Prevalence of hepatic steatosis and fibrosis in Turner syndrome: A prospective case-control study.

BACKGROUND AND AIMS: Abnormal liver chemistries are common in Turner syndrome (TS). Guidelines suggest that TS patients undergo annual screening of liver enzymes, but the role of non-invasive screening for steatosis and fibrosis is not clearly defined. We compared the prevalence of hepatic steatosis and fibrosis among TS patients to healthy controls using ultrasound with shear-wave elastography (SWE) and assessed for risk factors associated with steatosis and fibrosis in TS. METHODS: Prospective case-control study of TS versus control patients from 2019 to 2021. All patients underwent abdominal ultrasound with doppler and SWE to assess hepatic fibrosis and steatosis. Risk factors were compared between TS and controls, as well as within the TS group. RESULTS: A total of 55 TS and 50 control patients were included. Mean age was 23.6 years vs. 24.6 years in the control group (p = .75). TS patients had significantly more steatosis (65% vs. 12%, stage 1 vs. 0, p < .0001) and fibrosis (39% vs. 2%, average Metavir F2 vs. F0, p < .00001) than controls. These findings remained significant after adjusting for body mass index (BMI) (p < .01). GGT is more sensitive than AST or ALT in identifying these changes. CONCLUSION: TS is associated with an increased prevalence of hepatic steatosis and fibrosis compared to healthy controls. Our findings suggest that serum GGT and ultrasound with SWE may help identify TS patients with liver disease. Early risk factor mitigation including timely oestrogen replacement, weight control, normalization of lipids and promoting multidisciplinary collaboration should be encouraged.

Impaired Myocardial Work in Children with Hypertrophic Cardiomyopathy and Left Ventricular Fibrosis on Cardiac Magnetic Resonance Imaging.

Echocardiography is pivotal for diagnosis and monitoring of hypertrophic cardiomyopathy (HCM) and can evaluate myocardial function using myocardial work (MW) calculations. Echocardiography is often supplemented by cardiovascular magnetic resonance (CMR) imaging, which can detect myocardial fibrosis using late gadolinium enhancement (LGE). We sought to study the relationship between baseline LGE and MW at baseline and during follow-up in pediatric HCM patients. During the study period (2008-2023), 75 patients were followed up for HCM. In 14 patients (age 14.2 ± 2.8 years, 50.0% male, 6.4 ± 2.9 years follow-up), both LGE-CMR and echocardiography were performed. Global work index (GWI), global constructive work (GCW), global wasted work, and global work efficiency (GWE) were measured, and myocardial fibrosis was estimated by qualitative assessment of LGE. Patients with LGE (n = 7) exhibited significantly impaired baseline MW, including GWI (mean difference, MD - 487.4 mmHg %, 95% CI [- 866.8 mmHg % to - 108.3 mmHg %], p = 0.027), GCW (MD - 536.8 mmHg %, 95% CI [- 929.8 mmHg % to - 144.4 mmHg %], p = 0.020), and GWE (MD - 4.4%, 95% CI [- 8.1% to - 0.7%], p = 0.039). Regional analysis revealed impaired MW indices in segments with LGE, notably basal and mid septal segments. GWI demonstrated high diagnostic performance for LGE presence (sensitivity 93%, specificity 88%, and area under receiver operating characteristic curve 0.85). Baseline LGE presence had no significant impact on MW deterioration during follow-up. MW is significantly impaired in HCM patients with myocardial fibrosis, highlighting potential utility of echocardiography-derived MW analysis as a valuable tool.

Predictors of Valve Failure Following Surgical Atrioventricular Valve Replacement with a Melody Valve in Infants and Children.

