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Aims We hypothesized left atrial (LA) stiffness may serve as a surrogate marker in children to differentiate elevated pulmonary capillary wedge pressure (PCWP) from normal and help detect diastolic dysfunction in myocardial injury due to multisystem inflammatory syndrome in children (MIS-C). Methods and results We validated LA stiffness in 76 patients (median age 10.5 years), 33 had normal PCWP ( Conclusion In children with elevated PCWP, LA stiffness was significantly increased. When applied to children with MIS-C, LA stiffness classified myocardial injury accurately. LA stiffness and strain may serve as noninvasive markers of diastolic function in the pediatric population.

The safety and benefits of cardiac contrast-enhanced ultrasound (CEUS) have been demonstrated in children and adolescents for a variety of clinical indications, including congenital heart disease. Cardiac CEUS is performed with US and the intravenous administration of ultrasound contrast agents (UCAs). It improves transthoracic echocardiography, which can be challenging in children and adults with acoustic window limitations (e.g., from obesity) and alterations in chest wall and cardiac geometry (e.g., from prior surgical procedures). Cardiac CEUS is also used to evaluate ischemia in the follow-up of congenital and acquired heart disease. In 2019, the United States Food and Drug Administration (FDA) approved a UCA for pediatric echocardiography. This article focuses on the clinical applications of UCAs in pediatric and adult echocardiography, outlining its diagnostic value, safety and potential for future applications.

OBJECTIVE To assess the ability of point-of-care ultrasound (POCUS) to identify venous cannula position in neonates on extracorporeal membrane oxygenation (ECMO) and compare with conventional imaging. STUDY DESIGN Retrospective review of 37 infants on ECMO with 51 POCUS studies between January 2017 and October 2019. Studies were reviewed for identification of venous cannula location and compared with plain radiography and echocardiography. Kappa statistic and predictive values were calculated. RESULTS Venous cannula tip position was identified in 90% of POCUS studies. Fifty percent of the cannula tips were malpositioned. Plain radiography, the most commonly used method for evaluating tip position, showed poor agreement (57%) with POCUS (kappa 0.13). There was substantial agreement (89%) between echocardiography and POCUS (kappa 0.78). CONCLUSION This study provides preliminary evidence that POCUS is more accurate than plain radiography for the evaluation of ECMO venous cannula position. Adoption of this practice may prevent potentially catastrophic ECMO complications.

The safety and benefits of cardiac contrast-enhanced ultrasound (CEUS) have been demonstrated in children and adolescents for a variety of clinical indications, including congenital heart disease. Cardiac CEUS is performed with US and the intravenous administration of ultrasound contrast agents (UCAs). It improves transthoracic echocardiography, which can be challenging in children and adults with acoustic window limitations (e.g., from obesity) and alterations in chest wall and cardiac geometry (e.g., from prior surgical procedures). Cardiac CEUS is also used to evaluate ischemia in the follow-up of congenital and acquired heart disease. In 2019, the United States Food and Drug Administration (FDA) approved a UCA for pediatric echocardiography. This article focuses on the clinical applications of UCAs in pediatric and adult echocardiography, outlining its diagnostic value, safety and potential for future applications.

Introduction: Three-dimensional echocardiography (3DE) has been shown to be superior to 2DE when quantifying chamber dimensions. However, the clinical utility of 3DE has been limited by time-consuming analysis and need for 3D-specific training. This study aims to assess the clinical utility of a “hands-off,” artificial intelligence-driven software program, Philips Dynamic HeartModelA.I. (DHM), in a pediatric population. Hypothesis: We hypothesize that this program will be able to accurately calculate LVEF in children when compared to Cardiac MRI (CMRI) used as a gold standard. Methods: To date, 10 patients (Average Age 14.8±1.9) have undergone CMRI immediately followed by echocardiography at the Children’s Hospital of Philadelphia. Echocardiographic images were analyzed via DHM without manual editing and from full-volume 3D border tracing (3DEF). We excluded 3D imaging of 2 patients due to poor image quality. 2D LVEF was also evaluated visually in all patients by three experts, and by 2D Simpson’s Biplane method. All analyses were timed with a stopwatch. Each technique was correlated with CMRI. Results: Our results showed that LVEF measured via DHM was rapid and correlated very well with CMRI (R=0.85). DHM had a stronger correlation with CMRI than Biplane, 3DEF, and visual EF (R=0.56; R=0.68; R=0.57). Moreover, DHM analysis was faster than both Biplane and 3DEF (32±8 seconds vs 100±19 seconds, p Conclusions: Based on our pilot data, DHM analysis in children was faster than both 3DEF and biplane method likely due to its hands-off nature. Furthermore, its correlation with CMRI was superior to both 3DEF and Biplane. Based on further patient enrollment by us and others, this completely automated method will likely be clinically useful as it has potential to become a standard application in large volume, busy echo labs.

