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Background Ventricular interdependence may account for altered ventricular mechanics in congenital heart disease. The present study aimed to identify differences in load-dependent right ventricular (RV)–left ventricular (LV) interactions in porcine models of pulmonary stenosis (PS) and pulmonary insufficiency (PI) by invasive admittance-derived hemodynamics in conjunction with noninvasive cardiovascular magnetic resonance (CMR). Methods Seventeen pigs were used in the study (7 with PS, 7 with PI, and 3 controls). Progressive PS was created by tightening a Teflon tape around the pulmonary artery, and PI was created by excising 2 leaflets of the pulmonary valve. Admittance catheterization data were obtained for the RV and LV at 10 to 12 weeks after model creation, with the animal ventilated under temporary diaphragm paralysis. CMR was performed in all animals immediately prior to pressure–volume catheterization. Results In the PS group, RV contractility was increased, manifested by increased end-systolic elastance (mean difference, 1.29 mm Hg/mL; 95% confidence interval [CI], 0.57-2.00 mm Hg/mL). However, in the PI group, no significant changes were observed in RV systolic function despite significant changes in RV diastolic function. In the PS group, LV end-systolic volume was significantly lower compared with controls (mean difference, 25.1 mL; 95% CI, -40.5 to -90.7 mL), whereas in the PI group, the LV showed diastolic dysfunction, demonstrated by an elevated isovolumic relaxation constant and ventricular stiffness (mean difference, 0.03 mL−1; 95% CI, -0.02 to 0.09 mL−1). Conclusions The LV exhibits systolic dysfunction and noncompliance with PI. PS is associated with preserved LV systolic function and evidence of some LV diastolic dysfunction. Interventricular interactions influence LV filling and likely account for differential effects of RV pressure and volume overload on LV function.

Prior to the 1990s, d-TGA was palliated with the atrial switch procedure resulting in a systemic right ventricle associated with significant long-term morbidity and mortality. Determining the optimal timing of heart transplantation (HT) in these patients has been difficult. While cardiopulmonary exercise testing (CPET) is commonly used to try and risk stratify these patients, traditional exercise parameters have lacked the sensitivity and specificity to assess long-term risk. We sought to assess changes in exercise parameters over time in order to determine if any CPET parameter or combination of parameters could reliably identify risk for adverse outcome in this patient group. A retrospective review of serial CPET for 40 patients over 17 years was completed. Patients with adverse event within 6 months prior to CPET were noted. CPET parameters were compared and linear mixed model regression with repeated measures was performed on serial tests for longitudinal assessment. The linear mixed model regression identified OUES indexed to BSA to be the most sensitive parameter in identifying patients at risk of adverse event and became a stronger predictor of adverse event when combined with peak heart rate. CPET is useful in identifying patients with atrial switch at increased risk of adverse outcome. Indexed OUES and peak heart rate are better prognostic indicators than VO2 and VE/VCO2.

Background The aims of this study were to investigate the dynamic changes in the vena contracta (VC) and proximal isovelocity surface area (PISA) through systole in patients with hypoplastic left heart syndrome and tricuspid regurgitation and to identify the stage of systole (early, mid, or late) in which VC and PISA radius are optimal. Methods Twenty-eight patients with hypoplastic left heart syndrome were prospectively studied using continuous two-dimensional (2D) and three-dimensional (3D) echocardiography. Two-dimensional VC width, 3D VC area, and PISA radii (2D and 3D) were measured frame by frame throughout systole. The maximal 2D VC width, 3D VC area, and PISA radii in the first, middle, and last thirds of systole were compared, and correlations were explored with 3D tricuspid annular areas, right atrial volumes, and right ventricular volumes. Results In all, 35 data sets that met inclusion criteria were analyzed. On frame-by-frame analysis, maximal 2D VC width and 3D VC area were found in the first third of systole in 17% and 20% of studies, in the second third in 34% and 31%, and in the final third in 49% and 49%. Similarly, the maximal 2D and 3D PISA radii were found in the first third of systole in 26% and 17% of studies, in the second third in 28% and 34%, and in the final third in 46% and 49%. Conclusions In hypoplastic left heart syndrome, detailed temporal analysis of tricuspid regurgitation–associated VC and PISA by 2D and 3D echocardiography reveals no reliable pattern predicting when in systole these parameters peak. Frame-by-frame measurement is necessary for identification of maximal VC and PISA radius on 2D and 3D color Doppler echocardiography because the severity of tricuspid regurgitation could be underestimated because of temporal variability in VC and PISA.

Heart murmur, a thoracic auscultatory finding of cardiovascular origin, is extremely common in childhood and can appear at any age from premature newborn to late adolescence. The objective of this review is to provide a modern examination and update of cardiac murmur auscultation in this new era of artificial intelligence (AI) and telemedicine. First, we provide a comprehensive review of the causes and differential diagnosis, clinical features, evaluation, and long-term management of paediatric heart murmurs. Next, we provide a brief history of computer-assisted auscultation and murmur analysis, along with insight into the engineering design of the digital stethoscope. We conclude with a discussion of the paradigm shifting impact of deep learning on murmur analysis, AI-assisted auscultation, and the implications of these technologies on telemedicine in paediatric cardiology. It is our hope that this article provides an updated perspective on the impact of AI on cardiac auscultation for the modern paediatric cardiologist.

Objective The objective of this study was to assess papillary muscle (PM) and mitral valve (MV) structure and function in children and young adults with mild and moderate hypertrophic cardiomyopathy (HCM) using real-time three-dimensional echocardiography (3DE) and to correlate them with HCM related adverse outcomes. Methods Transthoracic research 3DE was performed in HCM patients and controls matched for age and gender. Anterolateral and posteromedial PM mass, apical displacement of anterolateral PM, and left ventricular (LV) mass were measured and indexed to body surface area. The MV annulus and leaflet structure and function were analyzed. Individual PMs were manually planimetered by tracing the endocardial borders on each mid systole frame, taking care to distinguish PMs as distinct from the LV wall. Apical PM displacement was expressed as ratio of the distance between the apex and the base of the anterolateral PM to the entire length of the LV lateral wall (APL index). All 3DE measurements were correlated to adverse outcomes. Results Forty subjects were studied, including 20 HCM patients (age 18.1 ± 9.6 years, 16 male and 4 female), and 20 controls (18.2 ± 9.6 years, 16 male and 4 female). The indexed LV mass in HCM was 74.8 ± 25.8 g/m2 compared to 50.8 ± 12.4 g/m2 in controls (p = 0.001). The anterolateral, posteromedial and combined PM mass were 3.1 ± 2.2 g/m2, 1.7 ± 1.2 g/m2 and 4.9 ± 2.7 g/m2 in HCM, in contrast to respective measurements of 1.1 ± 0.6 g/m2, 1.2 ± 0.6 g/m2 and 2.3 ± 0.8 g/m2 in controls (p Conclusion It is feasible to evaluate PM muscles and MV annulus geometry in children and young adults with HCM using 3DE. The morphologic and functional changes of anterolateral PM may occur in the absence of MV annulus changes. Prospective validation will be required to determine if LV lateral wall length and LV mass may be used as predictors of adverse events.

