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Cardiac malposition is a rare but important finding when detected on fetal ultrasound. The purpose of this study was to evaluate the incidence of fetal cardiac malposition, associated abnormalities, and clinical outcome in a tertiary-care medical center. Records of fetuses (1993 to 2006) with dextroposition, dextrocardia, mesocardia, ectopia cordis, or heterotaxy were reviewed. The presence of congenital heart disease (CHD), extracardiac anomalies, and outcome were noted. Cardiac malposition was present in 101 fetuses among a total of 3313 (3%) pregnancies. In 78 (78%) patients, the heart was positioned in the right hemithorax. Of those, 26 (33%) had dextrocardia (CHD = 21), and 52 (67%) had dextroposition (CHD = 14). Sixteen (16%) patients had mesocardia (CHD = 8), and 7 (7%) had ectopia cordis (CHD = 6). The majority (58%) of fetal cardiac malposition was caused by intrathoracic masses. Concomitant CHD occurred in 50%. Outcome was available in 97%. The perinatal and neonatal mortality rate was 30%; the elective termination rate was 4%. Patients with CHD had higher mortality rates. The highest mortality rates occurred in ectopia cordis and combined disease of diaphragmatic hernia and CHD. [ABSTRACT FROM AUTHOR] more...

Little is known about the characteristics and outcomes of fetuses with pericardial effusions (PEs); therefore, this study sought to identify factors associated with fetal PEs and the natural histories and outcomes of fetuses with PEs. Large PEs are associated with a greater likelihood of structural heart disease, impaired cardiac function, and chromosomal abnormalities, and PEs with hydrops or extracardiac malformations are associated with death. Most fetal PEs resolve, and fetuses with isolated PEs have a very good prognosis. [ABSTRACT FROM AUTHOR] more...

The novel Coronavirus Disease 2019 (COVID-19), caused by the SARS-CoV-2 virus, created a need for evidence-based guidelines for the evaluation, management, and follow-up after infection. Data have become rapidly available, creating a challenge for medical providers to stay abreast of the ever-evolving recommendations. This document, written collaboratively by pediatric cardiovascular experts, pediatricians, and sports medicine specialists, is focused on SARS-- CoV-2-related pediatric cardiac manifestations. It aims to provide a systemic review of high-yield literature related to all cardiovascular entities as a tool for primary pediatric clinicians to utilize as they consider the cardiac consequences of acute SARS-CoV-2 infection, MIS-C, vaccine-related myocarditis, return-to-play, and long COVID-19 syndrome., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.) more...

While significant progress has been made in reducing disparities within the US health care system, notable gaps remain. This article explores existing disparities within pediatric congenital heart disease care. Congenital heart disease, the most common birth defect and a leading cause of infant death, has garnered substantial attention, revealing certain disparities within the US health care system. Factors such as race, ethnicity, insurance coverage, socioeconomic status, and geographic location are all commonalities that significantly affect health disparities in pediatric congenital heart disease. This comprehensive review sheds light on disparities from diverse perspectives in pediatric care, demonstrates the inequities and inequalities leading to these disparities, presents effective solutions, and issues a call to action for providers, institutions, and the health care system. Recognizing and addressing these disparities is imperative for ensuring equitable care and enhancing the long-term well-being of children affected by congenital heart disease. Implementing robust, evidence-based frameworks that promote responsible and safe interventions is fundamental to enduring change. more...

Echocardiography is a fundamental component of pediatric cardiology, and appropriate indications have been established for its use in the setting of suspected, congenital, or acquired heart disease in children. Since the publication of guidelines for pediatric transthoracic echocardiography in 2006 and 2010, advances in knowledge and technology have expanded the scope of practice beyond the use of traditional modalities such as two-dimensional, M-mode, and Doppler echocardiography to evaluate the cardiac segmental structures and their function. Adjunct modalities such as contrast, three-dimensional, and speckle-tracking echocardiography are now used routinely at many pediatric centers. Guidelines and recommendations for the use of traditional and newer adjunct modalities in children are described in detail in this document. In addition, suggested protocols related to standard operations, infection control, sedation, and quality assurance and improvement are included to provide an organizational structure for centers performing pediatric transthoracic echocardiograms., Competing Interests: Conflicts of Interest The authors reported no actual or potential conflicts of interest in relation to this document., (Copyright © 2023. Published by Elsevier Inc.) more...

Importance: Paediatricians play an integral role in the lifelong care of children with CHD, many of whom will undergo cardiac surgery. There is a paucity of literature for the paediatrician regarding the post-operative care of such patients., Observations: The aim of this manuscript is to summarise essential principles and pertinent lesion-specific context for the care of patients who have undergone surgery or intervention resulting in a biventricular circulation., Conclusions and Relevance: Familiarity with common issues following cardiac surgery or intervention, as well as key details regarding specific lesions and surgeries, will aid the paediatrician in providing optimal care for these patients. more...

Targeted neonatal echocardiography (TNE) has been increasingly used at the bedside in neonatal care to provide an enhanced understanding of physiology, affecting management in hemodynamically unstable patients. Traditional methods of bedside assessment, including blood pressure, heart rate monitoring, and capillary refill are unable to provide a complete picture of tissue perfusion and oxygenation. TNE allows for precision medicine, providing a tool for identifying pathophysiology and to continually reassess rapid changes in hemodynamics. A relationship with cardiology is integral both in training as well as quality assurance. It is imperative that congenital heart disease is ruled out when utilizing TNE for hemodynamic management, as pathophysiology varies substantially in the assessment and management of patients with congenital heart disease. Utilizing TNE for longitudinal hemodynamic assessment requires extensive training. As the field continues to grow, guidelines and protocols for training and indications are essential for ensuring optimal use and providing a platform for quality assurance., (Copyright © 2022 by the American Academy of Pediatrics.) more...

Importance: Single ventricle CHD affects about 5 out of 100,000 newborns, resulting in complex anatomy often requiring multiple, staged palliative surgeries. Paediatricians are an essential part of the team that cares for children with single ventricle CHD. These patients often encounter their paediatrician first when a complication arises, so it is critical to ensure the paediatrician is knowledgeable of these issues to provide optimal care., Observations: We reviewed the subtypes of single ventricle heart disease and the various palliative surgeries these patients undergo. We then searched the literature to detail the general paediatrician's approach to single ventricle patients at different stages of surgical palliation., Conclusions and Relevance: Single ventricle patients undergo staged palliation that drastically changes physiology after each intervention. Coordinated care between their paediatrician and cardiologist is requisite to provide excellent care. This review highlights what to expect when these patients are seen by their paediatrician for either well child visits or additional visits for parental or patient concern. more...