Introduction: Three-dimensional echocardiography (3DE) has been shown to be superior to two-dimensional echocardiography (2DE) for the quantification of chamber volumes and dimensions. However, the utility of 3DE in routine clinical practice has been restricted by time-consuming analysis and the need for 3D-specific training. This study aims to assess the accuracy and clinical utility of Philips Dynamic Heart ModelA.I. (DHM), a “hands off” automated software program, that may be used to measure LA chamber dimensions. This is notable, as indexed LA volume has been used as a predictor of heart failure in adults. Hypothesis: We hypothesize that DHM will be able to quickly and accurately calculate left atrial chamber volume when compared to Cardiac MRI (CMRI) used as a gold standard. Methods: To date, 10 patients (Average Age 14.8±1.9) have undergone clinically indicated CMRI, immediately followed by 3DE, at the Children’s Hospital of Philadelphia. 3D images were analyzed via DHM without manual editing (hands-off approach). DHM automatically calculated maximum LA volume. LA volume was also calculated from CMRI images using the biplane method. LA volume measurements by DHM were correlated with CMRI. Results: DHM was able to calculate LA volume in an average time of 32±8 seconds, without additional human input. Furthermore, inter-technique correlations showed that LA volume measurements made via DHM correlated very well with LA volume measurements made via CMRI (R=0.85, p Conclusions: In children, fully automated evaluation of LA volume based on artificial intelligence is a viable alternative to time-consuming 3D analysis when compared to a CMRI gold standard. This method of “hands-off” analysis may prove to be useful in situations where trained 3D personnel are unavailable. Furthermore, the utilization of this method may result in a greater integration of 3D LA volume into routine clinical practice.

Background Centers from Europe and United States have reported an exceedingly high number of children with a severe inflammatory syndrome in the setting of COVID-19, which has been termed multisystem inflammatory syndrome in children (MIS-C). Objectives This study aimed to analyze echocardiographic manifestations in MIS-C. Methods We retrospectively reviewed 28 MIS-C, 20 healthy controls and 20 classic Kawasaki disease (KD) patients. We reviewed echocardiographic parameters in acute phase of MIS-C and KD groups, and during subacute period in MIS-C group (interval: 5.2 ± 3 days). Results Only 1 case in MIS-C (4%) manifested coronary artery dilatation (z score=3.15) in acute phase, showing resolution during early follow up. Left ventricular (LV) systolic and diastolic function measured by deformation parameters, were worse in MIS-C compared to KD. Moreover, MIS-C patients with myocardial injury (+) were more affected than myocardial injury (-) MIS-C with respect to all functional parameters. The strongest parameters to predict myocardial injury in MIS-C were global longitudinal strain (GLS), global circumferential strain (GCS), peak left atrial strain (LAS) and peak longitudinal strain of right ventricular free wall (RVFWLS) (Odds ratio: 1.45 (1.08-1.95), 1.39 (1.04-1.88), 0.84 (0.73-0.96), 1.59 (1.09-2.34) respectively). The preserved LVEF group in MIS-C showed diastolic dysfunction. During subacute period, LVEF returned to normal (median: from 54% to 64%, p Conclusions Unlike classic KD, coronary arteries may be spared in early MIS-C, however, myocardial injury is common. Even preserved EF patients showed subtle changes in myocardial deformation, suggesting subclinical myocardial injury. During an abbreviated follow-up, there was good recovery of systolic function but persistence of diastolic dysfunction and no coronary aneurysms. Condensed abstract Multisystem inflammatory syndrome in children (MIS-C) is an illness that resembles Kawasaki Disease (KD) or toxic shock, reported in children with a recent history of COVID-19 infection. This study analyzed echocardiographic manifestations of this illness. In our cohort of 28 MIS-C patients, left ventricular systolic and diastolic function were worse than in classic KD. These functional parameters correlated with biomarkers of myocardial injury. However, coronary arteries were typically spared. The strongest predictors of myocardial injury were global longitudinal strain, right ventricular strain, and left atrial strain. During subacute period, there was good recovery of systolic function, but diastolic dysfunction persisted.

OBJECTIVE: The study was designed to assess the impact of socioeconomic barriers on the rate of prenatal diagnosis of critical congenital heart disease (CCHD). METHODS: This was a retrospective review of the Medicaid analytic extract (MAX) dataset, a national Medicaid administrative claims database with linked maternal-infant claims, from 2007 to 2012. Infants with CCHD were identified by searching for International Classification of Diseases (ICD) 9 codes and Procedural Coding System (PCS) codes for CCHD within the first 6 months after the delivery date. Multivariate logistic regression was used to evaluate the effect of maternal and socioeconomic factors on the prenatal diagnosis rate. RESULTS: There were 4702 mother-infant dyads included in the analysis. The prenatal diagnosis rate of CCHD was 27.9%. Factors independently associated with odds of prenatal diagnosis of CCHD were presence of maternal diabetes (OR, 2.055; P < .001), ZIP code level median household income (OR, 1.005; P = .015), sonographer labor quotient (OR, 1.804; P = .047), the year of the delivery (OR, 1.155; P < .001), and needing a view other than a 4 chamber or outflow tract view to obtain the diagnosis (OR, 0.383; P < .001). CONCLUSION: Maternal health, diabetes, socioeconomic factors, and access to sonographers impacts prenatal diagnosis of CCHD.