BACKGROUND The size of the Fontan population with end-stage heart failure is growing. In this population, heart transplantation has been the only option. This study sought to investigate the efficacy of ventricular assist device (VAD) support in Fontan patients. METHODS We conducted a retrospective study of Fontan patients in the Advanced Cardiac Therapies Improving Outcomes Network. We evaluated patient characteristics, and the clinical and physiologic outcomes after VAD implantation. RESULTS We identified 45 Fontan patients implanted with VAD. The average age of patients was 10 years (interquartile range: 4.5–18) and 30% were female. The majority had a morphologic right ventricle (69%), moderate or greater ventricular dysfunction (83%), and moderate or greater atrioventricular valve regurgitation (65%). The majority of implants were as a bridge to transplantation (76%), and the majority of patients were Interagency Registry for Mechanically Assisted Circulatory Support Profile 2 (56%). The most commonly employed device was the Medtronic HeartWare HVAD (56%). A total of 13 patients were discharged on device support, and 67% of patients experienced adverse events, the most common of which were neurologic (25%). At 1 year after device implantation, the rate of transplantation was 69.5%, 9.2% of patients continued to be VAD supported, and 21.3% of patients had died. Hemodynamically, VAD was effective in decreasing both Fontan and ventricular end-diastolic pressures in some individuals. CONCLUSIONS VAD is effective in supporting patients with end-stage Fontan failure awaiting heart transplantation. Future research should focus on identifying clinical and physiologic characteristics predictive of a favorable response to VAD support.

BACKGROUND We implemented sophisticated color M-mode analysis to assess age-dependent progression of left ventricular (LV) diastolic function. METHODS Normal infants were prospectively enrolled for serial echocardiograms at 1 week, 1 month, 6 months, 1 year, and 2 years. From color M-mode scans, propagation velocity (VP), strength of filling (VS), and intraventricular pressure difference (IVPD) in 3 segments along apex-to-mitral valve scan line were measured. RESULTS Age-wise comparisons of diastolic filling from 121 echocardiograms in 31 infants showed VP (cm/s), VS (cm2/s), and E-wave IVPD (mmHg) at 1 week to be 66.2 ± 11.9, 75.3 ± 19.9, and 1.5 ± 0.4, respectively, while VP, VS, and E-wave IVPD at 1 month were 80.3 ± 14.4, 101.2 ± 28.3, and 2.42 ± 1.1, respectively. There were significant differences in VP and segmental IVPD between first week and first month (p

PURPOSE OF REVIEW Artificial intelligence (AI) has changed virtually every aspect of modern life, and medicine is no exception. Pediatric cardiology is both a perceptual and a cognitive subspecialty that involves complex decision-making, so AI is a particularly attractive tool for this medical discipline. This review summarizes the foundational work and incremental progress made as AI applications have emerged in pediatric cardiology since 2020. RECENT FINDINGS AI-based algorithms can be useful for pediatric cardiology in many areas, including: (1) clinical examination and diagnosis, (2) image processing, (3) planning and management of cardiac interventions, (4) prognosis and risk stratification, (5) omics and precision medicine, and (6) fetal cardiology. Most AI initiatives showcased in medical journals seem to work well in silico, but progress toward implementation in actual clinical practice has been more limited. Several barriers to implementation are identified, some encountered throughout medicine generally, and others specific to pediatric cardiology. SUMMARY Despite barriers to acceptance in clinical practice, AI is already establishing a durable role in pediatric cardiology. Its potential remains great, but to fully realize its benefits, substantial investment to develop and refine AI for pediatric cardiology applications will be necessary to overcome the challenges of implementation.

Objective:This prospective observational study evaluates the utility of expert focused cardiac ultrasound (eFCU) with spectral Doppler to inform decision making in established patients with specific, selected congenital cardiac defects in outreach clinics. Secondary objectives include determining if the addition of eFCU expands capacity in paediatric cardiology outreach clinics and if it improves the patient experience.Methods:Patients aged 2 months to 19 years old with a diagnosis of ventricular septal defect, atrial septal defect, atrioventricular septal defect, patent ductus arteriosus, aortic valve stenosis, or pulmonary valve stenosis with the need for follow-up echocardiography in an outreach clinic from August 2017 to June 2018 were studied. A novel assessment tool was used to determine the success of eFCU.Results:Forty-two patients from 11 clinics underwent eFCU with one unsuccessful exam making the failure rate 2.3% (95% CI 0.0006–0.1256). Addition of eFCU led to a significant increase in volume of patients able to be seen 19 versus 15.5 (p < 0.01). A majority of parents/patients reported a positive experience with eFCU.Conclusion:Expert focused cardiac ultrasound with spectral Doppler can be used successfully for follow-up in patients with select CHD and the addition of eFCU permits increased patient capacity in outreach clinics and has the potential to improve the patient experience.

To test the hypothesis that right ventricular (RV) function has age-specific patterns of development, we tracked the evolution of RV strain mechanics by 2D-speckle tracking echocardiography (2DSTE) in healthy subjects from mid-gestation through one year of age.We conducted a prospective longitudinal echocardiography study in 50 healthy subjects at five time periods across gestation (16-20 weeks, 21-25 weeks, 26-30 weeks, 31-35 weeks, and 36-40 weeks) and four time periods following delivery (1 week, 1 month, 6 months, and 1 year of age). We characterized RV function by measuring RV global and free wall longitudinal strain and systolic strain rate, and segmental longitudinal strain at the apical-, mid-, and basal- ventricular levels of the free wall. Possible associations of gestational age, postnatal age, estimated fetal weight, body surface area, gender, and heart rate on strain were investigated.The magnitudes of RV global and free wall longitudinal strain and global strain rate were decreased throughout gestation (p 0.05 for all). Following birth, the magnitudes of all measures increased from one week through one year (p 0.001 for all). RV segmental longitudinal strain maintained a base-to-apex gradient (highest-to-lowest) from mid-gestation through one year (p 0.001). There was no significant difference in strain patterns based on gender or hear rate.The maturational patterns of RV strain are gestational- and postnatal age- specific. With accepted physiological maturation patterns in healthy subjects, these myocardial deformation parameters can provide a valid basis that allows comparison between health and disease.

Background Patients with adult congenital heart disease (ACHD) experience long waitlist times for heart transplantation (HTx) while a large proportion of donor hearts are refused. The goal of this study was to inform optimal donor selection for patients with ACHD listed for HTx by examining the impact of donor characteristics on post‐HTx outcomes. Methods and Results Using the Scientific Registry of Transplant Recipients, we conducted a retrospective analysis of patients aged ≥18 years listed for HTx in the United States between 2000 and 2016. We compared waitlist times between patients with ACHD and patients with noncongenital heart disease and constructed multivariate hazard models to identify donor characteristics associated with increased waitlist time. We then compared post‐HTx survival between patients with ACHD and patients with noncongenital heart disease and constructed multivariate hazard models to identify donor characteristics associated with mortality. There were very few differences in donor characteristics between HTx recipients with ACHD and those with noncongenital heart disease. Status 1A–listed patients with ACHD experienced longer waitlist times compared with patients with noncongenital heart disease. Increased wait times were associated with some donor characteristics. Post‐HTx outcomes varied over time, with patients with ACHD having inferior early mortality (0 to 30 days), similar intermediate mortality (31 days to 4 years), and superior late mortality (>4 years). We identified no donor characteristics associated with mortality to justify the observed differences in donor selection or waitlist time. Conclusions HTx candidates with ACHD wait longer for transplant but do not require unique donor selection criteria. HTx teams should consider liberalizing donor criteria and focusing only on evidence‐based selection to improve waitlist outcomes and reduce the recipient–donor disparity.