The presence of a genetic condition is a risk factor for increased mortality in hypoplastic left heart syndrome (HLHS). Speckle tracking strain analysis in interstage echocardiograms have shown promise in identifying patients with HLHS at increased risk of mortality. We hypothesized that fetuses with a genetic condition and HLHS have impaired right ventricular global longitudinal strain compared with fetuses with HLHS and no evident genetic condition. We performed a retrospective analysis of 60 patients diagnosed in fetal life with HLHS from 11/2015 to 11/2019. We evaluated presenting echocardiograms and calculated right ventricular global longitudinal strain (RV GLS) and fractional area of change (FAC) using post-processing software. We first compared RV GLS and FAC between those with genetic conditions to those without. We examined the secondary outcome of mortality among those with and without genetic conditions and among HLHS subgroups. Of the 60 patients with available genetic testing, 11 (18%) had an identified genetic condition. Neither RV GLS nor FAC was significantly different between patients with and without genetic conditions. There was no difference in RV GLS or FAC among HLHS phenotype or those who died or survived as infants. However, patients with a genetic syndrome had increased neonatal and overall mortality. In this cohort, RV GLS did not differ between those with and without a genetic diagnosis, among HLHS phenotypes, or between those surviving and dying as infants. Further analysis of strain throughout gestation and after birth could provide insight into the developing heart in fetuses with HLHS., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.) more...

We aimed to investigate intradialytic changes in ventricular and atrial function using speckle tracking echocardiography (STE) in pediatric hemodialysis (HD). Children with HD vintage > 3 months were enrolled, and echocardiography was performed prior to, during, and after HD. STE was analyzed using GE EchoPAC. Left ventricular (LV) global longitudinal strain (GLS), strain rate (Sr), and mechanical dispersion index (MDI) were calculated as the average from 3 apical views; diastolic strain (Ds) and Sr from 4-chamber tracing; left atrial strain (LAS) and Sr from the 4- and 2-chamber views. A total of 15 patients were enrolled at a median age of 12 years (IQR 8, 16) and median HD vintage of 13 months (IQR 9, 25). GLS worsened during HD (- 15.8 ± 2.2% vs - 19.9 ± 1.9%, p < 0.001). Post-HD GLS was associated with BP decrease (coefficient = 0.62, p = 0.01). LV MDI and systolic Sr did not change. LV Ds progressively worsened (- 8.4% (- 9.2, - 8.0) vs - 11.9% (- 13.4, - 10.3), p < 0.001). LAS changes at mid-HD returned to baseline post-HD. Ds, DSr, LAS, LASr were not associated with BV removal or BP decrease (p > 0.1). In conclusions, intradialytic LV strain and LAS changes consistent with subclinical systolic and diastolic dysfunction were observed during HD in children. Changes in Ds, DSr, LAS, and LASr were not associated with BP change or BV removal and may be related to the disease progression. Longitudinal study using these novel indices may unfold the effect of these subclinical changes on long-term cardiovascular health in children requiring chronic HD., (© 2021. The Author(s), under exclusive licence to Springer Nature B.V.) more...

During the initial surge of the COVID-19 pandemic in the spring and summer of 2020, paediatric heart centres were forced to rapidly alter the way patient care was provided to minimise interruption to patient care as well as exposure to the virus. In this survey-based descriptive study, we characterise changes that occurred within paediatric cardiology practices across the United States and described provider experience and attitudes towards these changes during the pandemic. Common changes that were implemented included decreased numbers of procedures, limiting visitors and shifting towards telemedicine encounters. The information obtained from this survey may be useful in guiding and standardising responses to future public health crises. more...

Over the 12 months since the start of the coronavirus disease 2019 pandemic, an explosion of investigation and an increase in experience have led to vast improvement in our knowledge about this disease. However, coronavirus disease 2019 remains a huge public health threat., (Copyright © 2021 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.) more...

Mastering pediatric echocardiography (PE) requires a substantial knowledge base of echo theory and congenital heart disease (CHD), along with technical proficiency. Online self-directed learning has become increasingly utilized among medical trainees to promote competency within medical subspecialties. We sought to evaluate the impact of online learning combined with lecture-based and hands-on teaching on the acquisition of PE knowledge and confidence in pediatric cardiology and pediatric critical care fellows. We prospectively enrolled 124 learners from 2016 to 2019. These included 40 incoming cardiology and critical care fellows at Texas Children's Hospital (TCH) as well as 84 national and international participants recruited from our online echo education website. All participants completed online learning modules through our website ( www.pedecho.org ), which covers pediatric echo physics, Doppler, technique, normal anatomy, atrial septal defects, and ventricular septal defects. TCH cardiology and critical care fellows subsequently participated in an Echo Boot Camp (BC), a 3-day training program with hands-on workshops and didactic lectures. Knowledge was assessed using an 80-question pre and post-test multiple choice exams. The online learning group demonstrated improvement in exam scores following online learning (PRE 49.1 ± 15.3 vs. POST 67.8 ± 17%; p ≤ 0.01). Echo Boot Camp fellows were noted to have further incremental improvement in test scores following BC (PRE 48% ± 13% vs. POST MODULE 68.6% ± 15% vs. POST BC 75.7% ± 13%; p ≤ 0.01). Self-assessment regarding confidence in context areas showed a substantial gain in self perceived interpretive confidence across all groups as well as procedural confidence in BC participants. Online learning significantly promotes the acquisition of echocardiography knowledge. Additional lecture-based and hands-on teaching in the form of an Echo Boot Camp can further enhance knowledge and interpretative skills. Both of these learning platforms appear to work in concert as powerful and effective tools in fellow education. more...

Objectives: To evaluate the association of abnormal Doppler velocimetric patterns in the umbilical arteries (UAs) and right ventricular outflow tract abnormalities (RVOTAs) in twin- twin transfusion syndrome (TTTS) cases., Methods: This retrospective study involved women who had laser surgery for TTTS between January 2012 and May 2018 at a single institution. The prevalence of an RVOTA in either twin was compared among TTTS cases in which both twins had positive end-diastolic flow (EDF) in the UA and those in which either twin had intermittent or persistent absent/reversed UA EDF. Nonparametric tests were used for comparisons. Logistic regression was performed to identify variables associated with an RVOTA in either twin, adjusted for moderate or severe tricuspid regurgitation, right ventricular hypertrophy, right ventricular systolic or diastolic dysfunction, the Quintero stage, and other confounders. P < .05 was considered significant., Results: A total of 126 consecutive TTTS cases were included. Right ventricular outflow tract abnormalities were seen in 8.7% (11of 126) of cases, all in recipient twins. Significant differences in the rate of RVOTAs in the recipient twin were seen between TTTS cases with intermittent absent/reversed UA EDF and those with positive UA EDF (26.9% [7 of 26] versus 3.7% [3 of 82]; P = .002]. However, no significant differences were noted among the other study groups. Intermittent absent/reversed UA EDF was associated with a significantly increased risk for an RVOTA (adjusted odds ratio, 20.6 [95% confidence interval, 3.1-138]; P = .002) after adjusting for confounders., Conclusions: Intermittent changes in vascular impedance to UA flow may contribute to the pathogenesis of acquired right-sided cardiac lesions in the recipient twin affected with TTTS., (© 2020 American Institute of Ultrasound in Medicine.) more...

