7 am census (133 to 18, P < 0.001), and days when additional faculty were pulled for clinical care (61 to 9, P < 0.001). Resident noon conference attendance was unchanged and there was no adverse effect on examination scores or duty hour violations. CONCLUSIONS: Changing from a bolus to a drip model for admissions to inpatient teams resulted in a more even distribution of the workload and a more efficient use of physician resources without negatively affecting resident education.

Antibody binding to native cytomegalovirus glycoprotein B predicts efficacy of the gB/MF59 vaccine in humans.

Human cytomegalovirus (CMV) is the most common infectious cause of infant brain damage and posttransplant complications worldwide. Despite the high global burden of disease, vaccine development to prevent infection remains hampered by challenges in generating protective immunity. The most efficacious CMV vaccine candidate tested to date is a soluble glycoprotein B (gB) subunit vaccine with MF59 adjuvant (gB/MF59), which achieved 50% protection in multiple historical phase 2 clinical trials. The vaccine-elicited immune responses that conferred this protection have remained unclear. We investigated the humoral immune correlates of protection from CMV acquisition in populations of CMV-seronegative adolescent and postpartum women who received the gB/MF59 vaccine. We found that gB/MF59 immunization elicited distinct CMV-specific immunoglobulin G (IgG)-binding profiles and IgG-mediated functional responses in adolescent and postpartum vaccinees, with heterologous CMV strain neutralization observed primarily in adolescent vaccinees. Using penalized multiple logistic regression analysis, we determined that protection against primary CMV infection in both cohorts was associated with serum IgG binding to gB present on a cell surface but not binding to the soluble vaccine antigen, suggesting that IgG binding to cell-associated gB is an immune correlate of vaccine efficacy. Supporting this, we identified gB-specific monoclonal antibodies that differentially recognized soluble or cell-associated gB, revealing that there are structural differences in cell-associated and soluble gB are relevant to the generation of protective immunity. Our results highlight the importance of the native, cell-associated gB conformation in future CMV vaccine design.

Multisystem Inflammatory Syndrome in Children Associated with Severe Acute Respiratory Syndrome Coronavirus 2 Infection (MIS-C): A Multi-institutional Study from New York City.

OBJECTIVE: To assess clinical characteristics and outcomes of severe acute respiratory syndrome coronavirus 2-associated multisystem inflammatory syndrome in children (MIS-C). STUDY DESIGN: Children with MIS-C admitted to pediatric intensive care units in New York City between April 23 and May 23, 2020, were included. Demographic and clinical data were collected. RESULTS: Of 33 children with MIS-C, the median age was 10 years; 61% were male; 45% were Hispanic/Latino; and 39% were black. Comorbidities were present in 45%. Fever (93%) and vomiting (69%) were the most common presenting symptoms. Depressed left ventricular ejection fraction was found in 63% of patients with median ejection fraction of 46.6% (IQR, 39.5-52.8). C-reactive protein, procalcitonin, d-dimer, and pro-B-type natriuretic peptide levels were elevated in all patients. For treatment, intravenous immunoglobulin was used in 18 (54%), corticosteroids in 17 (51%), tocilizumab in 12 (36%), remdesivir in 7 (21%), vasopressors in 17 (51%), mechanical ventilation in 5 (15%), extracorporeal membrane oxygenation in 1 (3%), and intra-aortic balloon pump in 1 (3%). The left ventricular ejection fraction normalized in 95% of those with a depressed ejection fraction. All patients were discharged home with median duration of pediatric intensive care unit stay of 4.7 days (IQR, 4-8 days) and a hospital stay of 7.8 days (IQR, 6.0-10.1 days). One patient (3%) died after withdrawal of care secondary to stroke while on extracorporeal membrane oxygenation. CONCLUSIONS: Critically ill children with coronavirus disease-2019-associated MIS-C have a spectrum of severity broader than described previously but still require careful supportive intensive care. Rapid, complete clinical and myocardial recovery was almost universal.

Loss of ventricular preexcitation during noninvasive testing does not exclude high-risk accessory pathways: A multicenter study of WPW in children.