Highlights • Patterns of energy loss in the LV differ among subjects with HCM, DCM, and normal controls. • Our study demonstrated significant elevation in diastolic WSS and indexed systolic EL in HCM. • Few differences were observed in energetic parameters in DCM. • Ventricular energetics may represent novel indices of ventricular diastolic dysfunction., Background Vector flow mapping is an emerging echocardiographic method allowing for investigation of intracardiac blood flow mechanics, wall shear stress (WSS), and energy loss (EL). We hypothesized that alterations in EL and WSS will differ among subjects with hypertrophic (HCM), dilated (DCM) cardiomyopathy, and normal controls. Methods Echocardiograms were prospectively performed with the ProSound F75CV (Hitachi HealthCare., Tokyo, Japan) on all subjects. 2D color Doppler cine loop images were obtained from apical 5 and the apical long axis views and stored digitally. Measurements were averaged over three cardiac cycles using VFM software to derive flow patterns, WSS, and EL. Standard left ventricular (LV) systolic and diastolic functional parameters were also obtained. Results A total of 85 subjects, 22 with HCM (age 18 ± 9 yrs.), 18 DCM (age 18 ± 9 yrs.), and 45 age and gender matched controls were included in the study. Diastolic wall shear stress was found significantly different in HCM (0.004 ± 0.185 N/m2) compared with DCM (0.397 ± 0.301 N/m2, P

Patients with the Turner syndrome (TS) often have longer QT intervals compared with age-matched peers although the significance of this remains unknown. We sought to determine the degree, frequency and impact of QTc prolongation in patients with TS. A chart review of all patients with an electrocardiogram (ECG) and genetically proven TS was performed. Medications at the time of the ECG were reviewed and QTc calculated. Medications were classified according to QTc risk using www.crediblemeds.com. ECG parameters were compared with an age, gender, and cardiac lesion-matched control group. Over the 10-year period of review, 112 TS patients with a mean age of 34 ± 25 years underwent 226 ECGs. At least 1 QTc prolonging medication was prescribed in 81 (74%) patients. Longer QTc interval correlated with absence of y chromosomal material (p = 0.01), older age (p0.0001), increased number of QTc prolonging and nonprolonging medications (p0.0001 each). During the 7.0 ± 5.1 years of follow-up, no patient had ventricular arrhythmia or unexplained sudden death. QTc was significantly shorter in matched controls using either Bazett or Hodges formula (424 ± 16 ms vs 448 ± 28 ms, p0.0001; and 414.8 ± 16 ms vs 424.2 ± 20 ms; p = 0.0002, respectively). However, there was no difference in the frequency of QTc prolongation460 msec (2.8% vs 2.6%, p = 0.9). In conclusion, despite frequent use of QT-prolonging medications, ventricular arrhythmias are rare in TS.

BACKGROUND Changes in 2D echocardiography (2DE) speckle tracking imaging (STI) derived left ventricular (LV) strain (S) and strain rate (SR) precedes diminution of LV ejection fraction (LVEF) in adult valvular aortic stenosis (AS). We prospectively examined whether 2DE-STI derived multidirectional LV S and SR correlate with AS severity in children using LV mass index (MI) as the principal outcome variable. METHODS 52 children (10.4 ± 7.3 years) with isolated congenital AS were included; 13 mild (2.5 m/s 4.0 m/s). 2DE including Doppler and STI longitudinal strain (LS), strain rate (LSR), circumferential strain (CS), and strain rate (CSR) were measured. Univariate and multivariable linear regressions identified correlations between LVMI and strain indices. RESULTS Three clinical and 2DE variables, and four strain indices were independently associated with LVMI. LVMI correlated positively with systolic blood pressure and aortic regurgitation, and negatively with LVEF. LVMI correlated positively with LSR (four-chamber) and CSR (basal), and negatively with segmental CS in the inferior (basal) and anteroseptal (distal) segments. LVMI showed significant inverse association with LS (P = .05), LSR (P

The Fontan circulation, accomplished by direct surgical connection of the vena cavae to the pulmonary arteries, can be an effective palliation for patients with a single ventricle. However, failure of the Fontan circulation can result from mechanical obstruction, cardiac arrhythmias, increasing pulmonary vascular resistance, or deteriorating ventricular performance. Although systolic ventricular failure can usually be identified by a combination of clinical signs, symptoms, and imaging findings, diastolic ventricular dysfunction is likely an underrecognized cause of Fontan failure. Methods for detection of impaired diastolic function in a single ventricle are evolving, and established techniques appropriate in the biventricular heart lack validation in single ventricle. Association of biomarkers, cardiac magnetic resonance, and echocardiographic findings with invasively acquired pressure-volume loop data in humans may offer a way forward to accurate, noninvasive diagnosis. Today, therapy for severe diastolic ventricular dysfunction in the Fontan circulation is often disappointing and may require consideration of a ventricular assist device or even cardiac transplant. Progress toward improved outcomes of the Fontan palliation likely depends on successful innovation in primary prevention strategies and the development of more effective pharmacotherapy.

This study evaluated hepatic stiffness by shear wave elastography to investigate subclinical hepatic changes in a cohort of patients with congenital biventricular heart disease (BHD).The BHD patients and age-matched healthy controls were prospectively recruited for hepatic ultrasonography and shear wave elastography. Real-time B-mode imaging with Doppler was performed for celiac axis, superior mesenteric artery, and main portal vein, and hepatic shear wave elastography was assessed. Vascular Doppler indices included peak velocities; velocity time integral, resistive, pulsatility, and acceleration indices; and portal vein volumetric flow. One-way analysis of variance was used for comparisons between controls, BHD, and a cohort of Glenn and Fontan patients.In all, 66 subjects were included. Thirty-six subjects were in the BHD group (male, 25; female, 11; mean age 27.4 ± 4.6 years; mean weight 76.8 ± 18.5 kg), and 30 were healthy controls (male, 11; female, 23, mean age 27.4 ± 3.8 years; mean weight 70 ± 17.2 kg). Shear wave elastography was increased in BHD (8.11 ± 2.07 kPa) compared with controls (5.44 ± 1.18 kPa; P.001). Hepatic stiffness in BHD was significantly different from that in the Fontan cohort but not in the infant Glenn cohort.Increased hepatic stiffness was observed in young adults with repaired BHD. Although cause is not established, possibilities include hepatic congestion early in life or elevated central venous pressures due to right heart burden. Further research is required to determine whether these patients will ultimately have clinically relevant liver disease.