Background: Longitudinal clinical surveillance by transthoracic echocardiography (TTE) is an established practice in children with repaired tetralogy of Fallot (TOF). Non-Invasive Imaging Guidelines recommends a list of reporting elements that should be addressed during routine TTE in this population. In this study, we assessed the adherence to these recommendations., Methods: This was a multi-center (n = 8) retrospective review of TTE reports in children ≤11 years of age who have had complete TOF repair. We included 10 patients from each participating center (n = 80) and scored 2 outpatient follow-up TTE reports on each patient. The adherence rate was based on completeness of TTE reporting elements derived from the guidelines., Results: We reviewed 160 TTE reports on 80 patients. Median age was 4.4 months (IQR 1.5-6.6) and 3.6 years (IQR 1.3-6.4) at the time of complete surgical repair and first TTE report, respectively. The median adherence rate to recommended reporting elements was 61% (IQR 53-70). Of the 160 reports, 9 (7%) were ≥80% adherent and 40 (25%) were ≥70% adherent. Quantitative measurements of right ventricular outflow tract (RVOT), right ventricular (RV) size and function, and branch pulmonary arteries were least likely to be reported., Conclusions: Overall adherence to the most recent published imaging guidelines for surveillance of children with repaired TOF patients was suboptimal, especially for reporting of RVOT, RV size and function, and branch pulmonary arteries. Further studies are needed to explore the barriers to adherence to guidelines and most importantly, whether adherence is associated with clinical outcomes., (Copyright © 2019 Elsevier B.V. All rights reserved.) more...

Objective: Children with Kawasaki disease (KD) with persistent coronary artery aneurysms (CAAs) can develop chronic vasculopathy and subsequent myocardial ischemia. Early detection of this process is challenging. Myocardial deformation analysis can detect early alterations in myocardial performance. We aim to determine whether there are differences in myocardial deformation between KD patients with and without CAAs., Design: This is a cross-sectional study of 123 echocardiograms performed on 103 children with KD. Myocardial deformation was measured with two-dimensional speckle tracking (2DSTE). The echocardiograms were divided into groups according to the KD phase in which they were performed: acute, subacute, and convalescent/chronic. The convalescent/chronic phase group was then divided based on the presence or absence of CAAs. Left ventricular (LV) global longitudinal strain (GLS), global longitudinal strain rate (GLSSR), global circumferential strain (GCS), global circumferential systolic strain rate (GCSSR), peak torsion, and torsion rate were measured., Results: The numbers of echocardiograms analyzed in each of the KD phase groups were: 31 acute, 25 subacute, and 67 convalescent/chronic. Myocardial deformation was within normal limits in all groups. However, GLSSR, GCSSR, peak torsion, and torsion rate were lower in the convalescent/chronic phase group than in the acute phase group (mean, -1.37 ± 0.24 vs -1.55 ± 0.21 1/s; -1.63 ± 0.27 vs -1.84 ± 0.35 1/s; 2.49 ± 1.13 vs 3.41 ± 2.60 °/cm, and 21.97 ± 8.36 vs 26.69 ± 10.86 °/cm/s; P < .05 for all). The convalescent/chronic phase subgroup with CAAs had lower GLSSR and GCSSR than the subgroup without CAAs (mean, -1.23 ± 0.22 vs -1.42 ± 0.22 1/s; -1.46 ± 0.25 vs -1.68 ± 0.26 1/s, P < .05 for both)., Conclusions: Children in the convalescent/chronic phase of KD have a subtle decrease in strain rate when compared to the acute phase, although within the normal range. This decrease is more pronounced in children with CAAs than those without CAAs. Longitudinal studies are needed to discern whether low-normal strain rate predicts decreased myocardial function in the long term., (© 2019 Wiley Periodicals, Inc.) more...

Objectives: Patients with Kawasaki disease can develop life-altering coronary arterial abnormalities, particularly in those resistant to intravenous immunoglobulin (IVIg) therapy. We tested the tumor necrosis factor α receptor antagonist etanercept for reducing both IVIg resistance and coronary artery (CA) disease progression., Methods: In a double-blind multicenter trial, patients with Kawasaki disease received either etanercept (0.8 mg/kg; n = 100) or placebo ( n = 101) subcutaneously starting immediately after IVIg infusion. IVIg resistance was the primary outcome with prespecified subgroup analyses according to age, sex, and race. Secondary outcomes included echocardiographic CA measures within subgroups defined by coronary dilation ( z score >2.5) at baseline. We used generalized estimating equations to analyze z score change and a prespecified algorithm for change in absolute diameters., Results: IVIg resistance occurred in 22% (placebo) and 13% (etanercept) of patients ( P = .10). Etanercept reduced IVIg resistance in patients >1 year of age ( P = .03). In the entire population, 46 (23%) had a coronary z score >2.5 at baseline. Etanercept reduced coronary z score change in those with and without baseline dilation ( P = .04 and P = .001); no improvement occurred in the analogous placebo groups. Etanercept ( n = 22) reduced dilation progression compared with placebo ( n = 24) by algorithm in those with baseline dilation ( P = .03). No difference in the safety profile occurred between etanercept and placebo., Conclusions: Etanercept showed no significant benefit in IVIg resistance in the entire population. However, preplanned analyses showed benefit in patients >1 year. Importantly, etanercept appeared to ameliorate CA dilation, particularly in patients with baseline abnormalities., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2019 by the American Academy of Pediatrics.) more...

Introduction: In a pilot study of chronic maternal hyperoxygenation (CMH) in left heart hypoplasia (LHH), we sought to determine effect estimates of CMH on head size, vascular resistance indices, and neurodevelopment compared to controls., Material and Methods: Nine gravidae meeting the inclusion criteria (fetal LHH, ≥25.9 weeks' gestation, and ≥10% increase in percent aortic flow after acute hyperoxygenation) were prospectively enrolled. Controls were 9 contemporary gravidae with fetal LHH without CMH. Brain growth and Doppler-derived estimates of fetal cerebrovascular and placental resistance were blindly evaluated and compared using longitudinal regression. Postnatal anthropomorphic and neurodevelopmental assessments were compared., Results: There was no difference in baseline fetal measures between groups. There was significantly slower biparietal diameter (BPD) growth in the CMH group (z-score change -0.03 ± 0.02 vs. +0.09 ± 0.05 units/week, p = 0.02). At 6 months postnatal age, the mean head circumference z-score in the CMH group was smaller than that of controls (-0.20 ± 0.58 vs. +0.85 ± 1.11, p = 0.048). There were no differences in neurodevelopmental testing at 6 and 12 months., Discussion: In this pilot study, relatively diminished fetal BPD growth and smaller infant head circumference z-scores at 6 months were noted with in utero CMH exposure., (© 2018 S. Karger AG, Basel.) more...