Options for atrioventricular (AV) valve replacement in small pediatric patients are very limited. The Melody valve has shown reasonable short-term outcomes. This study was aimed at identifying predictors of valve failure following AV valve replacement with a Melody valve at a single-center. 26 patients underwent 37 AV valve replacements with 31/37 (84%) of valves placed in the systemic AV valve position. Median age at procedure was 17 months (IQR 4-33) and weight was 8.5 kg (IQR 6.25-12.85). Median balloon size for valve implant was 20 mm (IQR 18-22). Repeat intervention occurred in 21 cases (57%) with repeat surgery in all but one. Median freedom from re-intervention was 31 months; 19% were free from re-intervention at 60 months. Age < 12 months weight < 10 kg and BSA < 0.4 m2 were all significant risk factors for early valve failure (p = 0.003, p 0.017, p 0.025, respectively). Valve longevity was greatest with balloon inflation to diameter 1.20-1.35 times the patient's expected annular diameter (Z0), relative to both smaller or larger balloons (p = 0.038). In patients less than 12 months of age, patients with single ventricle physiology had an increased risk of early valve failure (p = 0.004).

Impact of Phlebotomy Volume Knowledge on Provider Laboratory Ordering and Transfusion Practices in the Pediatric Cardiac ICU.

OBJECTIVES: Phlebotomy can account for significant blood loss in post-surgical pediatric cardiac patients. We investigated the effectiveness of a phlebotomy volume display in the electronic medical record (EMR) to decrease laboratory sampling and blood transfusions. Cost analysis was performed. DESIGN: This is a prospective interrupted time series quality improvement study. Cross-sectional surveys were administered to medical personnel pre- and post-intervention. SETTING: The study was conducted in a 19-bed cardiac ICU (CICU) at a Children's hospital. PATIENTS: One hundred nine post-surgical pediatric cardiac patients weighing 10 kg or less with an ICU stay of 30 days or less were included. INTERVENTIONS: We implemented a phlebotomy volume display in the intake and output section of the EMR along with a calculated maximal phlebotomy volume display based on 3% of patient total blood volume as a reference. MEASUREMENTS AND MAIN RESULTS: Providers poorly estimated phlebotomy volume regardless of role, practice setting, or years in practice. Only 12% of providers reported the availability of laboratory sampling volume. After implementation of the phlebotomy display, there was a reduction in mean laboratories drawn per patient per day from 9.5 to 2.5 ( p = 0.005) and single electrolytes draw per patient over the CICU stay from 6.1 to 1.6 ( p = 0.016). After implementation of the reference display, mean phlebotomy volume per patient over the CICU stay decreased from 30.9 to 14.4 mL ( p = 0.038). Blood transfusion volume did not decrease. CICU length of stay, intubation time, number of reintubations, and infections rates did not increase. Nearly all CICU personnel supported the use of the display. The financial cost of laboratory studies per patient has a downward trend and decreased for hemoglobin studies and electrolytes per patient after the intervention. CONCLUSIONS: Providers may not readily have access to phlebotomy volume requirements for laboratories, and most estimate phlebotomy volumes inaccurately. A well-designed phlebotomy display in the EMR can reduce laboratory sampling and associated costs in the pediatric CICU without an increase in adverse patient outcomes.

Derivation and Validation of a General Predictive Model for Long Term Risks for Mortality and Invasive Interventions in Congenital and Acquired Cardiac Conditions Encountered in the Young.

Accurate prognostic assessment is a key driver of clinical decision making in heart disease in the young (HDY). This investigation aims to derive, validate, and calibrate multivariable predictive models for time to surgical or catheter-mediated intervention (INT) and for time to death in HDY. 4108 unique subjects were prospectively and consecutively enrolled, and randomized to derivation and validation cohorts. Total follow-up was 26,578 patient-years, with 102 deaths and 868 INTs. Accelerated failure time multivariable predictive models for the outcomes, based on primary and secondary diagnoses, pathophysiologic severity, age, sex, genetic comorbidities, and prior interventional history, were derived using piecewise exponential methodology. Model predictions were validated, calibrated, and evaluated for sensitivity to changes in the independent variables. Model validity was excellent for predicting mortality and INT at 4 months, 1, 5, 10, and 22 years (areas under receiver operating characteristic curves 0.813-0.915). Model calibration was better for INT than for mortality. Age, sex, and genetic comorbidities were significant independent factors, but predicted outcomes were most sensitive to variations in composite predictors incorporating primary diagnosis, pathophysiologic severity, secondary diagnosis, and prior intervention. Despite 22 years of data acquisition, no significant cohort effects were identified in which predicted mortality and intervention varied by study entry date. A piecewise exponential model predicting survival and freedom from INT is derived which demonstrates excellent validity, and performs well on a clinical sample of HDY outpatients. Objective model-based predictions could educate both patient and provider, and inform clinical decision making in HDY.