Congenitally corrected transposition of the great arteries (ccTGA) can occur in isolation, or in combination with other structural cardiac anomalies, most commonly ventricular septal defect, pulmonary stenosis and tricuspid valve disease. Clinical recognition can be challenging, so echocardiography is often the means by which definitive diagnosis is made. The tricuspid valve and right ventricle are on the systemic arterial side of the ccTGA circulation, and are therefore subject to progressive functional deterioration. The natural history of ccTGA is also greatly influenced by the nature and severity of accompanying lesions, some of which require surgical repair. Some management strategies leave the right ventricle as the systemic arterial pump, but carry the risk of worsening heart failure. More complex ‘double switch’ repairs establish the left ventricle as the systemic pump, and include an atrial baffle to redirect venous return in combination with either arterial switch or Rastelli operation (if a suitable ventricular septal defect permits). Occasionally, the anatomic peculiarities of ccTGA do not allow straightforward biventricular repair, and Fontan palliation is a reasonable option. Regardless of the approach selected, late cardiovascular complications are relatively common, so ongoing outpatient surveillance should be established in an age-appropriate facility with expertise in congenital heart disease care.

Background Congenital heart disease (CHD) is the most common type of major congenital malformations. CHD outcomes have improved in the past 20 years, and research efforts in the field have increased exponentially. The purpose of this investigation is to identify the most influential recent publications related to CHD, and reveal their shared features using bibliometric analysis. Methods Journal articles related to CHD published between 2000 and 2020 were retrieved from the Scopus database. In order of citation frequency, the 100 most-cited clinical original articles overall, the 20 most-cited clinical original articles of the years 2018–2020, and the top 10 most-cited review articles were compiled. The lists were analyzed to identify characteristics of highly cited publications. VOS Viewer was used to construct and visualize network maps based on co-authorship and co-occurring keywords. Results The total number of citations varied from 244 to 1741, and the number of citations per year varied from 13.2 to 121.9. Articles were most commonly published in Circulation, Journal of the American College of Cardiology, or Journal of Thoracic and Cardiovascular Surgery. Most originated from the USA, and had a sample size of 200 patients or less. A total of 46 authors had at least 3 publications among the list and were organized in 6 collaboration clusters. Tetralogy of Fallot, atrial septal defect, the perinatal period, Fontan, and cardiac surgery were identified as major areas of research. Conclusions This bibliographic analysis mapped two decades of research in CHD, highlighting important articles and defining major areas of research.

Inertial cavitation inducing ultrasound-mediated microbubble treatments can produce resolution of vasospasm and restoration of distal arterial flow after peripheral artery injury. Resolution of catheter-induced vasospasm is likely to be nitric oxide- mediated because improvements in stenosis diameter and downstream blood flow were blunted following pretreatment with L-NAME. The potential for clinical applicability of this therapy is significant because: 1) microbubbles can be delivered systemically into the site of injury enabling relatively high local concentration; 2) targeted transcutaneous ultrasound delivery is achievable due to the proximity of vessels; and 3) microbubbles and diagnostic ultrasound system used are commercially available.

Background We hypothesized that right atrial (RA) performance is abnormal in repaired tetralogy of Fallot (TOF). Methods TOF patients were prospectively enrolled for cardiovascular magnetic resonance (CMR), echocardiography and exercise stress following a standardized 14-center protocol. Peak RA longitudinal strain (RALS) and right ventricular longitudinal strain (RVLS) were measured using CMR feature tracking (FT) and correlated to RA and RV end diastolic volumes (EDVi) and ejection fraction (EF). Results The cohort had 311 subjects: 171 TOF (94 male, age 18.2±8years) and 140 healthy controls (69 male, 16.4±11years). RAEDVi, RALS, RVEDVi, RVLS, RAEF, and RVEF in TOF were 60.8±17.1ml/m 2 , 13.6±5.7%, 120.3±30.3ml/m 2 , 12.3±4.2%, 32.5±9.9% and 51.2±8.4% and differed from respective indices in controls: 51.7±15.7ml/m 2 , 27±10.1%, 74±19.0ml/m 2 , 18.5±5.3%, 54±8% and 62.5±5.5% (p Conclusion RALS, RAEDVi and RAEF are abnormal in TOF. Reduced RALS indicates decreased RA reservoir function. Because they correlate with other functional RV indices, these abnormalities may represent RA diastolic burden from chronic RV dysfunction in TOF. The young cohort age might explain the absence of RALS correlation to tricuspid regurgitation and peak oxygen uptake.

Children with congenital heart disease may require long-term central venous access for intensive care management; however, central venous access must also be preserved for future surgical and catheterization procedures. Transhepatic venous catheters may be an useful alternative. The objective of this study was to compare transhepatic venous catheters with traditional central venous catheters regarding complication rate and duration of catheter service.Retrospective review of 12 congenital heart disease patients from September 2013 to July 2015 who underwent placement of one or more transhepatic venous catheters.Single freestanding pediatric hospital located in the central United States.Pediatric patients with congenital heart disease who underwent placement of transhepatic venous catheter.Cohort's central venous catheter complication rates and duration of catheter service were compared with transhepatic venous catheter data.Twelve patients had a total of 19 transhepatic venous lines. Transhepatic venous lines had a significantly longer duration of service than central venous lines (p = 0.001). No difference between the two groups was found in the number of documented thrombi, thrombolytic burden, or catheter sites requiring wound care consultation. A higher frequency of infection in transhepatic venous lines versus central venous lines was found, isolated to four transhepatic venous lines that had a total of nine infections. All but one was successfully managed without catheter removal. The difference in the proportion of infections to catheters in transhepatic venous lines versus central venous lines was significant (p = 0.0001), but no difference in the rate of infection-related catheter removal was found.Without compromising future central venous access sites, transhepatic venous lines had superior duration of service without increased thrombosis, thrombolytic use, or insertion site complications relative to central venous lines. Transhepatic venous catheters had a higher infection rate, and further investigation into the etiology is warranted.

Left ventricular two-dimensional global longitudinal strain (LS) is superior to ejection fraction (EF) as predictor of outcome. We provide reference data for atrial and ventricular global LS during childhood and adolescence by CMR feature tracking (FT). We prospectively enrolled 115 healthy subjects (56 male, mean age 12.4 ± 4.1 years) at a single institution. CMR consisted of standard two-dimensional steady-state free-precession acquisitions. CMR-FT was performed on ventricular horizontal long-axis images for derivation of right and left atrial (RA, LA) and right and left ventricular (RV, LV) peak global LS. End-diastolic volumes (EDVs) and EF were measured. Correlations were explored for LS with age, EDV and EF of each chamber. Mean±SD of LS (%) for RA, RV, LA and LV were 26.56±10.2, -17.96±5.4, 26.45±10.6 and -17.47±5, respectively. There was a positive correlation of LS in LA, LV, RA and RV with corresponding EF (all P