Background: We previously reported on the short-term impact of an echocardiography "Boot Camp" on a single class of cardiology fellows (CF). The impact of the Boot Camp on performance throughout fellowship is unknown., Methods: We enrolled four classes of CFs and two classes of cardiac ICU fellows (CVs) prospectively into the Boot Camp and compared CFs to a historical cohort. Experience with echocardiography was surveyed. Outcome measures included written pre- and post-Camp exams, a performance based test (PBT), self-efficacy assessments, numbers of echocardiograms performed, and echocardiogram quality during the last 3 months of fellowship., Results: A total of 25 CFs and 7 CVs participated in the Boot Camp from July 2012-July 2015. Median experience score was 13/40 (4-23). Median self-efficacy improved from 22/147 (range 21-45) to 90/147 (range 49-133) (P=<.001), and written scores from 14/29 (8-24) to 24/29 (13-29) (P<.001). CFs who completed the Boot Camp performed more independent echocardiograms compared to controls at the end of the 1 st (37.7±12.2 vs 28.2±12.1, P=.15), 2 nd (71.3±24.4 vs 47.6±16.0, P=.044), and third year of fellowship (130.4±44.0 vs 100.0±29.3, P=.230), and on average achieved 150 total echocardiograms in the 4.8th quarter compared to the 7.8th quarter in controls, P=.053. 2D quality scores were higher and shortening fraction more often obtained in echocardiograms performed by Boot Camp CFs compared to controls., Conclusions: The pediatric echocardiography Boot Camp improved self-efficacy, acquisition, and retention of echocardiography skills and knowledge, and increased echocardiogram performance. Observed differences between Boot Camp and control CFs appear to wane across fellowship., (© 2017, Wiley Periodicals, Inc.) more...

Echocardiography is an excellent noninvasive imaging modality for evaluation and follow-up of cardiac lesions, especially coronary artery changes occurring as a result of Kawasaki disease. The information obtained has prognostic implications and can be complemented with other modes of imaging for risk stratification and optimization of both medical and interventional therapy. The aim of this article is to describe the time line of echocardiographic follow-up of patients affected with Kawasaki disease. The classification of coronary artery changes and transthoracic echocardiographic views recommended for detailed evaluation of the coronary arteries are delineated in detail in this report., (© 2017 Wiley Periodicals, Inc.) more...

Introduction: Left ventricular cardiac twist and torsion values have been described in premature and term neonates, but not in early infancy. Early and late peak untwist rates and time to peak (TTP) values have not been described in infants., Methods: 53 term infants were enrolled prospectively. The following parameters were obtained by two blinded observers at 1-2months postnatal age: peak twist and torsion (twist indexed to LV length), peak twist rate and torsion rate, TTP twist, early peak untwist rate, TTP early untwist rate, late peak untwist rate, TTP late untwist rate. Reproducibility was assessed using intraclass correlation and Bland Altman analysis., Results: Intraclass correlation was ≥0.87 for all peak rotational mechanics values. Measures of TTP values had intraclass correlation (ICC) values ≤0.77, with TTP twist rate demonstrating the lowest ICC (0.69). The only measure which demonstrated significant bias was TTP twist rate. Peak twist demonstrated modest correlation (R=0.52, p<0.001) with global circumferential strain, and no correlation with ejection fraction, global longitudinal strain, or left ventricular myocardial performance index., Conclusions: Measurements of rotational mechanics and timing to peak values have acceptable reproducibility. Peak twist, twist rate, and early untwist rate values in early infancy are similar to those reported in premature neonates, and higher than those reported in older children. Twist indexed to LV length (torsion) is lower in early infancy than in premature neonates, but higher than in term neonates., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.) more...

Introduction: Twin-twin transfusion syndrome (TTTS) is the major cause for fetal demise in monochorionic diamniotic pregnancies. Estimated combined cardiac output (CCO) has not been studied in this setting. The primary aims of this study were to compare CCO in twin donor and recipient fetuses and assess differences in CCO before and after laser coagulation therapy. A secondary aim was to evaluate the relationship of CCO with perinatal mortality, defined as intrauterine or neonatal mortality (≤30 days of age) in fetuses that underwent laser coagulation therapy., Methods: We performed a retrospective review of fetuses with TTTS who received echocardiograms prior to laser therapy. Prelaser echocardiograms were performed in 66 fetuses, of which 45 (21 donor and 24 recipient) survived to the postlaser echocardiogram., Results: Donor CCO increased from a mean of 487±137 mL/min/kg prelaser to 592±128 mL/min/kg postlaser (P=.025). There was no significant change in mean recipient CCO from prelaser 551±165 mL/min/kg to postlaser 575±112 mL/min/kg (P=.564). In recipient fetuses, higher prelaser CCO correlated with increasing Quintero stage (P=.010) and perinatal mortality (P=.003). Thirty-day postnatal outcome data were available for 38 fetuses, of whom 34 (89%) survived. Amongst survivors, 25 (74%) experienced an increase in CCO, while 9 (26%) experienced a decrease in CCO (P=.010). Donor fetuses had higher CCO after laser therapy., Conclusions: Higher prelaser CCO in recipient fetuses may indicate more advanced disease. Fetuses who exhibit a decrease in CCO after therapy laser may be at higher risk for perinatal mortality., (© 2016, Wiley Periodicals, Inc.) more...

Objectives: Primary fetal cardiomyopathy is a rare entity, with a poor prognosis. We sought to describe its echocardiographic characteristics and outcomes., Methods: We performed a 12-year retrospective review of fetuses with primary cardiomyopathy., Results: Of more than 6000 fetuses evaluated, 25 met criteria for primary cardiomyopathy, and 18 had sufficient echocardiographic and pregnancy outcome data for inclusion. At echocardiography, the median gestational age was 29.6 weeks (range, 21.0-36.4 weeks); median cardiovascular profile score was 6 (range, 1-9); median right ventricular Tei index was 0.52 (range, 0.32-0.94); and median left ventricular Tei index was 0.40 (range, 0.15-0.88). Two had fetal demise, and 16 survived to delivery. The median cardiovascular profile score in those with fetal demise was 3.0 and in those who survived to delivery was 6.5 (range, 3-9; P = .14). The median right ventricular Tei index in those with fetal demise was 0.39 and in those surviving to delivery was 0.53 (range, 0.38-0.94; P = .49). The median left ventricular Tei index in those with fetal demise was 0.29 and in those surviving to delivery was 0.42 (range, 0.15-0.88; P = .50). Sixty-day survival was available in 11 of 16 fetuses. In addition to the 2 with fetal demise, 4 had postnatal demise, and 5 were alive at a median follow-up of 39 months. Hydrops (P = .01), skin edema (P = .01), and mild or greater mitral regurgitation (P = .02) were associated with fetal or postnatal demise, with a trend toward an association between moderate or greater tricuspid regurgitation (P = .07) and fetal or postnatal demise., Conclusions: Hydrops and atrioventricular valvar regurgitation are ominous signs in primary fetal cardiomyopathy. Although other commonly used methods for assessing cardiovascular performance may help in diagnosing primary cardiomyopathy, these data suggest limited predictive value. more...