Impact of the new UNOS donor heart allocation system on waitlist outcomes and early posttransplant mortality among adults with congenital heart disease.

Adults with congenital heart disease (ACHD) experience worse waitlist outcomes and higher early posttransplant mortality compared to non-ACHD patients. On October 18, 2018; the UNOS donor heart allocation system was redesigned giving unique listing status to ACHD patients. The impact of this change on outcomes in transplant-listed patients is unstudied. Using the Scientific Registry of Transplant Recipients (SRTR) we compared ACHD patients listed for the first-time for heart transplantation from two eras of equal duration. We analyzed waitlist outcomes, posttransplant mortality and length of stay among ACHD patients in both eras and between ACHD and non-ACHD patients in the new era. Of 12 723 listed patients, 535 had ACHD (293 in the new era) and 12 188 did not (6258 in the new era). A total of 163 (56%) ACHD patients in the new era versus 150 (62%) in the prior era were transplanted; 11 (3.8%) versus 15 (6.2%) died on the waitlist; 32 (11%) versus 35 (14%) were delisted and 15 (9.2%) versus 19 (12.7%) died within 30 days of transplant, respectively. The new UNOS donor heart allocation system improved waitlist time and decreased the proportion not transplanted during the first 300 days after listing among ACHD patients without altering early posttransplant outcomes or significantly changing the gap in outcomes compared to non-ACHD patients.

Artificial intelligence in pediatric cardiology: taking baby steps in the big world of data.

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.

Left ventricular myocardial work indices in pediatric hypertension: correlations with conventional echocardiographic assessment and subphenotyping.

Myocardial work (MW) is a novel parameter that incorporates non-invasive estimates of left ventricular (LV) systolic pressure into the interpretation of strain, thus overcoming the limitations of load dependency traditionally encountered with LV fractional shortening (LVFS), ejection fraction (LVEF), and global longitudinal strain (GLS). However, data on MW in the pediatric population with hypertension are lacking. Conventional markers of LV function and MW indices were obtained from 88 echocardiographic examinations in 76 children with hypertension (47 males, 15.5 ± 2.96 years). When compared with a previously published cohort of 52 healthy controls, global work index (GWI) and global constructive work (GCW) were both significantly elevated while LVEF and GLS were not impaired but rather mildly increased. On multivariable analysis, GWI was correlated with systolic blood pressure (slope = + 16, p < 0.001) and GLS (slope = -100, p < 0.001), while GCW was correlated with systolic blood pressure (slope = + 18, p < 0.001), GLS (slope = -101, p < 0.001), male sex (slope = -75, p = 0.016), and LV mass (slope = -0.93, p < 0.001). Global wasted work (GWW) was correlated with age at echo visit (slope = -4.5, p = 0.005) and GLS (slope = + 5.5, p < 0.001). The opposite occurred for global work efficiency (GWE; slope = + 0.20, p = 0.011, and slope = -0.48, p < 0.001, respectively). Principal component analysis and k-means clustering revealed 4 subphenotypes which differed in terms of etiology, afterload, and compensation stage of the disease.    Conclusion: Non-invasive MW shows a good correlation with conventional markers of LV function and may help refine the assessment of hypertensive heart disease in children. What is Known: • Myocardial work (MW) is a novel parameter that incorporates non-invasive estimates of left ventricular (LV) systolic pressure into the interpretation of strain, thereby accounting for both deformation and afterload. • Although the usefulness of MW in the assessment of myocardial function beyond conventional markers has been demonstrated in various clinical populations, data in the pediatric population with hypertension are currently lacking. What is New: • Compared to normal values in healthy children, global work index and global constructive work were increased in those with hypertension, while LV ejection fraction and global longitudinal strain were not impaired. • Machine learning identified 4 subphenotypes which differed in terms of etiology, afterload, and compensation stage of the disease.

Assessment of Hypertension, Guideline-Directed Counseling, and Outcomes in the ACHD Population.