OBJECTIVE Experimental evidence suggests that changes in the fetal myocardium result from intrauterine effects of maternal diabetes mellitus and obesity. The aim of this study was to assess fetal cardiac function using two-dimensional speckle-tracking echocardiography to determine the effects of maternal diabetes and obesity on the fetal myocardium. METHODS Comparative cross-sectional evaluation of myocardial function in fetuses of mothers with diabetes mellitus (FDM) or obesity (FO) and normal gestational age-matched control fetuses (FC) was performed using two-dimensional speckle-tracking echocardiography at two centers. RESULTS In total, 178 fetuses (82 FDM, 26 FO and 70 FC) met the enrolment criteria. Mean gestational age at assessment was similar among groups: 25.3 ± 5.1 weeks for FDM, 25.0 ± 4.6 weeks for FO and 25.1 ± 4.9 weeks for FC. Mean maternal body mass index was significantly higher in FDM and FO groups compared with the FC group. Statistically significant differences in fetal cardiac function were detected between FDM and FC for global longitudinal strain (mean ± SD, -21.4 ± 6.5% vs -27.0 ± 5.2%; P

Echocardiographic assessment of right atrial (RA) volume, inferior vena cava (IVC) diameter, and hepatic vein flow velocity independently correlate with the RA pressure by direct catheter measurement in adults. We prospectively collected invasive RA pressure measurements and echocardiographic data in infants and young children with the goal of developing a predictive model to noninvasively determine normal RA pressure. All subjects had a central venous catheter through which RA pressure could be transduced. Specific inclusion criteria consisted of (1) biventricular heart, (2) absence of inotropes, (3) sinus rhythm, and (4) at least 24 hours from surgery. Two-dimensional echocardiography (2DE)-Doppler and 3DE-Doppler were used to measure RA volume, systemic venous diameters, and flow velocity. Regression equations of RA pressure with RA volume, systemic venous size, and flow velocity were explored. Of 46 studies, 43 (93%) had echocardiograms adequate for analysis. RA pressure did not correlate with body surface area or age (p = 0.69, p = 0.87). The mean indexed RA volume by 3DE-Doppler was significantly higher than by 2DE (p 2 ). RA pressure did not show correlation with systemic venous diameters or systolic and diastolic flow velocities in the SVC and hepatic veins. In conclusion, regression incorporating 2DE-derived RA volume and IVC systolic flow velocity provided the best noninvasive estimate of normal RA pressure in infants and children. The model derived requires validation in an independent sample.

Background and objectives Our purpose was to investigate cardiovascular abnormalities in children with osteogenesis imperfecta (OI). Methods Two hundred children (100 OI, 100 matched volunteers) were prospectively studied. Aortic and left ventricular (LV) measurements were performed using transthoracic echocardiography. Patients were typed according to modified phenotypical Sillence classification as published in the Nosology and Classification of Genetic Skeletal disorders: 2015 Revision. Results Patients (age 9.6±4.1 years, body surface area 1.08±0.47 m2) consisted of OI types: 1 (n=44), 3/4 (n=54), 4 (n=1) and 15 (n=1). The 95% CIs for Z­score of aortic annulus, sinus, sinotubular junction and ascending aorta for OI were 0.43 to 0.73, 0.56 to 0.94, 0.28 to 0.70 and 0.78 to 1.24, respectively. In type 1, sinus, sinotubular junction and ascending aorta diameters were 2.29 cm, 1.81 cm and 2.05 cm, respectively, which did not differ compared with controls. The LV dimensions were larger in type 1. In type 3/4, aortic dimensions were larger than controls at all levels: annulus (1.61 vs 1.50 cm, p Conclusions Cardiovascular effects are identifiable in childhood even in mild forms of OI. Aortic dilation was the predominant finding, while valvular abnormalities were infrequent. Patients with more severe skeletal pathology (types 3/4) have more significant findings. Aortic and LV dilation in type 1 vs type 3/4 appears to differ based on the biochemical mechanism of disease.

Purpose We used two-dimensional echocardiographic speckle-tracking to investigate whether left and right atrial (LA and RA) phasic function in pediatric heart transplantation (HT) patients is altered and explored the relationship to HT-related clinical variables. Methods Eighty-six subjects (36 HT and 50 normal children) were prospectively enrolled in two centers. Clinical data included age at HT, bypass time, ischemia time, donor age, and incidence of rejection. Atrial deformation indices including strain and strain rates (SRs) were measured using two-dimensional echocardiographic speckle-tracking. Components of phasic atrial function—reservoir (er, SRr), conduit (ecd, SRcd), and booster (ect, SRct) were calculated. Comparisons with controls were made using t test or Kruskal-Wallis test, and correlations to clinical variables were explored. Results The mean age and body surface area of HT subjects were 10.2 ± 6.2 years and 1.2 ± 0.6 m2, respectively. The mean heart rates were higher in HT (96 ± 18 vs 88 ± 21 in controls). There were reductions in RA and LA reservoir (er, SRr), conduit (ecd, SRcd), and booster (ect, SRct) function in HT compared with controls. There was no relationship of LA and RA deformation indices with mean age at HT, bypass time, or ischemia time. The LA ecd correlated weakly with donor age (r = –0.49, P = .04) and RA SRr, and SRcd showed association with duration of HT (P Conclusions Atrial strain determination is feasible in pediatric HT recipients and demonstrates disruption of reservoir, conduit, and booster function of both atria in this population; we speculate this may be a consequence of ventricular diastolic dysfunction.

Quantification of pulmonary regurgitation (PR), pulmonary flow distribution, and ventricular function is important for clinical surveillance in repaired Tetralogy of Fallot (TOF). Cardiovascular magnetic resonance (CMR) is the established reference, but cost, test duration, and patient discomfort are potential limitations to its serial use. We investigated whether an Abbreviated CMR protocol would alter clinical decisions in TOF from those that would have been made using a full protocol. Patients > 7 years with repaired TOF were identified. CMR was performed according to standard complete imaging protocol. CMRs were prepared in two ways, Full and Abbreviated and submitted for review by two imaging specialists. In conjunction with clinical information and case-specific quantitative CMR data (PR fraction, ventricular volumes, ejection fraction, branch pulmonary artery flow), Full and Abbreviated image sets were anonymized and uploaded for review. For the first half, Imager 1 received Abbreviated, and Imager 2 Full and for the remaining, Imager 1 received Full and Imager 2 received Abbreviated. Blinded to the other’s choices, Imagers provided clinical decisions. Inter-rater agreement for each decision was measured. In all, 124 studies from 80 patients (mean 17.8 years) were analyzed. For ‘intervention versus no-intervention’ decision, the inter-rater agreement was strong [κ 0.75, p

To the Editor We thank the correspondents for their insightful comments1 regarding the benefits of pharmacotherapy in selected patients with systemic right ventricles. The underlying principles of pharmacologic management of the systemic right ventricle are necessarily based on less than robust clinical experience, but are probably similar to those guiding management of congestive left ventricular failure. Therefore, we also use ACE inhibitors for …

The Fontan operation for single ventricle palliation consists of the creation of a complete cavopulmonary connection, usually by incorporating inferior vena caval flow into a pulmonary arterial circulation already receiving flow from the superior vena cava. In single ventricle palliated in this way, the anatomy is complex, and the pathophysiological complications are frequent; so, cardiac imaging is a key aspect of clinical surveillance. Common problems that echocardiography and MRI may disclose and characterise in the Fontan palliation of single ventricle include obstruction of systemic venous and pulmonary arterial flow, atrioventricular and semilunar valve dysfunction, unintended collateral flow patterns, ventricular dysfunction, aortic arch obstruction, interatrial obstruction, fenestration flow and patch leaks. Despite the broad scope of these modalities for detection of such problems, often no single imaging method is comprehensive in any given patient. Therefore, physicians must recognise the limitations of each modality, and circumvent these by application of suitable alternatives. New imaging tools are becoming available, which may ultimately prove to be of value in the Fontan circulation. Proper application of diverse new technologies such as four dimensional flow, computational fluid dynamics and three-dimensional printing will require critical evaluation in the single ventricle population.