To assess the ability of point-of-care ultrasound (POCUS) to identify venous cannula position in neonates on extracorporeal membrane oxygenation (ECMO) and compare with conventional imaging.Retrospective review of 37 infants on ECMO with 51 POCUS studies between January 2017 and October 2019. Studies were reviewed for identification of venous cannula location and compared with plain radiography and echocardiography. Kappa statistic and predictive values were calculated.Venous cannula tip position was identified in 90% of POCUS studies. Fifty percent of the cannula tips were malpositioned. Plain radiography, the most commonly used method for evaluating tip position, showed poor agreement (57%) with POCUS (kappa 0.13). There was substantial agreement (89%) between echocardiography and POCUS (kappa 0.78).This study provides preliminary evidence that POCUS is more accurate than plain radiography for the evaluation of ECMO venous cannula position. Adoption of this practice may prevent potentially catastrophic ECMO complications.

Noonan Syndrome with Multiple Lentigines (NSML) and Noonan Syndrome (NS) can be difficult to differentiate clinically in early childhood. This study aims to describe characteristics of the ventricular septum that may differentiate NSML from NS. We hypothesize that the shape of the ventricular septum determined by echocardiography correlates with genotype and may distinguish patients with NSML from those with NS.We analyzed data from 17 NSML and 67 NS patients. Forty normal and 30 sarcomeric hypertrophic cardiomyopathy (HCM) patients were included as controls. Septal morphology was qualitatively evaluated, and septal angle was measured quantitatively at end diastole. We recorded the presence of a ventricular septal bulge (VSB) and reviewed genetic testing results for each patient.The most important findings were a sigmoid septum (71%) and VSB (71%) in NSML. NSML septal angle was decreased compared to the normal and sarcomeric HCM control groups, respectively (149 ± 13 vs. 177 ± 3, p 0.001; 149 ± 13 vs. 172 ± 7, p 0.001). NS septal angle was similar to the controls (176 ± 6 vs. 177 ± 3, p 0.5; 176 ± 6 vs. 172 ± 7, p 0.5). NSML-linked pathogenic variants were associated with sigmoid septum and VSB.These findings provide novel phenotypic evidence to clinicians that may offer incremental diagnostic value in counseling families in ambiguous NSML/NS cases.Characteristics of the ventricular septum are linked to specific gene variants that cause NSML and NS. Sigmoid septum and VSB are associated with NSML. This novel echocardiographic association may help clinicians distinguish NSML from NS in ambiguous cases. Early distinction between the two may be important, as syndrome-specific therapies may become available in the near future. This study may encourage further research into genotype-phenotype associations in other forms of HCM.

The early postnatal course for a newborn with critical congenital heart disease (CHD) can be negatively impacted if diagnosis is delayed. Despite this, there continues to be inconsistent evidence regarding potential benefits associated with prenatal diagnosis (PND) in neonates who undergo cardiac surgery. The objective of this study was to better define the impact of a PND on pre-operative morbidity by utilizing a large clinical database. Neonates (

Graphical abstract, Highlights • Total anomalous pulmonary venous return with no connection has a poor prognosis. • Echocardiography aids in diagnosing unique features associated with this disease. • Additional diagnostic imaging modalities may be needed to confirm diagnosis.

Background Pulmonary valve replacement is becoming the most frequent congenital heart surgery performed on adolescents and young adults. Numerous surgical options are available, including autologous pericardium, mechanical valves, allografts, and bioprosthetic valves. Each option has limitations with durability and freedom from reintervention for stenosis or insufficiency, particularly in the pediatric population. The purpose of this study was to analyze our uniquely designed, expanded polytetrafluoroethylene (ePTFE) valved conduit (VC) for right ventricular outflow tract reconstruction. Methods Beginning in 2012, ePTFE VC were implanted in 26 patients with a median age of 9.8 years (range, 1.0 to 15.9). Bicuspid VC were used in the first 3 patients, and tricuspid VC were used in 23 patients. The ePTFE VC is fashioned from commercially available ePTFE tube graft (16 mm in 1 patient, 20 mm in 7 patients, 24 mm in 18 patients) and 0.1 mm thick ePTFE membrane for the leaflet material. Valve function was assessed by echocardiogram after the implantation. Results There were no postoperative hospital deaths. Mean follow-up was 2.6 years (range, 4 months to 4.7 years), and there have been no reoperations for the ePTFE VC. There was one reintervention for stenosis at the distal anastomosis. Pulmonary insufficiency was mild or less in 24 patients (92%). The average peak instantaneous pressure gradient between the right ventricle and the pulmonary artery was 22.4 ± 15.1 mm Hg at latest follow-up. Conclusions Compared with historical data for other pulmonary valve replacement options, our ePTFE VC shows superior medium-term performance, with less reintervention or significant valve dysfunction.

PURPOSE: In children with a mitral annulus too small to accommodate traditional prostheses, surgical implantation of stent-based valves is a promising option. However, no reliable pre-operative methods exist to guide patient selection, device sizing and positioning. We describe a novel methodology to visualize and quantify device fit in three-dimensional echocardiogram (3DE)-derived heart models. DESCRIPTION: Heart models were created from existing pre-operative 3DE using custom software. Valve models were virtually implanted into the models and both device fit and left ventricular outflow tract (LVOT) area were quantified. EVALUATION: The 3DE of three patients who underwent Melody valve placement in the mitral position were retrospectively modeled — one with left ventricular outflow tract obstruction(LVOTO), one with perivalvar leak, and one without complications. In all cases 2D measurements underestimated 3D annular dimensions, and the patient with clinical LVOT obstruction had the lowest predicted LVOT area/Aortic area ratio (0.5). CONCLUSIONS: 3DE based pre-operative modeling of surgical implantation of stent-based valves in the mitral position may improve quantification of mitral valve dimensions and inform risk stratification for potential LVOTO.