Objective: Acute maternal hyperoxygenation (AMH) results in increased fetal left heart blood flow. Our aim was to perform a pilot study to determine the safety, feasibility and direction and magnitude of effect of chronic maternal hyperoxygenation (CMH) on mitral and aortic valve annular dimensions in fetuses with left heart hypoplasia (LHH) after CMH., Methods: Gravidae with fetal LHH were eligible for inclusion in a prospective evaluation of CMH. LHH was defined as: sum of aortic and mitral valve annuli Z-scores < -4.5, arch flow reversal and left-to-right or bidirectional atrial level shunting without hypoplastic left heart syndrome or severe aortic stenosis. Gravidae with an affected fetus and with ≥ 10% increase in aortic/combined cardiac output flow after 10 min of AMH at 8 L/min 100% fraction of inspired oxygen were offered enrollment. Nine gravidae were enrolled from February 2014 to January 2015. The goal therapy was ≥ 8 h daily CMH from enrollment until delivery. Gravidae who were cared for from July 2012 to October 2014 with fetal LHH and no CMH were identified as historical controls (n = 9). Rates of growth in aortic and mitral annuli over the final trimester were compared between groups using longitudinal regression., Results: There were no significant maternal or fetal complications in the CMH cohort. Mean gestational age at study initiation was 29.6 ± 3.2 weeks for the intervention group and 28.4 ± 1.8 weeks for controls (P = 0.35). Mean relative increase in aortic/combined cardiac output after AMH was 35.3% (range, 18.1-47.9%). Median number of hours per day on CMH therapy was 9.3 (range, 6.5-14.6) and median duration of CMH was 48 (range, 33-84) days. Mean mitral annular growth was 0.19 ± 0.05 mm/week compared with 0.14 ± 0.05 mm/week in CMH vs controls (mean difference 0.05 ± 0.05 mm/week, P = 0.33). Mean aortic annular growth was 0.14 ± 0.03 mm/week compared with 0.13 ± 0.03 mm/week in CMH vs controls (mean difference 0.01 ± 0.03 mm/week, P = 0.75). More than 9 h CMH daily (n = 6) was associated with better growth of the aortic annulus in intervention fetuses (0.16 ± 0.03 vs 0.08 ± 0.02 mm/week, P = 0.014)., Conclusions: CMH is both safe and feasible for continued research. In this pilot study, the effect estimates of annular growth, using the studied method of delivery and dose of oxygen, were small. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd., (Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.) more...

Background: Normative fetal diastolic strain rate values have not been comprehensively reported. The aims of this study were to report normative data for diastolic strain rate parameters across gestation and upon delivery, determine the effect of advancing gestational age on these measures, and compare interobserver variability at multiple gestational age time points., Methods: Sixty gravid women were enrolled before 20 weeks' gestation. The following measures were obtained by two blinded observers at five time points across gestation and at 4 to 8 weeks' postnatal age: global left ventricular circumferential strain rate peak E and A waves, global left and right ventricular longitudinal strain rate peak E and A waves, and mitral and tricuspid valve E/A ratios. Reproducibility was assessed using intraclass correlation and Bland-Altman analysis. Least square means analysis was used to evaluate for changes across gestational age., Results: Left ventricular longitudinal and circumferential diastolic strain rate values decreased across gestation, while right ventricular longitudinal values remained stable. With delivery, left ventricular values remained fairly stable, while right ventricular values increased. Intraclass correlations for diastolic strain rate values were 0.68 to 0.94 at ≥24 weeks' gestation and 0.25 to 0.82 for values at 20 to 21 weeks' gestation. Intraclass correlations ranged from 0.49 to 0.90 for mitral valve and tricuspid valve E/A ratios across gestation., Conclusions: Fetal measures of diastolic strain rate have acceptable reproducibility after 24 weeks' gestation. The described changes across gestation likely reflect intrinsic myocardial adaptation to loading conditions. These reference ranges can be used to assess effects of various disease states on fetal myocardial deformation., (Copyright © 2016 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.) more...

Background: Normative data for fetal myocardial deformation values have not been comprehensively described in a longitudinal cohort. The effect of gestational age on these values and on interobserver variability require further investigation., Methods: Sixty gravid women were prospectively enrolled before 20 weeks' gestation. The following measures were obtained by two blinded observers at five time points across gestation and also at 4 to 8 weeks' postnatal age: global circumferential strain and strain rate, global longitudinal left ventricular strain and strain rate, global longitudinal right ventricular strain and strain rate, and left and right ventricular myocardial performance indices. Optimal myocardial visualization and frame rate (≥100 frames/sec) were ensured., Results: For gestational age groups ≥24 weeks, intraclass correlation coefficients between observers were >0.70 for all measures and >0.85 for the majority of measures of myocardial deformation. At 20 to 21 weeks' gestation, intraclass correlation coefficients were 0.35 to 0.52 for longitudinal measures and 0.74 to 0.82 for circumferential measures. Myocardial performance index intraclass correlation coefficients were <0.80 at all time points and <0.70 for most time points. Global longitudinal left ventricular strain and global circumferential strain values remained stable across gestational age groups. Global longitudinal right ventricular strain values remained stable across gestation and increased after birth. Global circumferential strain rate, global longitudinal left ventricular strain rate, and global longitudinal right ventricular strain rate decreased from 20 to 21 weeks' gestation to the remainder of gestation and then remained stable until delivery. Upon delivery, global circumferential strain rate and global longitudinal left ventricular strain rate decreased, and global longitudinal right ventricular strain rate increased., Conclusions: Interobserver variability of fetal strain and strain rate measured at ≥24 weeks' gestation was lower in comparison with values obtained at 20 to 21 weeks' gestation and lower in comparison with left ventricular and right ventricular myocardial performance indices using the described protocol. Gestational changes in fetal myocardial deformation values likely reflect changes in preload and/or afterload on the fetal heart., (Copyright © 2016 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.) more...