Background Predischarge (pre-d/c) transthoracic echocardiography (TTE) is routine after surgery for congenital heart disease, but how it affects clinical care is unknown. The aim of this study was to test the hypothesis that pre-d/c TTE frequently reveals findings associated with short-term clinical course through a systematic review of findings on pre-d/c TTE and clinical events that followed. Methods Clinical outcomes of mortality, hospitalization, catheterization, and surgery at 1 year were examined for pediatric patients undergoing pre-d/c TTE between June 2010 and June 2012. Using logistic regression, a multivariate model was generated associating clinical, pre-d/c transthoracic echocardiographic, and demographic variables with unplanned postdischarge cardiac events (UCEs) within 1 year. Results Of 462 patients who underwent pre-d/c TTE, there were 265 male patients (57%) and 197 female patients (43%); the median age was 0.8 years (range, 0–33 years). Two hundred thirty–seven patients (51%) had findings (valve regurgitation, hemodynamic obstruction, ventricular dysfunction, unintended shunt, or pericardial effusion) on pre-d/c TTE, 57 of which were of more than mild severity. Agreement between pre-d/c TTE and postoperative transesophageal echocardiographic findings was only fair to moderate (κ = 0.27–0.43). Sixty-four patients (14%) had UCEs. Univariate analysis revealed that UCE were more frequent in patients with diagnoses and surgical procedures of high complexity. After accounting for these confounding nonechocardiographic variables, pre-d/c transthoracic echocardiographic findings, specifically valve regurgitation of more than mild severity, and ventricular dysfunction and obstructions of any severity were independently associated with UCEs (odds ratios, 1.90, 1.99, and 1.85, respectively). Conclusions Findings on pre-d/c TTE are frequent, commonly discordant with postoperative transesophageal echocardiographic results, and associated with adverse clinical events after surgery for congenital heart disease. These data would strongly support the practice of pre-d/c TTE after surgery for congenital heart disease.

Patients with atrioventricular nodal reentry tachycardia (AVNRT) often are managed successfully by ablation of the slow pathway with success rates reported as high as 99%. Low voltage bridges (LVBs) have been demonstrated to be helpful in guiding AVNRT ablation. Patients may present to the electrophysiology lab without evidence of inducible arrhythmia. In these scenarios, the demonstration of LVBs may be diagnostic and guide catheter ablation treatment. The purpose of our study was to prospectively investigate the specificity of LVBs as a diagnostic marker of AVNRT. Patients aged

The functional significance of pulmonary arterial end-diastolic forward flow (EDFF) in patients with repaired tetralogy of Fallot (rTOF) is not fully understood, with conflicting reports regarding its associations with pulmonary regurgitation (PR), right ventricular (RV) size and function, and so-called restrictive RV physiology. To examine these associations, we retrospectively analyzed 399 patients with rTOF who had contemporaneous echocardiography (Echo) and cardiovascular magnetic resonance (CMR) studies. The median age at TOF repair was 0.7 years (0.21, 2.66), age at CMR was 19.8 years (13.0, 29.4), and interval between Echo and CMR was 48 days (0, 182). Doppler identified EDFF in 122 (31%) patients and CMR in 113 patients (28%). Compared with those without EDFF, patients with EDFF were younger, had greater PR, and higher RV end-diastolic volume, stroke volume, and ejection fraction. Markers of RV restriction such as right atrial size did not differ between groups. On multivariable regression, EDFF was associated with higher RV stroke volume and lower left ventricular end-diastolic volume. The association between Echo and CMR measurements of EDFF was modest (area under the receiver operating characteristic curve = 0.684, r = 0.374, p 0.001). In conclusion, EDFF was common in this large cohort of patients with rTOF, but its presence and extent varied between Echo and CMR. EDFF was associated with greater PR and larger RV size, but not with markers of poor RV compliance such as right atrial enlargement. Mechanisms beyond RV noncompliance may contribute to the presence of EDFF.

Background Compromise of right heart function is an important feature of many forms of congenital heart disease, and right atrial (RA) pressure is clinically relevant. Inferior vena cava (IVC) diameter and inspiratory collapse are indices of RA pressure, but pediatric data are lacking. Methods RA measurements, systemic venous diameters, and Doppler filling fractions were prospectively investigated in healthy volunteer children and adolescents. The IVC was measured in its long axis just above the junction with the hepatic veins in the subxiphoid view and the superior vena cava at its junction with the right atrium in the right parasternal view. The changes in IVC diameter (IVCD) during quiet respiration and with a sniff were recorded. Hepatic venous systolic filling fraction was calculated from Doppler velocities in the first hepatic vein. RA major-axis length, area, and volume were measured from the apical four-chamber view. Three measurements of each parameter were averaged over at least three respiratory cycles. The IVC collapsibility index (IVCCI) was calculated as [(IVCDmax − IVCDmin)/IVCDmax] × 100. Substituting IVCDsniff for IVCDmin in the formula, the IVCCIsniff was calculated. Results Of 132 subjects enrolled, data in 120 (mean age, 8.3 ± 4.5 years) were analyzed. The maximal (expiratory) and minimal (inspiratory) diameters during free breathing were 12.1 ± 3.8 and 8.9 ± 3.8 mm for the IVC and 11.9 ± 3.4 and 7.9 ± 2.6 mm for the superior vena cava. IVCCImin and IVCCIsniff were 30 ± 13 and 47 ± 18, respectively. The RA major-axis length, area, and indexed maximal volume were 3.7 ± 0.7 cm, 10.3 ± 3.6 cm 2 , and 22.3 ± 7.0 mL/m 2 , respectively. Correlations of maximal superior vena cava and IVC dimensions with body surface area were slightly better than with age and much stronger than with RA volume. No significant correlation was found between IVCCIs and age, gender, or indexed RA volume. Conclusions Measurement of systemic venous diameters, collapsibility indices, and RA volumes is feasible in healthy children and adolescents. Venous diameters increase predictably with growth and so must be interpreted in light of body surface area. IVCCIs and hepatic venous filling fraction compare closely with those reported in adults. Pediatric nomograms for these parameters are provided, and they should next be evaluated for relation to directly measured RA pressure in this age group.