Patients with functional single ventricles after the Fontan procedure have abnormal cardiac mechanics. The aims of this study were to determine factors that influence diastolic function and to describe associations of diastolic function with current clinical status.Echocardiograms were obtained as part of the Pediatric Heart Network Fontan Cross-Sectional Study. Diastolic function grade (DFG) was assessed as normal (grade 0), impaired relaxation (grade 1), pseudonymization (grade 2), or restrictive (grade 3). Studies were also classified dichotomously (restrictive pattern present or absent). Relationships between DFG and pre-Fontan variables (e.g., ventricular morphology, age at Fontan, history of volume-unloading surgery) and current status (e.g., systolic function, valvar regurgitation, exercise performance) were explored.DFG was calculable in 326 of 546 subjects (60%) (mean age, 11.7 ± 3.3 years). Overall, 32% of patients had grade 0, 9% grade 1, 37% grade 2, and 22% grade 3 diastolic function. Although there was no association between ventricular morphology and DFG, there was an association between ventricular morphology and E', which was lowest in those with right ventricular morphology (P .001); this association remained significant when using Z scores adjusted for age (P .001). DFG was associated with achieving maximal effort on exercise testing (P = .004); the majority (64%) of those not achieving maximal effort had DFG 2 or 3. No additional significant associations of DFG with laboratory or clinical measures were identified.Assessment of diastolic function by current algorithms results in a high percentage of patients with abnormal DFG, but few clinically or statistically significant associations were found. This may imply a lack of impact of abnormal diastolic function on clinical outcomes in this cohort, or it may indicate that the methodology may not be applicable to pediatric patients with functional single ventricles.

Prenatal diagnosis of critical congenital heart disease (CCHD) can lead to safe delivery planning, as well as reduce neonatal morbidity and mortality. There is wide regional variation in the prenatal diagnosis rates of CCHD, and the reasons for this variation are poorly understood. We analyzed the

Quantifying unbalance, the threshold for single ventricle palliation vs biventricular repair in patients with unbalanced complete atrioventricular septal defect (AVSD), is challenging. Using a core laboratory review of baseline echocardiograms, we sought to assess the correlations among commonly used measures of unbalance and common atrioventricular valve (AVV) and ventricular sizes. A single reviewer evaluated baseline echocardiograms from an inception cohort of babies age 1 year with complete AVSD admitted to 1 of 25 Congenital Heart Surgeon's Society institutions. A standardized echo review protocol of 111 quantitative and qualitative measures was used. Descriptive statistics were computed and Pearson correlation coefficients were calculated to assess correlation among unbalance indices with valvar and ventricular dimensions. Two-hundred fifty-seven baseline echocardiograms of infants with complete AVSD were included. Median age at baseline echocardiogram was 11 days (interquartile range 1-79) and mean atrioventricular valve index was 0.45 ± 0.1. Mean right ventricle/left ventricle inflow angle was 90.2 ± 15.6° and median left ventricular inflow index was 0.46 (interquartile range 0.4-0.5). There are weak or moderate correlations between the measures of unbalance. Correlations between the measures of unbalance with common AVV leaflet or ventricular sizes are also weak to moderate, when statistically significant. Measures of unbalance in common clinical use correlate poorly, or not at all, with one another, common AVV, and ventricular dimensions. The concept of "unbalance" is difficult to define using baseline echocardiographic indices. These findings suggest that the indices may describe different morphologic and functional characteristics. Further analysis is necessary to quantify the contributions of unbalance indices to patient outcome.

While counseling parents of a fetus diagnosed with hypoplastic left heart syndrome (HLHS), pediatric cardiologists play a critical role in shaping a family's expectations for the months and years to come. However, techniques for the most effective counseling practices have not been studied, and significant variation among physicians is likely present. Web-based survey of pediatric cardiologists that perform fetal echocardiography using snowball sampling. 201 physicians responded (61% male, 81% from academic centers, and 95% from the U.S.), with an average experience of 12 years. The majority of respondents (73%) typically received initial referrals for HLHS between 20 and 24 weeks of gestation. Most physicians counsel families alone (54%), while others counsel with a nurse (35%), social worker (12%), and/or maternal-fetal medicine colleague (15%). Termination of pregnancy was discussed by 79% of respondents, although 15% did not know their state's legal limit for termination. While initial counseling sessions routinely described the typical earlier ramifications of HLHS, many long-term sequelae of the disease were not commonly discussed. Content of counseling was affected by region of the country, but not by practice setting, experience, or fetal volume. Respondents identified multiple barriers that limited their counseling practices. Our data suggest that current counseling practices often fail to cover important information. Perceived barriers to a full discourse on long-term sequelae of HLHS are common and may lead to a disconnect between reality and a family's understanding of the natural history of palliated HLHS. Opportunities to improve counseling practices exist, and there may be benefits to gain from more formal training.