Background: Right ventricular (RV) dilation, RV dysfunction, RV outflow tract obstruction, and branch pulmonary artery stenosis are risk factors associated with the need for future surgical or catheter-based intervention commonly assessed by cardiac magnetic resonance in patients with tetralogy of Fallot who have undergone initial repair. The ability of echocardiography to predict the presence of these risk factors is limited., Objective: We aimed to determine the association between echocardiographic parameters of right ventricular diastolic dysfunction and risk factors for intervention as identified on cardiac magnetic resonance imaging (CMR)., Design: We reviewed all echocardiograms and CMRs performed at our institution within 1 year of each other in patients with repaired tetralogy of Fallot. We evaluated patients for the following: RV systolic dysfunction (RV ejection fraction <45%), RV dilation (right ventricular end-diastolic volume ≥ 160 mL/m(2)), and RV obstruction (RV outflow tract gradient ≥ 3 m/s by echocardiogram or branch pulmonary artery stenosis assessed by CMR). We evaluated for associations between the above CMR findings and echocardiographic parameters of diastolic function., Results: CMR and echocardiographic images performed between March 2007 and March 2012 were available in 99 patients. RV obstruction was associated with lower E/A ratio (P = .02), E/A reversal (P = .02), and prograde end diastolic pulmonary flow (P < .01). Low tricuspid annular a' and low septal s' were associated with lower RV ejection fraction (P < .01, P < .01). Elevated E/e' was associated with higher RV end diastolic volume (P = .04). In predicting the presence of any of the evaluated risk factors for future intervention, TV E/e' ≥ 4.7 had positive predictive value (PPV) of 86% and negative predictive value (NPV) of 50%, TV E/A reversal had PPV of 89% and NPV of 34, and prograde end diastolic pulmonary flow on echocardiogram had PPV of 91% and NPV of 38%., Conclusions: Echocardiographic parameters of diastolic dysfunction have a reasonable PPV but poor NPV for predicting clinically significant risk factors identified by CMR., (© 2015 Wiley Periodicals, Inc.) more...

Background: Dynamic training schedules introduce novel challenges to medical specialty training programs that require manual dexterity. The aim of this study was to examine the effect of a 3-day, intensive pediatric echocardiography course ("boot camp") on trainee self-efficacy and on the acquisition and short-term retention of basic echocardiographic knowledge and skills for first-year pediatric cardiology fellows (CFs)., Methods: The boot camp consisted of hands-on structured practice guided by sonographers and cardiology faculty members, didactic lectures, and reading. Pre-boot camp experience was assessed using an experience score. Outcome measures included written precamp and postcamp examinations, a performance-based test, precamp and postcamp self-efficacy assessments, and the number and quality of echocardiographic examinations performed in the first 3 months of fellowship., Results: Six CFs completed the boot camp. Two of the six CFs reported experience scores of 2 out of 10, whereas the remainder reported experience scores of 0 out of 10. Performance-based test scores ranged from 68 to 99 out of 147. All six CFs reported precamp self-efficacy scores of 21 (the minimum score), compared with median postcamp scores of 82 (range, 49-94) (P = .01). Scores on the written examination improved from median of 16 (range, 11-18) to 23.5 (range, 22-28) (P = .01). CFs who completed the boot camp completed 28 independent echocardiographic examinations (median, 4 per CF) during the first 3 months of fellowship, an increase from six independent examinations (median, 1 per CF) by CFs during the year before institution of the boot camp (P = .030). Echocardiograms obtained by CFs who had completed the boot camp scored higher on total quality (P = .004), overall two-dimensional image quality (P = .011), functional assessments (P = .015), and assessment for pericardial effusion (P = .031)., Conclusions: The echocardiography boot camp improves self-efficacy in performing an echocardiographic examination and the acquisition and short-term retention of skills and knowledge required to perform pediatric echocardiography., (Copyright © 2013 American Society of Echocardiography. Published by Mosby, Inc. All rights reserved.) more...

Purpose: The aim of this study was to identify the most accurate prenatal predictors of outcomes and need for fetal surgery for fetuses with high-risk lung masses and vascular tumors., Methods: The records of all fetuses with high-risk lung mass (congenital cystic adenomatoid malformation-volume ratio > 1.6 or findings of hydrops) and vascular tumor evaluated between July 2001 and March 2011 were reviewed retrospectively. Hydrops was defined as accumulation of fluid in 2 or more compartments., Results: Of fetuses with high-risk lung mass, hydrops was identified in 46% (11/24). Fetuses with hydrops and an abnormal echocardiogram (n = 8) demonstrated poor survival without fetal surgery (13%) compared with 100% survival in fetuses with hydrops and a normal echocardiogram (n = 3; P = .02). Of 21 fetuses with vascular tumor (11 sacrococcygeal and 8 cervical teratomas; 2 hemangioendotheliomas), hydrops was identified in 29% and an abnormal echocardiogram in 57%. All fetuses with hydrops had an abnormal echocardiogram and either died (n = 5) or required fetal surgery (n = 1). However, all fetuses with abnormal echocardiograms alone (n = 7) survived without fetal intervention., Conclusions: For fetuses with lung mass, an abnormal echocardiogram in the setting of hydrops is the best predictor of mortality and need for fetal surgery. For fetuses with vascular tumor, hydrops in the setting of high-output physiology best predicts demise and need for fetal surgery., (Copyright © 2012 Elsevier Inc. All rights reserved.) more...

Introduction: This post-hoc analysis examined time to recovery in patients with acute, painful musculoskeletal conditions following cyclobenzaprine extended release (CER; AMRIX(®), Cephalon, Inc., Frazer, PA, USA), cyclobenzaprine immediate release (CIR; Flexeril(®), Pliva, Pomona, NY, USA), or placebo administration., Methods: Data were pooled from two identically designed, double-blind, placebo-controlled, parallel-group studies. Adults with acute, painful local muscle spasm were randomized to once-daily CER 15 mg, once-daily CER 30 mg, three-times-daily CIR 10 mg, or placebo for 14 days. Efficacy assessments were time to first rating of "a lot" or "complete" relief from local pain or restriction of movement and patient-rated medication helpfulness., Results: A total of 504 patients were randomized, and 330 (65.5%) completed the studies. Median times to "a lot" or "complete" relief from local pain were faster with CER 15 mg (6 days, P=0.016), CER 30 mg (5 days, P=0.002), and CIR (5 days, P=0.002) versus placebo (8 days). Time to "a lot" or "complete" relief from restriction of movement was faster with CER 30 mg (5 days, P=0.004) and CIR (5 days, P=0.009) versus placebo (7 days). Median times to "very good" or "excellent" medication helpfulness were 10 days (CER 15 mg, P=0.020), 8 days (CER 30 mg, P=0.004), and 7 days (CIR, P<0.001) versus >14 days (placebo). Dry mouth, constipation, dizziness, headache, and somnolence were the most commonly reported adverse events. Somnolence rates were lower with CER 15 mg (0.8%, P=0.008) and CER 30 mg (1.6%, P=0.028) versus CIR (7.3%)., Conclusion: Relief from local pain and restriction of movement occurred sooner with CER and CIR than placebo. CER was associated with less somnolence than CIR. more...