Guidelines for the diagnosis and treatment of hypertension were published by the American Heart Association (AHA) in 2017. The prevalence of hypertension in adults with congenital heart disease (ACHD) under these guidelines has yet to be characterized. We sought to assess the prevalence, impact, and provider response to hypertension under current guidelines. Data were obtained retrospectively from records of routine clinic visits over a 10 year period. Potential hypertension-related adverse outcomes including stroke, myocardial infarction, surgical intervention for aortic aneurysm, aortic dissection, atrial fibrillation or flutter, cardiac transplantation and death were recorded. The 1070 patients who met inclusion criteria had a mean age of 30.8 ± 10.0 years. The prevalence of hypertension under the 2017 guidelines was 46.6%. Multivariate modeling identified cyanosis, male gender, older age, and overweight/obesity as independent risk factors for hypertension. Guideline-directed management of hypertension in ACHD patients occurred more frequently in ACHD and adult cardiology clinics than in pediatric cardiology clinics (44.1% and 45.1% vs. 24.0%, p < 0.01, respectively). Adverse outcomes were reported in 217 (20%) patients, the most prevalent of which was atrial fibrillation or flutter (11%). Multivariable modelling for any adverse outcome identified older age, hypertension, cyanosis, greater complexity ACHD, and obesity as risk factors. Modifiable risk factors for atherosclerotic cardiovascular disease are common and often under addressed in the ACHD population.

Oxygen Uptake Efficiency Slope Predicts Adverse Outcome Following Atrial Switch Procedure.

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.

Machine Learning-Enabled Fully Automated Assessment of Left Ventricular Volume, Ejection Fraction and Strain: Experience in Pediatric and Young Adult Echocardiography.

BACKGROUND: Left ventricular (LV) volumes, ejection fraction (EF), and myocardial strain have been shown to be predictive of clinical and subclinical heart disease. Automation of LV functional assessment overcomes difficult technical challenges and complexities. We sought to assess whether a fully automated assessment of LV function could be reliably used in children and young adults. METHODS: Fifty normal volunteers (22/28, female/male) were prospectively recruited for research echocardiography. LV volumes, EF, and strain were measured both manually and automatically. An experienced sonographer performed all the manual analysis and recorded the analysis timing. The fully automated analyses were accomplished by 5 groups of observers with different knowledge and medical background. AutoLV and AutoSTRAIN (TomTec) were employed for the fully automated LV analysis. The LV volumes, EF, strain, and analysis time were compared between manual and automated methods, and among the 5 groups of observers. RESULTS: Software-determined endocardial border detection was achievable in all subjects. The analysis times of the experienced sonographer were significantly shorter for AutoLV and AutoSTRAIN than manual analyses (both p < 0.001). Strong correlations were seen between conventional EF and AutoLV (r = 0.8373), and between conventional three view global longitudinal strain (GLS) and AutoSTRAIN (r = 0.9766). The volumes from AutoLV and three view GLS from AutoSTRAIN had strong correlations among different observers regardless of level of expertise. EF from AutoLV analysis had moderately strong correlations among different observers. CONCLUSION: Automated pediatric LV analysis is feasible in normal hearts. Machine learning-enabled image analysis saves time and produces results that are comparable to traditional methods.

Fetal Echocardiographic Predictors of Postnatal Surgical Strategies in Critical Pulmonary Stenosis or Atresia with Intact Ventricular Septum: A Meta-Analysis.

BACKGROUND: Critical pulmonary stenosis or atresia with intact ventricular septum (PSAIVS) may be managed either by biventricular repair or univentricular palliation. This systematic review and meta-analysis aimed to synthesize the evidence for the role of fetal echocardiography in predicting the postnatal treatment pathway. METHODS: PubMed/MEDLINE, CINHAL, Cochrane Library, Academic Search Complete, Web of Science, and Trip Pro were searched for observational studies published before July 2021. Random-effects meta-analysis was performed to identify factors associated with biventricular repair. RESULTS: Eleven individual studies published between 2006 and 2021, including a total of 285 participants (159 biventricular repair; 126 univentricular palliation), met our eligibility criteria. The pooled estimated prevalence of biventricular repair among patients with PSAIVS was 55.6% (95% confidence interval 48.5-62.5%). Those who underwent biventricular repair had greater right to left ventricle and tricuspid to mitral valve dimension ratios, greater TV z score, and longer TV inflow duration/cardiac cycle length by fetal echocardiography. They were also more likely to have significant tricuspid regurgitation and less likely to have ventriculo-coronary connections (VCCs). CONCLUSIONS: Commonly obtained fetal echocardiographic measurements have strong associations with treatment pathway choice for patients with PSAIVS. Greater RV growth appears to favor biventricular repair, whereas patients with VCC almost invariably undergo univentricular palliation. Future studies should aim to establish how these fetal echocardiographic parameters might predict outcomes for the two treatment pathways.