Outcomes in patients with ventricular dysfunction undergoing superior cavopulmonary connection (SCPC) are not well known. We reviewed records of patients undergoing SCPC at our center from December 2005 to October 2009 and studied those whose pre-SCPC echocardiograms demonstrated at least moderate systemic ventricular dysfunction. Of the 213 patients undergoing SCPC, 19 (9%) met inclusion criteria. Diagnoses were hypoplastic left heart syndrome (n = 18) and rightward unbalanced atrioventricular canal with pulmonary stenosis (n = 1). In those surviving >2 months after SCPC, ventricular function was assessed by echocardiography 4.9 (range 3.5 to 9.7) months after SCPC and was improved in ten of 17 (59%), unchanged in six of 17 (35%), and worsened in one of 17 (16%) patients. After SCPC, three patients died, and one underwent heart transplant 21.9 months after SCPC. Transplant-free survival was attained by 15 of 19 (79%) patients during follow-up of 33.0 months (range 10.8 to 51.4). Fontan completion was performed on six survivors on reaching an appropriate age. Ventricular dysfunction before SCPC is not uncommon and occurs primarily in patients with a morphologic right ventricle. Ventricular performance improves in the majority of patients after SCPC, and midterm outcomes are comparable with previous reports, suggesting that these patients remain candidates for staged palliation.

Background Previous studies have demonstrated a correlation between E:Ea and ventricular filling pressure in adults after heart transplantation. We sought to determine if E:Ea correlates with filling pressure after heart transplantation in children.

Hall EK, Glatz AC, Quartermain MD, Ravishankar C, Kaufman B, Cohen MS, Hanna BD, Goldberg DJ. Brain-type natriuretic peptide correlates with right heart pressures in a cross section of pediatric heart transplant patients. Pediatr Transplantation 2011: 15:70–74. © 2010 John Wiley & Sons A/S. Abstract: Serum brain-type natriuretic peptide level (BNP) correlates with hemodynamic parameters measured during cardiac catheterization in adult patients with heart failure. We sought to describe the relationship of BNP with invasive hemodynamic measurements and cellular rejection in children following OHT. Children undergoing catheterization for OHT surveillance had simultaneous measurement of BNP. A total of 62 subjects were studied. The median BNP was 171 pg/mL (range 19–1130). There were significant positive correlations between BNP and mean PAP (R = 0.33, p = 0.009), RVSP (R = 0.25, p = 0.05), RVEDP (R = 0.29, p = 0.02), and mean RAP (R = 0.39, p = 0.002). Rejection grade varied from 0 to 3A (58 patients < ISHLT 3A and four patients ≥ ISHLT 3A). There was no significant difference in BNP based on cellular rejection grade. In a cohort of pediatric patients after heart transplantation, BNP correlates with direct measurements of right-sided pressures, but not with other hemodynamic measurements, time from transplant or rejection grade. This suggests that BNP may have a complimentary role in the monitoring of children following heart transplantation.

OBJECTIVES: The majority of previous studies that described the neuropsychological effects of cardiopulmonary bypass (CPB) in children were performed after surgery in infancy for complex congenital heart disease (CHD). We sought to limit confounding variables and isolate potential independent effects of CPB by describing neuropsychological function in school-aged children after repair of acyanotic CHD. METHODS: This was a prospective study of patients who were aged 5 to 18 years and undergoing repair of acyanotic CHD. Neuropsychological testing battery included assessment of intelligence, memory, motor, attention, executive function, and behavior before and 6 months after CPB. The independent effects of anesthesia, surgery, and hospitalization on neuropsychological function were assessed by testing a surgical control group of patients who were undergoing repair of pectus deformities. In addition, an outpatient group of children with mild CHD were enrolled to assess the practice effects of serial testing. RESULTS: Patients included CPB (n = 35), surgical control (n = 19), and nonsurgical (n = 12). Groups were comparable in age, gender, and race and demonstrated similar unadjusted group mean scores on baseline and 6-month follow-up neuropsychological testing. When adjusted for practice effects, the CPB group performed similar to the non-CPB groups in all assessed neuropsychological domains, with the exception of 1 of 4 tests of executive function. CONCLUSIONS: When controlling for the non-CPB effects of surgery (eg, hospitalization, anesthesia, thoracotomy) and the practice effects of serial testing, there were no consistent independent effects of CPB on neuropsychological status in a cohort of children and adolescents 6 months after repair of acyanotic CHD.

Background Prenatal ventricular size discrepancy with disproportionately smaller left ventricle than right ventricle (L-R/VD) can be a marker for important left-sided structural heart disease in the newborn. Methods We reviewed the echocardiograms of all fetuses evaluated at our center with L-R/VD from July 1, 2004 to January 1, 2008. Results Of the 35 fetuses, 20 (57%) had critical arch obstruction and underwent neonatal intervention (group 1); 15 (43%) did not require newborn intervention (group 2). Ratios comparing left with right heart structures were significantly lower in group 1 fetuses compared with group 2 fetuses. Aortic arch measurement ≤ 3 mm was the most sensitive, and abnormal direction of atrial level shunting was the most specific measure to predict the need for neonatal aortic arch intervention. Conclusion Ratios expressing the magnitude of L-R/VD, direction of flow at the atrial septum, and measurements of the aortic arch help identify fetuses that will require neonatal intervention.