Infants with hypoplastic left heart syndrome and intact or highly restrictive atrial septum require emergent therapy to relieve pulmonary congestion. Transcatheter stenting has become, in most large centers, the mainstay of therapy for relief of left atrial hypertension. Normally, this procedure is performed with the assistance of transesophageal echocardiographic guidance. However, the transesophageal approach is untenable in neonates of low birth weight, as the transducers can be traumatic and can cause ventilatory difficulty. We present two cases in which transthoracic echocardiographic guidance was used to guide atrial perforation and stenting in low birth-weight neonates with hypoplastic left heart syndrome with intact or highly restrictive atrial septum. Both procedures were straightforward and there were no complications. Real-time biplane imaging greatly assisted in the intervention in one case. The advantages of this approach are discussed. more...

Objective: To assess the efficacy and tolerability of once-daily cyclobenzaprine extended release (CER) 15 and 30 mg in relieving acute muscle spasm., Methods: This is a pooled analysis of 2 randomized, double-blind, placebo-controlled, parallel-group studies of identical design. Adults with local muscle spasm associated with neck/low back pain were randomized to treatment with once-daily CER 15 (n = 127) or 30 mg (n = 126), cyclobenzaprine immediate release (CIR) 10 mg 3 times daily (n = 123), or placebo (n = 128) for 14 days. Primary outcome measures were the patient's rating of medication helpfulness and physician's clinical global assessment of response to therapy at day 4., Results: Of 504 patients, 330 (65.5%) completed the studies. Significantly greater improvements in patient's rating of medication helpfulness were reported with CER 15 and 30 mg versus placebo at day 4 (P < 0.025). No differences were reported between groups in physician's clinical global assessment. Significantly greater improvements (P < 0.025) were noted in patient-rated secondary measures versus placebo: relief from local pain at days 4 (CER 30 mg) and 8 (CER 15 and 30 mg), global impression of change at days 4 and 8 (CER 30 mg), and restriction of movement at day 4 (CER 30 mg). Improvements with CER 15 and 30 mg on most efficacy measures were similar to CIR. There was less reported daytime drowsiness with CER 15 and 30 mg than with CIR (P < 0.05). Most adverse events (AEs) were mild in intensity. The most common AEs for all groups were dry mouth, constipation, dizziness, headache, and somnolence. The rate of somnolence reported as an AE was lower (P < 0.05) with CER 15 (0.8%) and 30 mg (1.6%) than with CIR (7.3%)., Conclusion: Once-daily CER was effective in relieving acute muscle spasm based on patient's rating of medication helpfulness at day 4 and was generally well tolerated with a low rate of reported somnolence. more...

Objective: To evaluate efficacy and tolerability of once-daily cyclobenzaprine extended release (CER) 15- and 30-mg capsules in patients with muscle spasm associated with acute, painful musculoskeletal conditions., Methods: Two identically designed, randomized, double-blind, placebo- and active-controlled, parallel-group studies in patients aged 18-75 years with muscle spasm associated with neck or back pain. Patients received CER 15 or 30 mg once daily, cyclobenzaprine immediate release (CIR) 10 mg three times daily, or placebo for 14 days. Primary efficacy measures were patient's rating of medication helpfulness and physician's clinical global assessment of response to therapy at day 4. Secondary measures were patient's rating of medication helpfulness and physician's clinical global assessment of response (days 8 and 14), relief from local pain, global impression of change, restriction in activities of daily living, restriction of movement, daytime drowsiness, quality of nighttime sleep (days 4, 8, and 14), and quality of life (days 8 and 14)., Results: A total of 156/254 randomized patients in study 1 and 174/250 in study 2 completed 14 days of treatment. Significant improvements in patient's rating of medication helpfulness were reported with CER versus placebo (CER 30 mg, study 1, p = 0.007; CER 15 mg, study 2, p = 0.018) at day 4. Significant improvements with CER 30 mg versus placebo were also seen at day 4 in study 1 for patient-rated global impression of change (p = 0.008), relief of local pain (p = 0.004), and restriction of movement (p = 0.002). Neither study reported differences between study groups on the physician's clinical global assessment. Improvements with CER were comparable to that of CIR. In both studies, daytime drowsiness was reported more frequently in active treatment groups than in the placebo group; however, reports of drowsiness decreased over time in all groups. In general, daytime drowsiness was reported more frequently in CIR groups than in CER groups. More adverse events were reported in the active treatment groups versus placebo and were similar in the CER and CIR groups, except somnolence, which occurred more frequently with CIR., Conclusions: Once-daily CER 15 mg (study 2) and CER 30 mg (study 1) were effective in treating muscle spasm associated with painful musculoskeletal conditions after 4 days of treatment. Differences between CER and placebo groups did not reach statistical significance on all efficacy measures, and the protocols were not powered to detect differences between active treatment arms. CER was generally safe and well tolerated, with low rates of somnolence. more...

Background: Doppler tissue imaging (DTI) velocities have been reported to be relatively independent of changes in ventricular loading conditions in adult studies. The clinical impact of altered left ventricular (LV) preload and afterload on DTI velocities in children with congenital heart disease has not been adequately evaluated. The purpose of this study was to evaluate the impact of chronic LV preload and afterload on DTI velocities in children with isolated ventricular septal defect and aortic valve stenosis compared with age-matched normal and abnormal (dilated cardiomyopathy) control groups., Methods: From an apical 4-chamber view, DTI velocities were obtained at the cardiac base at the lateral mitral annulus, lateral tricuspid annulus, and interventricular septum in early diastole, late diastole, and ventricular systole., Results: The majority of DTI velocities did not change significantly in patients with increased LV preload. Patients with increased LV afterload had significantly decreased systolic and early diastolic DTI velocities at both the lateral mitral annulus and ventricular septum compared with control subjects. Children with dilated cardiomyopathy had significantly decreased DTI velocities at all myocardial annular locations., Conclusions: We conclude that increases in chronic LV preload do not significantly affect the majority of DTI velocities in children with ventricular septal defects. In addition, significantly increased chronic LV afterload in children with aortic valve stenosis is associated with decreased DTI velocities in the absence of other identifiable abnormalities of LV function. Decreased DTI velocity may be secondary to increased afterload or may alternatively be an early marker of subclinical LV longitudinal dysfunction. more...