Progression of left ventricular diastolic function in the neonate and early childhood from transmitral color M-mode filling analysis.

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 < 0.005) and IVPD between the age groups (p < 0.001). CONCLUSIONS: Comprehensive analysis of transmitral color M-mode data is feasible in infants, enabling calculation of pressure drop between the LV base and apex and strength of propagation from two distinct slopes. Profound changes very early followed by relatively constant filling mechanics in later infancy indicate significant LV maturation occurring during the first month of life. IMPACT: We implemented sophisticated analytic methods for color M-mode echocardiography in infants to assess age- and dimension-dependent changes in left ventricular diastolic function. Comprehensive characterization of transmitral color M-mode flow was feasible, enabling calculation of pressure drop between left ventricular base and apex and strength of propagation. Left ventricular diastolic filling function has predictable maturational progression, with significant differences in the intraventricular pressure between infants from birth to 2 years. This study forms the basis for future studies to examine alteration of early diastolic filling in congenital heart disease.

Age-Related Changes in Inferior Vena Cava Dimensions among Children and Adolescents with Syncope.

OBJECTIVE: To test the hypothesis that increased venous compliance manifested as inferior vena cava (IVC) dilation is an important substrate for syncope in children. STUDY DESIGN: IVC diameters were measured in 191 children and adolescents with syncope and in 95 controls. Subjects were divided based on age <12 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. RESULTS: 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 m2. Among controls, all IVC dimensions varied linearly with BSA (P < .001). In the older group (140 patients with syncope and 60 controls), all iIVC dimensions were larger in the syncope cohort: iIVC-RA, 9 vs 7.7 mm/m2 (P < .0001); iIVC-RA10, 9.4 vs 8.1 mm/m2 (P < .0001); iIVCmax, 11.7 vs 10.6 mm/m2 (P = .002). In the younger group (51 patients with syncope and 35 controls), there were no differences in iIVC measurements between the syncope cohort and controls: iIVC-RA, 10.2 vs 11.3 mm/m2; iIVC-RA10, 11.7 vs 12.0 mm/m2; iIVCmax, 14.2 vs 14.7 mm/m2 (P > .05 for all). CONCLUSIONS: The 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.

Can Abbreviated Cardiac Magnetic Resonance Imaging Adequately Support Clinical Decision Making After Repair of Tetralogy of Fallot?

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 < 0.0001, 95% CI (0.630, 0.869)]. Agreement for recommended timing of follow-up imaging was good (κ 0.64, p < 0.0001, 95% CI (0.474, 0.811)] in the 'no-intervention' group. When raters were asked whether or not further imaging was necessary, agreement was modest [κ 0.363 (p < 0.0001), 95% CI (0.038, 0.687)]. In conclusion, Abbreviated CMR yield decisions for clinical care similar to those made using the standard full protocol. These results suggest a potential enhancement of clinical practice in which efficiency and cost saving might be achieved using Abbreviated CMR for routine follow-up surveillance of TOF.

Utility of expert focused cardiac ultrasound in paediatric cardiology outreach clinics.

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.

Myocardial deformation assessed by longitudinal strain: Chamber specific normative data for CMR-feature tracking from the German competence network for congenital heart defects.

PURPOSE: 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). METHODS: 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. RESULTS: 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<0.05); correlations with age were weak. Gender-wise differences were not significant for atrial and ventricular LS, strain rate and displacement. Inter- and intra-observer comparisons showed moderate agreements. CONCLUSIONS: Chamber-specific nomograms for paediatric atrial and ventricular LS are provided to serve as clinical reference, and to facilitate CMR-based deformation research. KEY POINTS: • No normative data exist for CMR-derived global longitudinal strain in the young. • This prospective study provides reference data for atrial and ventricular longitudinal strain. • The data will serve as reference for CMR-based clinical and research use.