Background— Transcatheter closure of secundum atrial septal defects (ASD) using the Amplatzer septal occluder is generally safe and effective, but erosion into the pericardial space or aorta has been described. Although the absolute risk of this complication is low, there has been no assessment of relative risk factors. Methods and Results— All erosions reported to St. Jude Medical after ASD closure with an Amplatzer septal occluder (cases) were compared with controls (matched 2:1) who underwent ASD closure but did not develop an erosion. A total of 125 erosions were reported between 2002 and 2014, including 95 with an available echocardiogram. The median duration from implant to erosion was 14 days, but was >1 year in 16 patients. Nine patients (all age ≥17 years) who died were more likely to have an oversized device, and to have erosion into the aorta, than survivors. Aortic or superior vena cava rim deficiencies were more common in cases than in controls. In addition, larger balloon-sized ASD diameter, Amplatzer septal occluder device size, and device size–ASD diameter difference, and smaller weight:device size ratio were associated with erosion. On multivariable analysis, deficiency of any rim, device >5 mm larger than ASD diameter, and weight:device size ratio were associated with erosion. Conclusions— In addition to aortic rim deficiency, which was almost universal among erosion cases, there were several relative risk factors for erosion after ASD closure with the Amplatzer septal occluder device. To understand the mechanisms of and absolute risk factors for this uncommon but serious complication, an adequately powered prospective study with thorough echocardiographic evaluation will be critical.

Background: Guidelines for evaluating left ventricular (LV) diastolic dysfunction in adults, often do not apply in children. We hypothesize left atrial (LA) stiffness may serve as a noninvasive surrogate of LV diastolic dysfunction in children with cardiomyopathy (CMP). Methods: We studied 41 CMP

Single umbilical artery (SUA) has been associated with an increased risk of congenital heart disease (CHD). Women carrying fetuses with an SUA are often referred for fetal echocardiography, but data to support the need for this testing remain controversial.A retrospective review of the records for all women carrying fetuses with an SUA who had undergone fetal echocardiography between 2009 and 2012 at our center was performed. Data on the maternal and fetal risk factors for CHD were collected, and the fetuses were categorized into three groups: low risk (LR; an SUA with no additional risk factors for CHD), moderate risk (MR; an SUA with one additional risk factor for CHD), and high risk (HR; an SUA with two or more additional risk factors for CHD).In total, 101 such patients were identified: 69 LR, 26 MR, and 6 HR. No fetuses in the LR group, three in the MR group, and two in the HR group had CHD (p = 0.0005).An SUA in an LR fetus did not increase the risk of CHD in our cohort, whereas an SUA in the presence of additional risk factors was associated with significantly increased risk for CHD. Our results suggest that referral for a fetal echocardiogram is indicated for women carrying fetuses with an SUA when additional risk factors for CHD are present. In an LR fetus with an SUA, however, echocardiography may not provide additional benefit unless CHD is suggested on screening obstetric sonography.

In anomalous left coronary artery from the pulmonary artery (ALCAPA), infants 6 to 12 weeks will often present with symptoms consistent with reflux or bronchiolitis. In those infants diagnosed with both ALCAPA and concomitant active respiratory syncytial virus (RSV) bronchiolitis, others have reported delaying revascularization therapy until resolution of the RSV bronchiolitis. Here, we report the case of a three-month-old infant, diagnosed with ALCAPA and active RSV bronchiolitis, who underwent successful myocardial revascularization within 24 hours of presentation and diagnosis.

Infants with shunt-dependent single-ventricle (SV) physiology are at risk for poor weight gain before superior cavopulmonary connection (SCPC). Lower weight-for-age z-score is a risk factor for prolonged length of stay (LOS) after SCPC. We sought to characterize infant growth and feeding and determine the effect of method of feeding on outcomes. Chart review of infants with shunt-dependent SV physiology born between October 2007 and September 2010 was performed. The cohort was divided into three groups based on feeding method at discharge after initial palliation; 53 in the oral feeding (PO) group, 56 in the nasogastric (NG) tube group, and 26 in the gastrostomy tube (GT) group. Birth weight z-score did not differ among groups (p = 0.39), but infants fed by NG or GT were smaller than PO-fed infants at hospital discharge (p = 0.0001), a difference that persisted through SCPC (p < 0.0001). Predictors of need for GT included Norwood procedure (p = 0.008) and longer LOS after initial palliation (p < 0.001). Interstage mortality and age at SCPC did not differ among groups. Risk factors for longer LOS at SCPC included longer LOS and need for supplemental feeds at discharge from initial palliation as well as lower weight at SCPC. Poor growth is common among infants with shunt-dependent SV physiology. Infants who require GT have lower weight-for-age z-scores at hospital discharge and remain smaller at SCPC than those fed PO. Although GT after initial palliation is associated with longer LOS after SCPC, it is not associated with an increase in interstage morbidity or mortality.

