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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. [ABSTRACT FROM AUTHOR]

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.ahj.2010.07.009 Byline: Maria E. Lagopoulos, Cedric Manlhiot, Brian W. McCrindle, Edgar T. Jaeggi, Mark K. Friedberg, Lynne E. Nield Abstract: We sought to investigate the influence of prenatal diagnosis and risk factors for adverse outcomes in double outlet right ventricle (DORV) not associated with heterotaxy. Article History: Received 26 March 2010; Accepted 2 July 2010

Pathogenic heterozygous variants in the NOTCH1 gene are known to be associated with both left and right‐sided congenital cardiac anomalies with strikingly incomplete penetrance and variable phenotypic expressivity. De novo NOTCH1 whole gene deletion has been reported rarely in the literature and its association with cardiac defects is less well established. Here, we report four cases of NOTCH1 gene deletion from two families associated with a spectrum of congenital heart defects from bicuspid aortic valve to complex cardiac anomalies. This is the first description of a familial NOTCH1 deletion, showing apparently high penetrance, which may be unique to this mechanism of disease. Immunohistochemical staining of cardiac tissue demonstrated reduced levels of NOTCH1 expression in both the left and right ventricular outflow tracts. These cases suggest that haploinsufficiency caused by NOTCH1 gene deletion is associated with both mild and severe cardiac defects, similar to those caused by pathogenic variants in the gene, but with apparently higher, if not complete, penetrance. [ABSTRACT FROM AUTHOR]

Aims: Transposition of the great arteries (TGA) is generally repaired using the arterial switch operation (ASO) involving coronary transfer. The objective of this prospective study was to determine whether specific coronary Doppler patterns intra-operatively predicted adverse early myocardial events. Methods and results: Patients < 3 months old with TGA undergoing the ASO were eligible. All patients (when feasible) underwent an intra-operative transoesophageal echo (TEE) plus an epicardial echo and had pre-op, early post-op, pre-discharge and follow-up functional echocardiograms. The primary endpoint was a composite myocardial ischaemic event (any of: post-operative ST changes, ventricular tachycardia, need for extracorporeal membrane oxygenation (ECMO). Associations of coronary Doppler flow patterns with outcomes were modelled with logistic regression models. From May 2009 to December 2012, 40 patients (29 male, birth weight 3.29 ± 0.58 kg) were recruited. n = 32 had TEE + epicardial, four TEE only, four epicardial only. Seven (18%) patients had an adverse myocardial event (five ST changes, two ventricular tachycardia (one also ECMO). There was one death. n = 3 had a coronary artery (CA) revision post-operatively, and three had re-operation for non-CA causes. By TEE, flow reversal in the left coronary artery was associated with the composite endpoint [Odds Ratio (OR) 31.5, P = 0.004], and for chest open > 3 days (OR 6.67, P = 0.0537). Coronary Doppler flow patterns were similar by TEE and epicardial echo. The tissue Doppler parameters showed an early post-op decrease (P < 0.001 for all measures), with full recovery at follow-up in 31/37 cases. Conclusion: Intra-operative evaluation of coronary artery flow patterns should be considered for patients undergoing the ASO. [ABSTRACT FROM AUTHOR]

Objective: Hypoplastic left heart syndrome is frequently diagnosed prenatally with variable benefit. We performed a systematic review to evaluate the impact of fetal diagnosis; the primary objective was to evaluate impact on mortality.Methods: Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were followed. Seven databases were searched. Meta-analysis was performed using a random effects model to evaluate the effect of fetal diagnosis on mortality.Results: Literature search revealed 2124 titles and abstracts for screening; 21 full texts were reviewed. Six studies and one abstract were included. Preoperative mortality in 609 neonates (228 prenatal and 381 postnatal) was evaluated. There were 11 deaths in prenatally diagnosed neonates versus 16 deaths in postnatally diagnosed neonates (OR 0.67, 95% CI 0.22-2.01, p = 0.48). Neonates with fetal diagnosis had less preoperative acidosis (mean difference 0.07, 95% CI 0.05, 0.1, p < 0.01) and required less inotropic support (OR 0.16, 95% CI 0.04, 0.7, p = 0.01). Post Stage I, there were 47 deaths in 227 prenatally diagnosed neonates versus 78 deaths in 299 postnatally diagnosed neonates (OR 0.84, 95% CI 0.43, 1.62, p = 0.59).Conclusions: There is no significant impact of prenatal diagnosis of hypoplastic left heart syndrome on preoperative or post Stage I mortality. Neonates with prenatal diagnosis were hemodynamically more stable. © 2016 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Aim: Public reporting of procedural outcomes leads to risk averse behavior because physicians do not believe the scores account for patient risk. We investigated the effects of more aggressive percutaneous coronary intervention (PCI) practice on risk-adjusted mortality. Methods & results: 8935 PCI were performed. Risk adjustment was performed with the New York State PCI risk score. The cohort was divided into two eras based on programs implemented to promote more aggressive care. Between eras, overall adjusted mortality ratios rose from 0.66 to 0.90 (observed/predicted, p = 0.02), despite evidence supporting consistent procedural quality. Conclusion: Evidence-based changes in PCI practice were associated with worsening risk-adjusted procedural mortality. These data are consistent with physician beliefs regarding risk-adjusted outcome measures. [ABSTRACT FROM AUTHOR]

: ObjectivesThis study was designed to document the association of endocardial fibroelastosis (EFE) and maternal autoantibodies.: BackgroundNeonatal lupus erythematosus is associated with the transplacental passage of maternal anti-Ro and anti-La antibodies, leading to complete atrioventricular block (CAVB). In some cases, CAVB is associated with EFE. Isolated EFE may be independently related to maternal anti-Ro and anti-La antibodies.: MethodsWe identified three cases (one fetus and two infants, all female) of isolated EFE in infants born to autoantibody-positive mothers in the absence of CAVB. Demographics, echocardiograms, and pathology were reviewed. Immunohistochemical analyses for immunoglobulin (Ig)G, IgM, IgA, T-cell, B-cell, and terminal deoxynucleoleotidyl transferase-mediated dUTP-biotin nick end-labeling (TUNEL) (test for cell apoptosis) staining were performed on multiple sections of the heart in each case and compared with negative controls.: ResultsTwo cases died and one received a cardiac transplant. All three cases had histologically confirmed EFE. All cases demonstrated significant diffuse IgG infiltration. To a lesser extent, myocardial tissue was also positive for IgM, CD43, and Granzyme B. None of the specimens were TUNEL positive.: ConclusionsThese are the first reported cases of isolated EFE associated with maternal anti-Ro and anti-La antibodies in the absence of CAVB. The diffuse deposition of IgG and the presence of a T-cell infiltrate throughout the myocardium suggest that the transplacental passage of maternal autoantibodies induces an immune reaction within the myocardium, leading to isolated EFE. Autoantibody-mediated EFE may be an etiologic factor in cases of fetal and neonatal “idiopathic” dilated cardiomyopathy. [Copyright &y& Elsevier]