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Objectives: We sought to describe the implementation and effectiveness of a statewide fetal tele-echocardiography program serving a resource-limited population., Study Design: In 2009, our heart center established six satellite clinics for fetal tele-echocardiography around the state. We retrospectively reviewed all fetal tele-echocardiograms performed through 2018. Yearly statewide prenatal detection rates of operable congenital heart disease were queried from the Society of Thoracic Surgeons database., Result: In 1164 fetuses, fetal tele-echocardiography identified all types of congenital heart disease, with a sensitivity of 74% and specificity of 97%. For the detection of ductal-dependent congenital heart disease, fetal tele-echocardiography was 100% sensitive and specific. Between 2009 and 2018, annual statewide prenatal detection rates of congenital heart disease requiring heart surgery in the first 6 months of life rose by 159% (17-44%; R 2  = 0.88, p < 0.01)., Conclusions: The present study provides a framework for an effective, large-scale fetal tele-echocardiography program.

Appropriate Use Criteria (AUC) for the initial use of outpatient pediatric echocardiography were established to aid all clinicians in the evaluation of children with possible heart disease, and limit low diagnostic yield studies. We sought to (1) assess PCPs' and trainees' awareness of the AUC document; (2) compare their knowledge of appropriate echocardiogram ordering with that of pediatric cardiologists; and (3) identify additional medical and nonmedical factors affecting PCP echocardiogram ordering. An online survey with clinical scenarios derived from the AUC guidelines was distributed to PCPs and trainees in Arkansas, and pediatric cardiologists from Arkansas Children's Hospital and Cincinnati Children's Hospital Medical Center. Respondents were also asked to rate whether additional medical and nonmedical factors have "no," "mild," "moderate," or "major" impact on PCP echocardiogram ordering. Survey data were collected from 148 respondents. Awareness of the AUC was significantly lower in PCPs (21.4%) and trainees (14%) than in pediatric cardiologists (90.5%, p <0.001). For all rarely appropriate clinical scenarios, cardiologists had stronger agreement with the AUC document (90.9%) than did the PCP group (50.3%) and trainees (53.3%, p <0.001). The strongest additional factors affecting PCP echocardiogram ordering were parental anxiety, difficulty distinguishing innocent from pathologic murmurs, and legal implications of a missed diagnosis. In conclusion, PCPs and trainees are largely unaware of the existence of the pediatric echocardiogram AUC. Educational strategies to improve appropriate echocardiogram ordering should address not only increasing awareness of AUC, but also other factors affecting decision-making., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: To test the hypothesis that fetuses exposed to maternal preeclampsia or chronic hypertension have deranged development of cardiac time intervals., Study Design: Pregnancies were divided into three groups: Intrauterine Growth Restricted (IUGR), Hypertensive, and Normal. Each group's mean fetal cardiac time intervals (P, PR, QRS and RR) derived by magnetocardiography were calculated using an analysis of covariance model's regression-adjusted estimates for a gestational age of 35 weeks., Results: We reviewed 141 recordings from 21 IUGR, 46 Hypertensive and 74 Normal patients. The IUGR, Hypertensive and Normal groups, respectively, had adjusted mean intervals in milliseconds of 66.4, 66.8 and 76.2 for P (P=0.001), 95.9, 101.6 and 109.6 for PR (P=0.002), 77.2, 78.7 and 78.7 for QRS (P=0.81) and 429.8, 429.2 and 428.5 for RR (P=0.97)., Conclusion: P and PR intervals are abbreviated in normotrophic fetuses exposed to maternal hypertension, suggesting shortened atrioventricular conduction times.

Congenital heart defects (CHDs) are a major cause of infant mortality. Most CHDs are thought to result from genetic, lifestyle, and environmental factors that include maternal obesity, diabetes, toxicant exposure, and alterations in anti-oxidant capacity. Since these well-documented risk factors are also associated with immune dysregulation, we sought to compare the maternal immune response in mothers carrying a fetus with a CHD with those mothers whose pregnancies were not affected by any birth defect. We conducted a case-control study to examine the maternal cytokine profile using multiplex technology in pregnant mothers (subject mean=26 weeks' gestation). This investigation revealed that whole blood cultures derived from case mothers produced higher levels of certain cytokines and chemokines compared with cultures from control subjects when activated with mitogen. Cultures from case subjects produced higher levels of IL-10, IL-13, IL-4, IL-5, IL-17, and IL-6, when stimulated with mitogen compared with control subjects. Plasma levels of chemokine MIP-1α were higher in cases compared with controls. In contrast, C-reactive protein levels were not statistically different. These results demonstrate the need to further examine the maternal cytokine signature in CHD-affected pregnancies. This information could pave the way toward maternal immunotherapeutic intervention to prevent CHDs, and novel biomarker discovery to improve pre-natal diagnosis., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

The absence of the pulmonary valve in connection with tricuspid atresia and intact ventricular septum is a rare malformation, associated with a poor prognosis. The right ventricle is severely hypertrophied, resembling a cardiac mass protruding into the left ventricular outflow tract. We report such a case that underwent successful palliation with a Blalock-Taussig shunt followed by a superior cavopulmonary anastomosis., (© 2011 Copyright the Authors. Congenital Heart Disease © 2011 Wiley Periodicals, Inc.)