A 4-year-old asymptomatic girl presented for elective transcatheter closure of her ostium secundum atrial septal defect (ASD). Her examination revealed a fixed split second heart sound with a 2/6 systolic ejection murmur at the left upper sternal border and a right ventricular lift. Transthoracic echocardiogram demonstrated a 15-mm secundum ASD with a mildly dilated right ventricle. The left coronary artery (LCA) could not be readily identified on the study. The patient was referred for percutaneous device closure when a preprocedural transesophageal echocardiogram revealed a moderate-sized secundum ASD with left to right shunting, moderate right ventricle dilation, and an anomalous origin of the LCA from the right sinus of Valsalva was identified (Figure 1A). The origin and distribution of the right coronary artery was normal. Cardiac catheterization revealed a Qp:Qs …

ObjectiveThe objective of this study was to describe characteristics and early outcomes across a large multicenter cohort undergoing coarctation or hypoplastic aortic arch repair.MethodsPatients undergoing coarctation or hypoplastic aortic arch repair (2006-2010) as their first cardiovascular operation in the Society of Thoracic Surgeons Congenital Heart Surgery Database were included. Group 1 patients consisted of those with coarctation or hypoplastic aortic arch without ventricular septal defect (coarctation or hypoplastic aortic arch, isolated); group 2, coarctation or hypoplastic aortic arch with ventricular septal defect (coarctation or hypoplastic aortic arch, ventricular septal defect); and group 3, coarctation or hypoplastic aortic arch with other major cardiac diagnoses (coarctation or hypoplastic aortic arch, other).ResultsThe cohort included 5025 patients (95 centers): group 1, 2705 (54%); group 2, 840 (17%); and group 3, 1480 (29%). Group 1 underwent coarctation or hypoplastic aortic arch repair at an older age than groups 2 and 3 (groups 1, 2, and 3, 75%, 99%, and 88%

Objectives: Twin-twin transfusion syndrome (TTTS) is a complex disorder with altered cardiovascular loading conditions that affects both donors and recipients. Myocardial tissue deformation analysis using vector velocity imaging is an angle-independent, speckle-tracking technique which can assess myocardial mechanics and may provide insight into cardiac dysfunction in TTTS. Methods: Digital dynamic two-dimensional four-chamber views were interrogated offline. Images were acquired utilizing standard video frame rates (30 frames/s). The global longitudinal strain, systolic strain rate, and diastolic strain rate were measured in the left (LV) and right ventricles (RV) of 25 fetal pairs with TTTS and compared to 25 gestational age-matched normal controls. Pulsatility indices for the umbilical artery and middle cerebral artery were measured. Results: The gestational age at evaluation was 20.5 ± 1.3 weeks. The donor LV systolic strain rate was higher, while the donor RV diastolic strain rate was significantly lower, than control values. The recipient longitudinal strain, systolic strain rate, and diastolic strain rate were significantly lower for both LV and RV in comparison to controls. The donor umbilical artery pulsatility index was higher than control values (1.92 ± 0.45 vs. 1.41 ± 0.25, p < 0.001), while the donor middle cerebral artery pulsatility index was lower (1.46 ± 0.28 vs. 1.87 ± 0.21). Recipient umbilical artery and middle cerebral artery pulsatility indices were no different than control values. Conclusions: In TTTS, both the donor and the recipient exhibit abnormalities of myocardial tissue deformation with ventricle-specific changes evident based on loading conditions. Donor LV systolic function is hyperdynamic due to hypovolemia and selective ejection into a low-resistance cerebrovascular circuit while the donor RV selectively ejects into a high-resistance placental circuit. Recipient RV and LV are both globally depressed with systolic and diastolic dysfunction. Further prospective validation of our findings using high frame rate analysis is indicated.

Objective To identify prenatal echocardiographic markers that could predict the need for neonatal intervention in fetuses with right ventricular outflow tract obstruction. Methods This was a retrospective study of 52 fetuses with right ventricular outflow tract obstruction. Echocardiograms were evaluated for fetuses with either two-ventricle anatomy with a large ventricular septal defect or single-ventricle anatomy. Fetuses with pulmonary atresia were excluded. Parameters were compared between groups that did and did not require an intervention at age

While counseling parents of a fetus diagnosed with hypoplastic left heart syndrome (HLHS), pediatric cardiologists play a critical role in shaping a family’s expectations for the months and years to come. However, techniques for the most effective counseling practices have not been studied, and significant variation among physicians is likely present. Web-based survey of pediatric cardiologists that perform fetal echocardiography using snowball sampling. 201 physicians responded (61% male, 81% from academic centers, and 95% from the U.S.), with an average experience of 12 years. The majority of respondents (73%) typically received initial referrals for HLHS between 20 and 24 weeks of gestation. Most physicians counsel families alone (54%), while others counsel with a nurse (35%), social worker (12%), and/or maternal-fetal medicine colleague (15%). Termination of pregnancy was discussed by 79% of respondents, although 15% did not know their state’s legal limit for termination. While initial counseling sessions routinely described the typical earlier ramifications of HLHS, many long-term sequelae of the disease were not commonly discussed. Content of counseling was affected by region of the country, but not by practice setting, experience, or fetal volume. Respondents identified multiple barriers that limited their counseling practices. Our data suggest that current counseling practices often fail to cover important information. Perceived barriers to a full discourse on long-term sequelae of HLHS are common and may lead to a disconnect between reality and a family’s understanding of the natural history of palliated HLHS. Opportunities to improve counseling practices exist, and there may be benefits to gain from more formal training.