BACKGROUND: Abrupt loss of ventricular preexcitation on noninvasive evaluation, or nonpersistent preexcitation, in Wolff-Parkinson-White syndrome (WPW) is thought to indicate a low risk of life-threatening events. OBJECTIVE: The purpose of this study was to compare accessory pathway (AP) characteristics and occurrences of sudden cardiac arrest (SCA) and rapidly conducted preexcited atrial fibrillation (RC-AF) in patients with nonpersistent and persistent preexcitation. METHODS: Patients 21 years or younger with WPW and invasive electrophysiology study (EPS) data, SCA, or RC-AF were identified from multicenter databases. Nonpersistent preexcitation was defined as absence/sudden loss of preexcitation on electrocardiogram, Holter monitoring, or exercise stress test. RC-AF was defined as clinical preexcited atrial fibrillation with shortest preexcited R-R interval (SPERRI) ≤ 250 ms. AP effective refractory period (APERP), SPERRI at EPS , and shortest preexcited paced cycle length (SPPCL) were collected. High-risk APs were defined as APERP, SPERRI, or SPPCL ≤ 250 ms. RESULTS: Of 1589 patients, 244 (15%) had nonpersistent preexcitation and 1345 (85%) had persistent preexcitation. There were no differences in sex (58% vs 60% male; P=.49) or age (13.3±3.6 years vs 13.1±3.9 years; P=.43) between groups. Although APERP (344±76 ms vs 312±61 ms; P<.001) and SPPCL (394±123 ms vs 317±82 ms; P<.001) were longer in nonpersistent vs persistent preexcitation, there was no difference in SPERRI at EPS (331±71 ms vs 316±73 ms; P=.15). Nonpersistent preexcitation was associated with fewer high-risk APs (13% vs 23%; P<.001) than persistent preexcitation. Of 61 patients with SCA or RC-AF, 6 (10%) had nonpersistent preexcitation (3 SCA, 3 RC-AF). CONCLUSION: Nonpersistent preexcitation was associated with fewer high-risk APs, though it did not exclude the risk of SCA or RC-AF in children with WPW.

Cytomegalovirus infection during pregnancy: state of the science.

Cytomegalovirus is the most common congenital infection, affecting 0.5-2% of all live births and the main nongenetic cause of congenital sensorineural hearing loss and neurological damage. Congenital cytomegalovirus can follow maternal primary infection or nonprimary infection. Sensorineurological morbidity is confined to the first trimester with up to 40-50% of infected neonates developing sequelae after first-trimester primary infection. Serological testing before 14 weeks is critical to identify primary infection within 3 months around conception but is not informative in women already immune before pregnancy. In Europe and the United States, primary infection in the first trimester are mainly seen in young parous women with a previous child younger than 3 years. Congenital cytomegalovirus should be evoked on prenatal ultrasound when the fetus is small for gestation and shows echogenic bowel, effusions, or any cerebral anomaly. Although the sensitivity of routine ultrasound in predicting neonatal symptoms is around 25%, serial targeted ultrasound and magnetic resonance imaging of known infected fetuses show greater than 95% sensitivity for brain anomalies. Fetal diagnosis is done by amniocentesis from 17 weeks. Prevention consists of both parents avoiding contact with body fluids from infected individuals, especially toddlers, from before conception until 14 weeks. Candidate vaccines failed to provide more than 75% protection for >2 years in preventing cytomegalovirus infection. Medical therapies such as cytomegalovirus hyperimmune globulins aim to reduce the risk of vertical transmission but 2 randomized controlled trials have not found any benefit. Valaciclovir given from the diagnosis of primary infection up to amniocentesis decreased vertical transmission rates from 29.8% to 11.1% in the treatment group in a randomized controlled trial of 90 pregnant women. In a phase II open-label trial, oral valaciclovir (8 g/d) given to pregnant women with a mildly symptomatic fetus was associated with a higher chance of delivering an asymptomatic neonate (82%), compared with an untreated historical cohort (43%). Valganciclovir given to symptomatic neonates is likely to improve hearing and neurological symptoms, the extent of which and the duration of treatment are still debated. In conclusion, congenital cytomegalovirus infection is a public health challenge. In view of recent knowledge on diagnosis and pre- and postnatal management, health care providers should reevaluate screening programs in early pregnancy and at birth.

Can the 12-lead ECG distinguish RVOT from aortic cusp PVCs in pediatric patients?

BACKGROUND: The ability to differentiate right ventricular outflow tract (RVOT) from coronary cusp (CC) site of origin (SOO) by 12-lead ECG in pediatric patients may impact efficacy and procedural time. The objective of this study was to predict RVOT versus CC SOO by ECG in pediatric patients. METHODS: Pediatric patients (<21 years) without structural heart disease with RVOT or CC premature ventricular contraction (PVC) ablations performed (2014-2018) were evaluated through multi-institution retrospective review. Demographics, ECG PVC parameters, ablation site, recurrence, and repeat procedures were collected. RESULTS: Thirty-seven patients were evaluated (mean age 14.6 years, weight 60.6 kg): 11 CC and 26 RVOT PVC SOO. CC PVCs were less likely to exhibit left bundle branch block (64% vs 100%, P = .005), had larger R-wave amplitude in V1 (0.27 vs 0.11 mV, P = .03), larger R/S ratio in V1 (0.37 vs 0.09, P = .003), and had precordial transition in V3 or earlier (73% vs 15%, P = .002). A composite score was created with the following variables: isodiphasic or positive QRS in V1, R/S ratio in V1 > 0.05, S wave in V1 < 0.9 mV, and precordial transition at or before V3. Composite score ≥ 2 was associated with a CC SOO (OR 42.0, P = .001, and AUC 0.86). CONCLUSIONS: 12-lead ECG of PVCs from the CC was associated with larger V1 R-wave amplitude, larger R/S ratio in V1, and precordial transition at or before V3. A composite score may help predict PVC/VT arising from the CC.