Assessment of ventriculo-vascular properties in repaired coarctation using cardiac magnetic resonance-derived aortic, left atrial and left ventricular strain.

OBJECTIVES: In patients with repaired coarctation of aorta (CoA), we assessed ventriculo-vascular characteristics using CMR-derived aortic area strain (AAS), left atrial (LA) and left ventricular (LV) longitudinal and circumferential strain (LS, CS). METHODS: Seventy-five subjects including 50 with repaired CoA divided into hypertensive (n = 25), normotensive (n = 25) and 25 controls were studied. AAS was measured at 3 levels: ascending aorta, proximal descending and descending aorta. LA and LV LS were measured using CMR-feature tracking. LA and LV end-diastolic volumes, ejection fraction (EF) and mass were measured. RESULTS: Mean patient age was 19.7 ± 6.7 and controls 23 ± 15 (years). All strains (LA, LV, ascending and descending aortic) were lower in CoA subgroups compared to controls except the AAS at diaphragm, which was not different. Comparisons between hypertensive and normotensive CoA showed no differences in LV mass, LV volumetric indices, and LA and LV strain indices; however, ascending AAS was lower in hypertensive subgroup (p = 0.02). Ascending AAS was correlated with LV mass (r = -0.4, p = 0.005), LVEF (r = -0.4, p = 0.004), systolic blood pressure (r = -0.5, p = 0.0001) and LVLS (r = 0.5, p = 0.001). CONCLUSIONS: Ascending AAS correlated with LV mass, EF and LVLS. In hypertensive CoA, ascending AAS was reduced compared to normotensive CoA and controls, indicating vascular remodelling differences influenced by ongoing hypertension. KEY POINTS: • Impaired arterial strain is a measure of increased stiffness in arteries • Ascending aorta strain correlates with left ventricular mass and longitudinal strain • Ascending aorta strain is significantly lower in hypertensive coarctation patients • Hypertension may be a consequence of vascular pathology persisting despite repair.

Traditional Long-Term Central Venous Catheters Versus Transhepatic Venous Catheters in Infants and Young Children.

OBJECTIVES: 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. DESIGN: Retrospective review of 12 congenital heart disease patients from September 2013 to July 2015 who underwent placement of one or more transhepatic venous catheters. SETTING: Single freestanding pediatric hospital located in the central United States. PATIENTS: Pediatric patients with congenital heart disease who underwent placement of transhepatic venous catheter. INTERVENTIONS: Cohort's central venous catheter complication rates and duration of catheter service were compared with transhepatic venous catheter data. MEASUREMENTS AND MAIN RESULTS: 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. CONCLUSIONS: 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.

Clinical Implications of a Multivariate Stratification Model for the Estimation of Prognosis in Ventricular Septal Defect.

OBJECTIVES: To derive and validate a multivariate stratification model for prediction of survival free from intervention (SFFI) in ventricular septal defect (VSD). A secondary aim is for this model to serve as proof of concept for derivation of a more general congenital heart disease prognostic model, of which the VSD model will be the first component. STUDY DESIGN: For 12 years, 2334 subjects with congenital heart disease were prospectively and consecutively enrolled. Of these, 675 had VSD and form the derivation cohort. One hundred seven other subjects with VSD followed in another practice formed the validation cohort. The derivation cohort was serially stratified based on clinical and demographic features correlating with SFFI. RESULTS: Six strata were defined, the most favorable predicting nearly 100% SFFI at 10 years, and the least favorable, a high likelihood of event within weeks. Strata with best SFFI had many subjects with nearly normal physiology, muscular VSD location, or prior intervention. In the validation cohort, the relation between predicted and actual SFFI at 6 months, 1 year, 2 years, and 5 years follow-up had areas under the receiver operating characteristic curves 0.800 or greater. CONCLUSIONS: A prediction model for SFFI in VSD has been derived and validated. It has potential for clinical application to the benefit of patients and families, medical trainees, and practicing physicians.