To test the hypothesis that increased venous compliance manifested as inferior vena cava (IVC) dilation is an important substrate for syncope in children.IVC diameters were measured in 191 children and adolescents with syncope and in 95 controls. Subjects were divided based on age12 years (younger group) and ≥12 years (older group). IVC measurements at the right atrial junction (IVC-RA), 10 mm below the IVC-RA junction (IVC-RA10), and at the point of maximal diameter (IVCmax) were made. The linear relation to body surface area (BSA) was confirmed, as were dimensions indexed to BSA (iIVC). Relationships between iIVC and the time of day were evaluated.In the syncope group, the mean age was 12.9 ± 3.6 years, mean weight was 54.7 ± 23 kg, and mean BSA was 1.5 ± 0.4 mThe IVC is enlarged in teenagers with syncope compared with controls, suggesting that venous capacitance and resultant pooling play roles in the pathogenesis of syncope. In contrast, younger children with syncope do not demonstrate IVC dilation, suggesting that their syncope arises from a different mechanism.

Patients with d-transposition and atrial switch repair are at risk for adverse outcome (ADV) due to decline in systemic right ventricular function. Exercise testing in adults with a systemic left ventricle and heart failure have identified prognostic values for ADV including oxygen consumption (VO2

Hepatic dysfunction is a recognized complication after Fontan palliation of congenital heart disease. We sought to quantitatively measure hepatic stiffness and vascular Doppler indices using ultrasound (US) and shear wave elastography (SWE) in a Fontan cohort. Subjects were prospectively recruited for echocardiography and real-time hepatic duplex US with SWE for hepatic stiffness (kPa). Doppler peak velocities, velocity time integral, resistive, pulsatility, acceleration indices (RI, PI, AI), and flow volume were measured in celiac artery, superior mesenteric artery, and main portal vein (MPV). A subset underwent cardiac catheterizations with liver biopsy. Correlations were explored between SWE, duplex, hemodynamic, and histopathologic data. In all, 106 subjects were studied including 41 patients with Fontan physiology (age 13.8 ± 6 years, weight 45.4 ± 23 kg) and 65 controls (age 15.0 ± 8.4 years, weight 47.9 ± 22 kg). Patients with Fontan physiology had significantly higher hepatic stiffness (15.6 versus 5.5 kPa, P

The admittance and Wei's equation is a new technique for ventricular volumetry to determine pressure-volume relations that addresses traditional conductance-related issues of parallel conductance and field correction factor. These issues with conductance have prevented researchers from obtaining real-time absolute ventricular volumes. Moreover, the time-consuming steps involved in processing conductance catheter data warrant the need for a better catheter-based technique for ventricular volumetry. We aimed to compare the accuracy of left ventricular (LV) volumetry between the new admittance catheterization technique and transoesophageal real-time three-dimensional echocardiography (RT3DE) in a large-animal model. Eight anaesthetized pigs were used. A 7 French admittance catheter was positioned in the LV via the right carotid artery. The catheter was connected to an admittance control unit (ADVantage; Transonic Scisense Inc.), and data were recorded on a four-channel acquisition system (FA404; iWorx Systems). Admittance catheterization data and transoesophageal RT3DE (X7-2; Philips) data were simultaneously obtained with the animal ventilated, under neuromuscular blockade and monitored in baseline conditions and during dobutamine infusion. Left ventricular volumes measured from admittance catheterization (Labscribe; iWorx Systems) and RT3DE (Qlab; Philips) were compared. In a subset of four animals, admittance volumes were compared with those obtained from traditional conductance catheterization (MPVS Ultra; Millar Instruments). Of 37 sets of measurements compared, admittance- and RT3DE-derived LV volumes and ejection fractions at baseline and in the presence of dobutamine exhibited general agreement, with mean percentage intermethod differences of 10% for end-diastolic volumes, 14% for end-systolic volumes and 9% for ejection fraction; the respective intermethod differences between admittance and conductance in eight data sets compared were 11, 11 and 12%. Admittance volumes were generally higher than those obtained by RT3DE, especially among the larger ventricles. It is concluded that it is feasible to derive pressure-volume relations using admittance catheterization in large animals. This study demonstrated agreements between admittance and RT3DE to within 10-14% mean intermethod difference in the estimation of LV volumes. Further investigation will be required to examine the accuracy of volumes in largest ventricles, where intermethod divergence is greatest.

We sought to determine whether global and regional left ventricular (LV) strain parameters were altered in repaired coarctation of the aorta (COA) with normal LV ejection fraction (EF) when compared with healthy adult controls, and whether such alterations were related to LV hypertrophy (LVH). We identified 81 patients after COA repair (31 female, age 25 ± 8.5 years) with inclusion criteria at follow-up CMR of: age ≥13 years, time post-repair ≥10 years, no aortic valve disease, LV-EF >50 %). LV deformation indices derived using CMR-feature tracking and volumetric EF were compared between COA patients and normal controls (n = 20, 10 female, age 37 ± 7 years), and between COA with versus without LVH. In repaired COA versus controls, LV-EF (%) was 62 ± 7.2 versus 58 ± 3.0 (p = 0.01), and LV mass (g/m2) 66 ± 16.8 versus 57.7 ± 6.0 (p = 0.0001). LV global longitudinal strain (GLS) was decreased to −17.0 ± 4.7 % in COA (−20 ± 5 % in controls, p = 0.02), and global radial strain (GRS) reduced to 40 ± 15 % (50 ± 12.4 % in controls, p = 0.003). The global circumferential strain (GCS) was preserved in COA at −23 ± 4.7 % (−24.6 ± 2.4 % in controls, p = 0.14). Regionally, LS decrease was marked in the basal segments (septal, p = 0.005, lateral, p = 0.013). In COA with LVH (n = 45, mass 76.3 ± 12.8 g/m2) versus without LVH (n = 36, mass 52.2 ± 10 g/m2), GLS was more markedly decreased (−15.7 ± 4.8 vs. −18.5 ± 4.2 %, p = 0.016, but GRS and GCS were similar (p = 0.49 and 0.27). In post-repair COA with normal LV-EF, GLS and GRS are reduced whilst GCS is preserved. GLS reduction is more pronounced in the presence of LVH. GLS may qualify as indicator of early LV dysfunction.

