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To report the prevalence of coarctation of the aorta (CoA) in fetuses with single left superior vena cava (SL-SVC) and to evaluate changes in echocardiographic measurements. Additionally, to report the prevalence of associated malformations. Retrospective observational study of fetuses diagnosed with SL-SVC between 2012 and 2021 at a tertiary fetal cardiology unit. In fetuses without intracardiac abnormalities, Z-scores of the ventricles, great arteries, and Doppler flow patterns are reported. We identified 47 fetuses with SL-SVC of which 8/47 (17%) had abnormal intracardiac anatomy. One fetus was lost to follow-up. Of those with normal intracardiac anatomy and postnatal follow-up (38), karyotype abnormalities were confirmed in 2/38 (5%) and ECA in 8/38 (21%). 33/38 were live-born. None developed CoA postnatally. Paired analysis of Z-scores between early and late scans of 24 fetuses showed that diameters of the right heart structures and Doppler flows of tricuspid valve increased significantly during pregnancy, while the left heart structures and flow patterns did not change. The median risk of CoA did not change between the early and the late scan. We did not observe CoA in this cohort. A degree of ventricular asymmetry was present, but this was due to right heart dominance rather than hypoplasia of left heart structures. This likely reflects redistribution of blood and does not appear to confer increased risk of CoA. Predictive models of the postnatal development of CoA which set the dimensions of right and left heart structures in relation might not be applicable in this situation. [ABSTRACT FROM AUTHOR]

Supplemental Digital Content is available in the text. Background: Assessment of the outflow tract views is an integral part of routine fetal cardiac scanning. For some congenital heart defects, notably coarctation of the aorta, pulmonary valve stenosis, and aortic valve stenosis, the size of vessels is important both for diagnosis and prognosis. Existing reference ranges of fetal outflow tracts are derived from a small number of cases. Methods and Results: The study population comprised 7945 fetuses at 13 to 36 weeks’ gestation with no detectable abnormalities from pregnancies resulting in normal live births. Prospective measurements were taken of (1) the aortic and pulmonary valves in diastole at the largest diameter with the valve closed, (2) the distal transverse aortic arch on the 3 vessel and trachea view beyond the trachea at the distal point at its widest systolic diameter, and (3) the arterial duct on the 3 vessel and trachea view at its widest systolic diameter. Regression analysis, with polynomial terms to assess for linear and nonlinear contributors, was used to establish the relationship between each measurement and gestational age. The measurement for each cardiac diameter was expressed as a z score (difference between observed and expected value divided by the fitted SD corrected for gestational age) and percentile. Analysis included calculation of gestation-specific SDs. Regression equations are provided for the cardiac outflow tracts and for the distal transverse aortic arch:arterial duct ratio. Conclusions: The study established reference ranges for fetal outflow tract measurements at 13 to 36 weeks’ gestation that are useful in clinical practice. [ABSTRACT FROM AUTHOR]

The author discusses the role of telemedicine in a fetal cardiology service. The article "Fetal diagnosis of congenital heart disease (CHD) by telemedicine" by B. A. McCrossan et al., offers a significant contribution relating to the role of telemedicine in the delivery of a system for prenatal detection and management of CHD.

A retrospective review of admission preductal oxygen saturations of neonates with antenatally diagnosed critical congenital heart disease (CCHD) was performed to investigate the differences in newborn pulse oximetry (Pulsox) by specific CCHD diagnosis. Saturations were recorded at median of < 1 h (range < 1-9 h) after delivery. Data was stratified by CCHD diagnosis and analysed according to the three different admission Pulsox thresholds, ≤ 90, ≤ 92 and ≤ 95%. Of the 276 neonates studied, 208 were clinically well at admission, with no co-morbidities, gestation > 34 weeks and birth weight > 1.8 kg. A statistically significant increase in the proportion with low admission saturations was seen using ≤ 95% saturation threshold (72% (95% CI 66-78)) compared to ≤ 92% (52% (95% CI 46-59)) and ≤ 90% (46% (95% CI 39-52)). Sub-group analysis found the proportion of neonates with low saturations varied according to the specific CCHD diagnosis with only 20-42% of neonates with aortic stenosis, coarctation of the aorta and pulmonary stenosis having saturations ≤ 95%.Conclusion: The proportion of neonates with low admission oxygen saturation varied by CCHD diagnosis with those without critically reduced pulmonary blood flow not having low admission saturations, in general, even using the ≤ 95% threshold which had the highest proportions of abnormal saturations. This data may assist developing Pulsox screening policies. What is Known: • The addition of pulse oximetry (Pulsox) screening to the routine newborn examination increases the sensitivity of CCHD detection. Pulsox screening is also highly specific for CCHD in asymptomatic neonates, with low false-positive rates. • Early diagnosis of CCHD improves patient outcomes in relation to both morbidity and mortality. What is New: • The proportion of affected infants with an abnormal Pulsox result varies by CCHD diagnosis and screening threshold. In our study using the ≤ 95% threshold gave the highest proportion of neonates with abnormal saturations at admission. • In general, Pulsox yield of abnormal results is low for CCHD diagnoses not associated with critically reduced pulmonary blood flow; however, increasing the Pulsox threshold increased the proportion of infants with an abnormal result. [ABSTRACT FROM AUTHOR]