Newborn With Severely Depressed Left Ventricular Function: Acute Myocardial Infarction in a Newborn.

We describe a rare case of spontaneous coronary artery thrombosis in a newborn leading to rapid severe ventricular dysfunction. Early diagnosis is critical and management strategies are varied including hemodynamic support with extracorporeal membrane oxygenation, systemic/local thrombolytic therapy with tissue plasminogen activator, or surgical thrombectomy. (Level of Difficulty: Advanced.).

"If at first you don't succeed": repeat ablations in young patients with supraventricular tachycardia.

PURPOSE: Success rates for catheter ablation of supraventricular tachycardia (SVT) in the young exceed 90%. While studies have described reasons for initial ablation failure, less is known about outcomes of repeat ablation attempts. The purpose of this study was to report acute and mid-term success rates for second ablation attempts in young patients, as well as to analyze factors that may affect these outcomes. METHODS: Retrospective single-center study of all patients undergoing a second ablation attempt for WPW (Wolff-Parkinson-White) or SVT from 2008 to 2017. Inclusion criteria are all patients < 21 years old at the time of their first ablation who underwent a second ablation attempt. An intention to treat analysis was performed. RESULTS: Fifty-five patients met inclusion criteria, with a median age of 15 years (IQR 12-16). The most common arrhythmia mechanisms at repeat procedure were single accessory pathways (n = 32, 58%) and AVNRT (n = 14, 25%). Six patients (11%) were found to have a different SVT mechanism than at initial ablation. Acute success at repeat ablation was achieved in 48 patients (87%). At mid-term follow-up (10.5 months, IQR 0.6-25), four patients (8% of acute successes) experienced SVT recurrence. The overall success rate of repeat ablations, accounting for acute and mid-term failures, was 80%. CONCLUSIONS: In this report of 55 young patients who underwent repeat ablation for WPW and/or SVT, acute and mid-term success rates were 87% and 80%, respectively. These data may help inform decision-making when caring for patients with persistent or recurrent SVT after an initial ablation attempt.

Validation of a novel automated signal analysis tool for ablation of Wolff-Parkinson-White Syndrome.

BACKGROUND: In previous pilot work we demonstrated that a novel automated signal analysis tool could accurately identify successful ablation sites during Wolff-Parkinson-White (WPW) ablation at a single center. OBJECTIVE: We sought to validate and refine this signal analysis tool in a larger multi-center cohort of children with WPW. METHODS: A retrospective review was performed of signal data from children with WPW who underwent ablation at two pediatric arrhythmia centers from 2008-2015. All patients with WPW ≤ 21 years who underwent invasive electrophysiology study and ablation with ablation signals available for review were included. Signals were excluded if temperature or power delivery was inadequate or lesion time was < 5 seconds. Ablation lesions were reviewed for each patient. Signals were classified as successful if there was loss of antegrade and retrograde accessory pathway (AP) conduction or unsuccessful if ablation did not eliminate AP conduction. Custom signal analysis software analyzed intracardiac electrograms for amplitudes, high and low frequency components, integrated area, and signal timing components to create a signal score. We validated the previously published signal score threshold 3.1 in this larger, more diverse cohort and explored additional scoring options. Logistic regression with lasso regularization using Youden's index criterion and a cost-benefit criterion to identify thresholds was considered as a refinement to this score. RESULTS: 347 signals (141 successful, 206 unsuccessful) in 144 pts were analyzed [mean age 13.2 ± 3.9 years, 96 (67%) male, 66 (45%) left sided APs]. The software correctly identified the signals as successful or unsuccessful in 276/347 (80%) at a threshold of 3.1. The performance of other thresholds did not significantly improve the predictive ability. A signal score threshold of 3.1 provided the following diagnostic accuracy for distinguishing a successful from unsuccessful signal: sensitivity 83%, specificity 77%, PPV 71%, NPV 87%. CONCLUSIONS: An automated signal analysis software tool reliably distinguished successful versus unsuccessful ablation electrograms in children with WPW when validated in a large, diverse cohort. Refining the tools using an alternative threshold and statistical method did not improve the original signal score at a threshold of 3.1. This software was effective across two centers and multiple operators and may be an effective tool for ablation of WPW.