Truly long-term follow-up data after transcatheter closure (TC) of atrial septal defects (ASDs) are scarce. We report the 5- to 20-year outcomes of TC and surgical closure (SC) for typical secundum ASD. We reviewed the records of patients with isolated secundum ASD and right ventricular volume overload who underwent TC or SC (January 1, 1986 to September 30, 2005). Follow-up was obtained through a combination of chart review, physician records, and telephone survey. We identified 375 patients (207 SC and 168 TC) and obtained follow-up data >5 years (median follow-up 10 years) for 300 (152 SC, 148 TC). Nine patients have died (3%). The New York Heart Association functional class was unchanged in 227 patients, improved in 25, and was worse in 15. Clinically significant arrhythmia was found in 28 patients (9.3%); 21% aged >40 years developed arrhythmia. On multivariate analysis, the odds of significant arrhythmia tended to be greater in the SC group, but this was statistically insignificant (95% confidence interval 0.68 to 3.9, p = 0.27). Age and preprocedure arrhythmia, but not TC or SC, were independent risk factors for late arrhythmia (p

Tricuspid regurgitation is an important risk factor for morbidity and mortality in congenital heart disease. We prospectively used three-dimensional echocardiography (3DE) to characterize the normal tricuspid valve (TV) in children focusing on geometry, structure and function of the annulus and

Surgical (S) and catheter mediated device (C) treatment of atrial septal defect (ASD) are both effective means of repair. Comparison of resource utilization depending on approach is therefore an area of considerable interest. We sought to compare the frequency and timing of post-procedural

Background: The purpose of this investigation was to test the hypothesis that flow patterns in the right ventricle are abnormal in patients with repaired tetralogy of Fallot (TOF). High-resolution echocardiographic contrast particle imaging velocimetry was used to investigate rotation intensity and kinetic energy dissipation of right ventricular (RV) flow in patients with TOF compared with normal controls. Methods: Forty-one subjects (16 with repaired TOF and varying degrees of RV dilation and 25 normal controls) underwent prospective contrast imaging using the lipid-encapsulated microbubble (Definity) on Sequoia systems. A mechanical index of 0.4, three-beat high–frame rate (>60 Hz) captures, and harmonic frequencies were used. Rotation intensity and kinetic energy dissipation of flow in the right and left ventricles were studied (Hyperflow). Ventricular volumes and ejection fractions in all subjects were derived from same-day cardiac magnetic resonance (CMR). Results: Measurable planar maps were obtained for the left ventricle in 14 patients and the right ventricle in 10 patients among those with TOF and for the left ventricle in 23 controls and the right ventricle in 21 controls. Compared with controls, the TOF group had higher RV indexed end-diastolic volumes (117.8 6 25.5 vs 88 6 15.4 mL/m2 , P < .001) and lower RV ejection fractions (44.6 6 3.6% vs 51.8 6 3.6%, P < .001). Steady-streaming (heartbeat-averaged) flow rotation intensities were higher in patients with TOF for the left ventricle (0.4 6 0.13 vs 0.29 6 0.08, P = .012) and the right ventricle (0.53 6 0.15 vs 0.26 6 0.12, P < .001), whereas kinetic energy dissipation in TOF ventricles was lower (for the left ventricle, 0.51 6 0.29 vs 1.52 6 0.69, P < .001; for the right ventricle, 0.4 6 0.24 vs 1.65 6 0.91, P < .001). Conclusions: It is feasible to characterize RV and left ventricular flow parameters and planar maps in adolescents and adults with repaired TOF using echocardiographic contrast particle imaging velocimetry. Intraventricular flow patterns in the abnormal and/or enlarged right ventricle in patients with TOF differ from those in normal young adults. The rotation intensity and energy dissipation trends in this investigation suggest that they may be quantitative markers of RV and left ventricular compliance abnormalities in patients with repaired TOF. This hypothesis merits further investigation.

Objective Our objective was to characterise the divergence of effort from outcome in congenital heart disease (CHD) care by measuring mortality-related resource utilisation fraction (MRRUF) for various CHD lesions across institutions of differing volumes. Methods Study design was observational analysis of an administrative database, the Pediatric Health Information System (PHIS). The setting was inpatient; 2004–2013. Patients were ≤21 years old with atrial septal defect (ASD), ventricular septal defect (VSD), tetralogy of Fallot (TOF), hypoplastic left heart syndrome (HLHS) or other single ventricle (SV). There were no interventions but diagnosis, institution (and volume), age, length of hospitalisation, billed charges and deaths were recorded. The main outcome measure was MRRUF, the ratio of investments during hospitalisations ending in fatality to investments during all hospitalisations. Results There were 50 939 admissions, 1711 deaths, 703 383 inpatient days, and $10 182 000 000 billed charges. MRRUF varied widely by diagnosis: highest in HLHS (21%), but present in ASD (2%) and VSD (4%). Highest among the very young, MRRUF also increased in HLHS and SV during adolescence. MRRUF increased with hospitalisation duration. MRRUF had no relation to institutional volume, and was static over the decade studied. Conclusions Even in the modern era we invest heavily in inpatient CHD care that does not produce the desired outcome. Although its magnitude varies by lesion and age, MRRUF is not limited to complex disease in the very young. MRRUF is not decreasing, and is not isolated to high or low volume institutions.

Background: Although the results of maternal diabetes on the newborn heart are well described, in-utero cardiovascular effects of maternal diabetes and obesity are not as well understood. Hypothesis: Echocardiographically derived measures of right and left heart flow volumes in fetuses of diabetic mothers (FDM) and fetuses of obese mothers (FOM) differ from those of normal fetuses. Methods: We prospectively enrolled 264 normal fetuses, 30 FDM and 49 FOM for prenatal research echocardiography. Estimated fetal weight (EFW) based norms for aortic and pulmonary valve velocity time integrals (AVVTI, PVVTI), right and left ventricular outflow tract diameters (LVOTD, RVOTD), aortic and pulmonary valve stroke volume (AVSV, PVSV), and cardiac output (LVCO, RVCO) were derived from the 264 control group fetuses using nonlinear regression to a Gompertz growth function. Z values for each parameter were determined, then analyzed using a non-paired two-tailed t-test to compare the three groups. Results: The mean ± SD EFW (g) for normal fetuses, FDM and FOM were 1610 ± 1201, 964 ± 694 and 1075 ± 1013; respective mean gestational ages (weeks) were 28.1±1.3, 25.3± 5, and 25 ± 6.6. The table shows mean Z value comparisons of VTI, OTD, SV and CO for the RV and LV in the three groups. Conclusions: FDM have significantly lower right and left heart stroke volumes and outputs than other fetuses matched for size, while FOM did not have demonstrable right or left heart stroke volume or cardiac output changes relative to normal fetuses. Similar to their relationship with gestational age, normal right and left ventricular stroke volumes and outputs follow a non-linear correlation with estimated fetal weight. Flow volumes in macrosomic fetuses may be better normalized to EFW rather than to gestational age. Reduced placental blood flow and ventricular compliance changes may be contributing to depressed biventricular cardiac output in FDM.

Background Quantifying resource utilization in the inpatient care of congenital heart diease is clinically relevant. Our purpose is to measure the investment of inpatient care resources to achieve survival in hypoplastic left heart syndrome (HLHS), and to determine how much of that investment occurs in hospitalizations that have a fatal outcome, the mortality-related resource utilization fraction (MRRUF). Methods A collaborative administrative database, the Pediatric Health Information System (PHIS) containing data for 43 children’s hospitals, was queried by primary diagnosis for HLHS admissions of patients ≤21 years old during 2004–2013. Institution, patient age, inpatient deaths, billed charges (BC) and length of stay (LOS) were recorded. Results In all, 11,122 HLHS admissions were identified which account for total LOS of 277,027 inpatient-days and $3,928,794,660 in BC. There were 1145 inpatient deaths (10.3 %). LOS was greater among inpatient deaths than among patients discharged alive (median 17 vs. 12, p