A correction to the article "Incidence of left ventricular hypertrophy in children with kidney disease: impact of method of indexation of left ventricular mass" that was published in 2010 issue is presented.

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Our goal was to describe postoperative complications and outcomes in a large contemporary cohort of children with an isolated double aortic arch (DAA) or a right aortic arch (RAA) with left arterial ligament and to assess the impact of foetal diagnosis on outcomes. METHODS We performed a retrospective analysis of all patients who underwent surgery for DAA or RAA with left arterial ligament between 2005 and 2019. RESULTS A total of 132 children were operated on for a DAA (n  = 77) or a RAA (n  = 55). Prenatal diagnosis was made in 100/132 (75.8%). Median age at surgery for DAA was 5.0 (1.7–13.3) months and for RAA was 13.9 (6.4–20.1) months. There was no difference in the age at surgery between the prenatal and postnatal cases (8.6 [4.0–15.6] vs 5.4 months [1.8–17.7]; P  = 0.37). No surgical deaths occurred. Vocal cord palsy was the most common complication, occurring in 12/132 (9%): of these, 11 resolved spontaneously and 1 required a temporary tracheostomy. Logistic regression demonstrated that older age at operation was the only predictor for a postoperative complication (P  = 0.02). Overall, 21/67 (31%) of prenatally detected, symptomatic cases reported residual symptoms/signs 1 year after surgery compared to 18/28 (64%) of postnatally detected cases. Postnatal diagnosis was associated with persistent postoperative symptoms/signs [ P  = 0.006, odds ratio = 3.9 (95% confidence interval 1.5–9.4)]. CONCLUSIONS Surgery to relieve a vascular ring resolves trache-oesophageal compressive symptoms in most cases, but parents/patients should be aware that symptoms/signs may persist in the first postoperative year despite effective release of the vascular ring. Earlier surgery and prenatal diagnosis may improve outcomes. [ABSTRACT FROM AUTHOR]

Highlights: [•] A new 3D velocity recovery method from 3+registered Doppler images is presented. [•] Variations in Doppler images are simulated with a new multiscale noise model. [•] We carry out a sensitivity analysis to view angles, noise and registration errors. [•] Error in volume on 3 volunteers agree with predicted error from simulation studies. [•] Preliminary results on three patients are accurate and capture complex flow patterns. [Copyright &y& Elsevier]

Coarctation of the aorta (COA) is suspected prenatally when there is ventricular asymmetry, arterial disproportion, and hypoplasia of the aortic arch/isthmus. The presence of fetal shunts creates difficulty in prenatal confirmation of the diagnosis so serial echocardiography after birth is necessary to confirm or refute the diagnosis. The first neonatal echocardiogram in prenatally suspected cases of COA was assessed for prediction of neonatal COA repair (NCOAR). This included morphological assessment, measurement of the aortic arch and calculation of the distal arch index (DAI = distance between left common carotid and left subclavian artery/diameter of the distal arch). NCOAR was undertaken in 23/60 (38%) cases. Transverse arch, aortic isthmus z-score, and DAI had an area under the receiver operator curve of 0.88 (95% CI 0.77–0.98), 0.86 (95% CI 0.75–0.96), and 0.84 (95% CI 0.74–0.95), respectively for the prediction of NCOAR. Using transverse arch z-score threshold < − 3 gave sensitivity 100%, NPV: 100%, specificity 76%; aortic isthmus z-score < − 3: NPV 92%, specificity 62% and DAI > 1.4: NPV 88%, specificity 78%. The size of the distal aortic arch in infants with a common origin of the innominate artery and left common carotid artery who did not require COA repair was similar to the NCOAR cases (p = 0.22). The early postnatal assessment of the size and morphology of the aortic arch can assist in risk stratification for development of neonatal COA. The branching pattern of the head/neck vessels impacts on the size of the distal aortic arch adding to the complexity of predicting COA based on vessel size. [ABSTRACT FROM AUTHOR]