Objective: The purpose of this study was to compare cardiac dynamics of high-risk and low-risk fetuses using beat to beat variability., Study Design: This study obtained 214 fetal magnetocardiography recordings from a group of high-risk fetuses with maternal conditions associated with placental insufficiency with the risk of developing intrauterine growth restriction or were already diagnosed with intrauterine growth restriction. For purpose of comparison, another 136 recordings were obtained from low-risk fetuses. The cardiac beat-to-beat intervals computed as RR intervals were analyzed from both groups using the mean and median interval, standard deviation of normal-to-normal intervals, root mean square of the successive differences, and the fraction of the normal-to-normal intervals (pNNx) that differ by more than the chosen tolerance level of 'x' milliseconds from the previous normal-to-normal intervals. Tests for significance between high-risk and low-risk fetuses were calculated using an independent samples t test within the following gestational age groups, 27-30 weeks, 31-35 weeks, and 36-40 weeks., Results: The root mean square of the successive differences, and the pNNx showed a significant difference between the low-risk and high-risk fetuses in the 31-35-week and the 36-40-week divisions. In the 31-35-week age division, low-risk fetuses had significantly lower root mean square of the successive differences, and pNN values than the high risk, but in the 36-40-week division, the low-risk fetuses had significantly higher root mean square of the successive differences, and pNN values., Conclusion: Cardiac dynamics for fetuses of mothers at risk for placental insufficiency mature quicker than fetuses not at risk for placental insufficiency.

Background: Right ventricular apical pacing leads to global left ventricular dysfunction. Assessment of regional myocardial function may allow for earlier detection of pacing-induced myocardial remodeling. The aim of this study was to evaluate regional myocardial function in paced children with congenital complete atrioventricular block (CCAVB)., Methods: We investigated regional myocardial function using strain echocardiography and tissue velocity imaging in 10 paced children with CCAVB and in 14 age-matched healthy children. Echocardiograms of the paced children were obtained during intrinsic junctional rhythm., Results: All patients had normal biventricular size and global function. The time from R wave to peak regional strain rate and the time from R wave to peak regional myocardial tissue velocity were measured in nine segments. The differences between maximum and minimum values were used as a measure of contraction delay (dSR and dTV). Study group patients had significantly higher dSR and dTV values compared to the control group. Mean peak strain rates and peak tissue velocities obtained from same segments were significantly decreased in the study group compared to the control group. Regional dysfunction was more prominent in the areas of ventricular pacing. No significant correlation is found between the duration of pacing (2.5-18 years) and dSR, dTV, mean peak strain rates, or mean peak tissue velocities., Conclusion: Pacing leads to regional myocardial dysfunction that can be detected with strain echocardiography and tissue velocity imaging during intrinsic junctional rhythm in children with CCAVB. Pacing-induced regional myocardial remodeling does not seem to progress with long-term right ventricular pacing in children with CCAVB.

Background: Fetal magnetocardiography is a noninvasive technique capable of identifying fetal arrhythmias and can simultaneously characterize the cardiac rhythm of the mother and fetus., Cases: Three patients, two singleton pregnancies and one twin pregnancy, were admitted for evidence of fetal supraventricular tachycardia. Fetal magnetocardiography was used to monitor the effects of flecainide therapy on the fetus and mother. Two singleton pregnancy fetuses showed improved heart rate and cardiac rhythm within 1 week of the initial dosing. Maintenance dosages controlled the conditions thereafter. Flecainide slowed the supraventricular tachycardia in the twin subject, but magnetocardiography revealed maternal adverse effects necessitating termination of flecainide therapy., Conclusion: Magnetocardiography is a valuable tool for rhythm diagnosis and for monitoring the maternal and fetal cardiac rhythms in a patient undergoing flecainide therapy for fetal supraventricular tachycardia.

A female neonate presented with evidence of a massive ventilation-perfusion mismatch. She was subsequently found to have a saddle pulmonary embolus. The infant successfully underwent surgical pulmonary embolectomy.

Cardiac complications of the ketogenic diet, in the absence of selenium deficiency, have not been reported. Twenty patients on the ketogenic diet at one institution were investigated. Prolonged QT interval (QTc) was found in 3 patients (15%). There was a significant correlation between prolonged QTc and both low serum bicarbonate and high beta-hydroxybutyrate. In addition, three patients had evidence of cardiac chamber enlargement. One patient with severe dilated cardiomyopathy and prolonged QTc normalized when the diet was discontinued.