This study was performed to determine the safety and efficacy of intravenous contrast echocardiography in children attending a tertiary cardiac center. This was a prospective study to evaluate the use of Optison contrast agent in children with severely limited transthoracic echocardiographic windows. Twenty children (median age, 15 years; range, 9-18) underwent fundamental imaging (FI), harmonic imaging (HI), and HI with intravenous contrast (Optison FS-069). Endocardial border delineation was determined based on a visual qualitative scoring system (0, none: 4, excellent). Endocardial border definition was significantly improved in all patients using contrast echocardiography (FI vs Optison, p < 0.001 for each). Improved border definition was most dramatic in the apical and left ventricular (LV) free wall regions. Left ventricular ejection fraction (LVEF) was measurable in 20 patients (100%) using contrast compared to 11 (55%) with FI or HI (p < 0.05). The echocardiographic diagnosis was correctly delineated in 1 patient with a severely dyskinetic LV segment only with use of intravenous contrast and HI. No patients suffered adverse hemodynamic effects, changes in taste, or flushing episodes. Three patients experienced transient headaches. Intravenous contrast echocardiography offers an additional tool in evaluating children with very poor transthoracic echocardiographic windows. Such a strategy increases diagnostic accuracy and allows accurate LVEF determination. Adverse hemodynamic effects related to intravenous contrast are exceedingly rare. more...

Purpose: To present a case of bilateral endogenous endophthalmitis as the primary manifestation of an aortic root abscess and endocarditis, both of which were undetected by transesophageal echocardiogram., Design: Observational case report., Methods: A 13-year-old male presented with bilateral endogenous endophthalmitis and previously undiagnosed ventricular septal defect, subaortic stenosis, and a mitral valve cleft., Results: Cardiac evaluation, systemic evaluation, and transesophageal echocardiogram were negative for endocarditis, but endocarditis and aortic root abscess were discovered at time of cardiac surgery. The patient responded to systemic treatment for endocarditis and surgical management of his cardiac defect., Conclusion: A cardiac source for endogenous endophthalmitis should be considered in the presence of clinical diagnosis of bacteremia, despite a negative transesophageal echocardiogram and systemic evaluation. more...

The objectives of this study were to evaluate changes in dimension of the neo-aortic annulus, aortic root, and aortic anastomosis following arterial switch operation (ASO) and to identify risk factors for developing abnormal neo-aortic root enlargement and aortic regurgitation (AR). Prior studies report development of neo-aortic root dilatation and AR in a small subset of patients after ASO. Predisposing factors for neo-aortic root dilatation and development of moderate/severe AR are poorly understood. We performed a retrospective review of all patients with d-transposition of the great arteries (d-TGA) or double-outlet right ventricle with subpulmonary ventricular septal defect (VSD) who underwent ASO from May 1986 to January 2001. Serial echocardiograms were reviewed to measure neo-aortic annulus, root, and anastomosis diameter (z scores) and to determine progression of AR. Potential risk factors were assessed for developing neo-aortic root enlargement and AR. There were 119 patients (44 female and 75 male): 73 patients had simple d-TGA, 36 had d-TGA with ventricular septal defect, and 10 had a Taussig-Bing heart. The median duration of follow-up was 65 months (range, 12-180). The median neo-aortic root (z = 0.55+/-2.2; p < 0.01) and aortic annulus dimensions (z = 1.57+/-1.75; p < 0.01) were significantly increased over the study period. Aortic anastomosis diameter correlated with growth of the ascending aorta (z = 0.55+/-1.24). Development of severe neo-aortic root enlargement was associated with prior pulmonary artery (PA) banding (p < 0.01), the presence of a VSD (p = 0.03), and Taussig-Bing anatomy (p < 0.01) but was independent of coronary arterial anatomy, coronary arterial transfer technique, or associated lesions (p > 0.05). At latest follow-up, there was no or trivial AR in 88 patients, mild AR in 29 patients, and moderate to severe AR in 3 patients. Risk factors for developing mild or worse AR included severe or rapid neo-aortic root dilatation (p < 0.01). Only 3 patients required surgical intervention for AR. Despite the significant prevalence of neo-aortic root enlargement at intermediate follow-up after ASO, there is a low incidence of significant AR. Prior PA banding, the presence of VSD, and Taussig-Bing anatomy are risk factors for severe root enlargement. Surgical intervention for AR was rare (2%), however, serial surveillance of such patients is vital to monitor for neo-aortic root enlargement and potential aortic valve dysfunction. more...

OBJECTIVE The Seroquel Patient Evaluation on Changing Treatment Relative to Usual Medication (SPECTRUM) study assessed the efficacy and tolerability of quetiapine (Seroquel™) in patients with schizophrenia switched from treatments providing suboptimal outcomes. METHODS This was an international, open-label, non-comparative study, designed with titration to 400 mg/day quetiapine over 7 days, then flexible dosing (300-750 mg/day) for 11 weeks. Efficacy was assessed with the Positive and Negative Syndrome Scale (PANSS); Clinical Global Impression (CGI) Severity of Illness and Global Improvement scores; and the Calgary Depression Scale for Schizophrenia (CDSS). Clinical benefit and tolerability were also assessed. RESULTS The mean modal dose of quetiapine was 505 mg/day; 509 patients switched to quetiapine from olanzapine (13%), risperidone (11%), conventional antipsychotics (37%) and combinations of antipsychotics (28%), amongst others. Significant decreases in CGI Severity of Illness and PANSS scores and a significant improvement in CDSS score resulted from the switch (all P&lt;0.001 versus baseline). There were significant reductions in extrapyramidal symptoms (EPS) on the Simpson-Angus Scale (SAS) and Barnes Akathisia Scale (BAS) (both P&lt;0.001 versus baseline) and a low incidence of EPS-related adverse events (4.7%). CONCLUSION Results indicate that switching to quetiapine was clinically beneficial for patients with poor efficacy or intolerable side effects on their previous antipsychotic medication. more...

Noncontrast harmonic imaging (HI) has been shown to improve image quality in adults with poor acoustic windows. The utility of fetal echocardiography may be limited by suboptimal acoustic windows, and the use of HI in fetal echocardiography has not previously been defined. The purpose of this study was to compare the quality of fundamental imaging (FI) and HI in fetal echocardiography. Sixty-two fetal echocardiograms, including 44 (71%) with limited acoustic windows, were performed with the use of FI and HI. Image quality and visualization of the ventricles, valves, and the aortic and ductal arches were evaluated and compared between FI and HI. Mean HI scores were higher than mean FI scores for all the structures evaluated. Compared with FI, HI improved the image quality and visualization of cardiac structures in this group of fetuses with predominantly suboptimal acoustic windows. Harmonic imaging is a useful adjunct to FI in echocardiography, and the benefits of HI extend to cardiac imaging in the fetus. more...

Awareness of respiratory syncytial virus (RSV) as a serious pathogen in the child with congenital heart disease is increasing. We studied the impact of RSV lower respiratory tract disease on patients in a large academic pediatric cardiology practice. We found that RSV disease necessitating hospitalization occurs in congenital heart disease patients well into the second year of life. Although pulmonary hypertension remains a significant risk factor for morbidity in these patients, it does not appear to be as much of a factor as in the past. By implementing a nasopharyngeal RSV enzyme-linked immunoassay screening of young patients prior to cardiac surgery we found a reduction in community-acquired postoperative RSV disease. We postulate this will lead to a reduction in nosocomial disease in the postoperative care unit. more...