Unlabelled: Targeted echocardiographic assessments of haemodynamic status are increasingly utilised in many settings. Application in the neonatal intensive care units (NICU) is increasingly demanded but challenging given the risk of underlying structural lesions. This statement follows discussions in UK led by the Neonatologists with an Interest in Cardiology and Haemodynamics (NICHe) group in collaboration with the British Congenital Cardiac Association (BCCA) and the Paediatricians with Expertise in Cardiology Special Interest Group (PECSIG). Clear consensus was agreed on multiple aspects of best practice for neonatologist-performed echocardiogram (NoPE)-rigorous attention to infection control and cardiorespiratory/thermal stability, early referral to paediatric cardiology with suspicion of structural disease, reporting on standardised templates, reliable image storage, regular skills maintenance, collaboration with a designated paediatric cardiologist, and regular scan audit/review. It was agreed that NoPE assessments should confidently exclude structural lesions at first scan. Practitioners would be expected to screen and establish gross normality of structure at first scan and obtain confirmation from paediatric cardiologist if required, and subsequently, functional echocardiography can be performed for haemodynamic assessment to guide management of newborn babies. To achieve training, NICHe group suggested that mandatory placements could be undertaken during core registrar training or neonatal subspecialty grid training with a paediatric cardiology placement for 6 months and a neonatology placement for a minimum of 6 months. In the future, we hope to define a precise curriculum for assessments. Technological advances may provide solutions-improvements in telemedicine may have neonatologists assessing haemodynamic status with paediatric cardiologists excluding structural lesions and neonatal echocardiography simulators could increase exposure to multiple pathologies and allow limitless practice in image acquisition.Conclusion: We propose developing training places in specialist paediatric cardiology centres and neonatal units to facilitate training and suggest all UK practitioners performing neonatologist-performed echocardiogram adopt this current best practice statement.What Is Known: Neonatologist-performed echocardiogram (NoPE) also known as targeted neonatal echocardiography (TNE) or functional ECHO is increasingly recognised and utilised in care of sick newborn and premature babies. There are differences in training for echocardiography across continents and formal accreditation processes are lacking.What Is New: This is the first document of consensus best practice statement for training of neonatologists in neonatologist-performed echocardiogram (NoPE), jointly drafted by Neonatologists with interest in cardiology & haemodynamics (NICHe), paediatric cardiology and paediatricians with expertise in cardiology interest groups in UK. Key elements of a code of practice for neonatologist-performed echocardiogram are suggested. [ABSTRACT FROM AUTHOR]

There are conflicting reports on the prenatal validity of echocardiographic indexes used to assess tricuspid valve malformations (TVM) in postnatal life. The aim of this study was to determine which echocardiographic factors are of most prognostic significance in prenatally-diagnosed TVM and to develop a clinically-based prognostic scoring system to better inform prenatal counseling. From a prospective database, 44 fetuses with isolated TVM were identified from 1995 to 2004, inclusive. Prenatal echocardiographic findings were correlated with known outcome in 43. Tricuspid valve dyplasia was diagnosed in 22, Ebstein’s anomaly in 21, and unguarded tricuspid valve orifice in 1. There were 19 terminations, 9 intrauterine deaths, 5 neonatal deaths, and 10 survivors >1 month. Survival was 35% at birth and 23% at 1 month, or 63% and 42% respectively on an intention-to-treat basis. There was no significant change to termination or overall survival rates over time, but there was a significant improvement in survival for live-born babies in the second 5 years compared with the first (p = 0.02). Factors significantly associated with increased mortality included increased cardiothoracic ratio (p <0.001), Celermajer index (p <0.001), and right–left ventricular ratio (p = 0.02); reduced/absent pulmonary valve flow (p = 0.02), and retrograde duct flow (p = 0.003). These factors were combined to give a prognostic score (SAS score) with good predictive value. In conclusion, the prenatal echocardiographic factors of most prognostic significance in TVM are similar to those used postnatally. The SAS score is a useful clinical tool that may be used to inform counseling in affected pregnancies. [Copyright &y& Elsevier]