Serum brain-type natriuretic peptide level (BNP) correlates with hemodynamic parameters measured during cardiac catheterization in adult patients with heart failure. We sought to describe the relationship of BNP with invasive hemodynamic measurements and cellular rejection in children following OHT. Children undergoing catheterization for OHT surveillance had simultaneous measurement of BNP. A total of 62 subjects were studied. The median BNP was 171 pg/mL (range 19-1130). There were significant positive correlations between BNP and mean PAP (R=0.33, p=0.009), RVSP (R=0.25, p=0.05), RVEDP (R=0.29, p=0.02), and mean RAP (R=0.39, p=0.002). Rejection grade varied from 0 to 3A (58 patientsISHLT 3A and four patients ≥ ISHLT 3A). There was no significant difference in BNP based on cellular rejection grade. In a cohort of pediatric patients after heart transplantation, BNP correlates with direct measurements of right-sided pressures, but not with other hemodynamic measurements, time from transplant or rejection grade. This suggests that BNP may have a complimentary role in the monitoring of children following heart transplantation.

Objective To determine whether the mode of delivery of infants prenatally diagnosed with hypoplastic left heart syndrome (HLHS) affects markers of perinatal hemodynamics. Study design A retrospective review of patients diagnosed prenatally with HLHS and delivered within our institution was undertaken. Arterial blood gases, echocardiographic data, and markers of end organ function were compared based on route of delivery. Results A total of 79 infants with HLHS were enrolled between January 2002 and December 2008. The infants delivered by elective cesarian delivery (CD) had younger gestational age compared with those delivered by vaginal delivery (VD) or by urgent CD/operative VD. Those delivered by elective CD had lower pH and higher partial pressure of CO2 on arterial cord blood gas analysis. There were no differences in partial pressure of O2 and base deficit among the 3 study groups. One-minute and 5-minute Apgar scores, markers of end organ function, echocardiographic parameters, length of hospitalization, and survival to discharge were similar among the groups. Conclusions Overall, newborns with a prenatal diagnosis of HLHS transitioned well to extrauterine life without significant acidosis regardless of the mode of delivery. Delivery of newborns with HLHS by elective CD did not demonstrate any hemodynamic advantage over VD in our cohort of patients.

Objectives To review our experience with the prenatal diagnosis of hypoplastic left heart syndrome (HLHS). Our goal was to establish the benchmark for perinatal and early surgical outcome in the current era, from a center with an aggressive surgical approach and a cohort with a high level of intention-to-treat. Methods Outcome was assessed in fetuses with HLHS following stratification into high-risk and standard-risk categories. High risk was defined as the presence of any of the following: extracardiac, genetic or chromosomal anomalies; prematurity of < 34 weeks' gestation; additional cardiac findings such as intact or highly restrictive atrial septum, severe degree of tricuspid regurgitation or severe ventricular dysfunction. Standard risk was defined as absence of these risk factors. Results Of 240 fetuses evaluated over 5 years, 162 (67.5%) were in the standard-risk group and 78 (32.5%) were in the high-risk group. Of the 240 sets of parents, 38 (15.8%) chose termination or non-intervention at birth at initial prenatal counseling and 185 of the neonates (77.1%) underwent first-stage Norwood surgery with 155 surviving and 30 deaths, giving an overall Norwood operative survival of 83.8%. Breakdown by risk class reveals a significant Norwood operative survival advantage for the standard-risk group (92.8%) over the high-risk group (56.5%) (P < 0.001). Conclusions Following prenatal diagnosis of HLHS, families should be strongly encouraged to undertake comprehensive prenatal evaluation in order to obtain an accurate prognosis. One-third have additional risk factors that limit survival outcome, however two-thirds do not and have an excellent chance of early survival. Copyright © 2010 ISUOG. Published by John Wiley & Sons, Ltd.

Background: Previous studies describing the neurocognitive (neuro) effects of cardiopulmonary bypass (CPB) have been performed following surgery in infancy for complex congenital heart disease (CHD) and in adults with acquired heart disease. These patients (pts) have many causes for adverse neuro outcomes with conflicting results regarding the contribution of CPB. The impact of CPB in children with less complex CHD is unknown. We sought to describe the effects of CPB on neuro function in children following repair of acyanotic CHD. Methods: We performed a prospective cohort study of pts 5–18 yrs of age undergoing primary repair of CHD. Neuro testing battery included assessment of intellectual function (IQ), memory, motor skills, attention, executive function, and an evaluation by a pediatric neurologist pre- and 6 months post-CPB. Non-CPB effects of surgery were assessed by similarly testing pts undergoing repair of pectus excavatum (non-CPB). Practice effects of serial testing were controlled by enrolling non-surgical pts (mild CHD) and creating a reliability-stability index to adjust surgical group scores. Anxiety was assessed as a covariate. Results: CPB pts ( n =41) included repair of atrial [17] or ventricular [6] septal defect, coronary anomaly [8], aortic valve [5] or other [5]. Control pts included pectus repair ( n =20) and non-surgical CHD pts ( n =16). Groups were similar in age, gender, race, socioeconomic status and baseline scores. Neurological exams before and after surgery were not different between groups. Controlling for the effects of serial testing, there were no significant (p1 SD) decline in scores after surgery. Conclusions : When controlling for the non-CPB effects of surgery (hospitalization, anxiety, etc.) and the practice effects of serial testing, there were no independent effects of CPB on neuro status 6 months after repair of acyanotic CHD. We believe that the potential neuro sequelae of CPB should not be a major factor in the decision to recommend CHD surgery in otherwise healthy children.

Prenatal diagnosis allows for improved peri-operative outcomes of fetuses with certain forms of congenital heart disease (CHD). Variability in prenatal diagnosis has been demonstrated in other countries, leading to efforts to improve access to care and fetal imaging protocols, but has not been