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Objectives Bisphosphonates are used in childhood osteoporosis but can cause an acute phase reaction (APR) and hypocalcemia. We present a child with cardiac thrombosis following zoledronate, a previously unreported complication. Case presentation An 11-year-old with Duchenne muscular dystrophy and steroid-induced osteoporosis presented 48 h after first zoledronate infusion with fever, tachycardia, tachypnoea and hypoglycaemia. This was managed as acute adrenal crisis and possible sepsis. He also had hypocalcemia, hypophosphatemia, hyponatraemia and hypokalaemia. Echocardiography performed due to persistent chest pain and tachycardia revealed a left ventricular thrombus. Conclusions Potential causes for intracardiac thrombosis in this patient include ventricular dysfunction due to acute adrenal crisis or electrolyte disturbance, and hypercoagulability due to the APR. Echocardiography should be considered in children with acute cardiovascular compromise following zoledronate. Stress-dose steroids to cover the APR and a reduced starting dose of zoledronate might have reduced the risk of this complication.

Childhood hypertrophic cardiomyopathy; Outcomes; Phenotype Miocardiopatía hipertrófica infantil; Resultados; Fenotipo Miocardiopatia hipertròfica infantil; Resultats; Fenotip Background Up to one-half of childhood sarcomeric hypertrophic cardiomyopathy (HCM) presents before the age of 12 years, but this patient group has not been systematically characterized. Objectives The aim of this study was to describe the clinical presentation and natural history of patients presenting with nonsyndromic HCM before the age of 12 years. Methods Data from the International Paediatric Hypertrophic Cardiomyopathy Consortium on 639 children diagnosed with HCM younger than 12 years were collected and compared with those from 568 children diagnosed between 12 and 16 years. Results At baseline, 339 patients (53.6%) had family histories of HCM, 132 (20.9%) had heart failure symptoms, and 250 (39.2%) were prescribed cardiac medications. The median maximal left ventricular wall thickness z-score was 8.7 (IQR: 5.3-14.4), and 145 patients (27.2%) had left ventricular outflow tract obstruction. Over a median follow-up period of 5.6 years (IQR: 2.3-10.0 years), 42 patients (6.6%) died, 21 (3.3%) underwent cardiac transplantation, and 69 (10.8%) had life-threatening arrhythmic events. Compared with those presenting after 12 years, a higher proportion of younger patients underwent myectomy (10.5% vs 7.2%; P = 0.045), but fewer received primary prevention implantable cardioverter-defibrillators (18.9% vs 30.1%; P = 0.041). The incidence of mortality or life-threatening arrhythmic events did not differ, but events occurred at a younger age. Conclusions Early-onset childhood HCM is associated with a comparable symptom burden and cardiac phenotype as in patients presenting later in childhood. Long-term outcomes including mortality did not differ by age of presentation, but patients presenting at younger than 12 years experienced adverse events at younger ages. This work was supported by the British Heart Foundation (grant FS/16/72/32270) to Drs Norrish and Kaski. This work is (partly) funded by the National Institute for Health Research Great Ormond Street Hospital Biomedical Research Centre. Dr Norrish is supported by Great Ormond Street Hospital Children’s Charity. Drs Field and Kaski are supported by Max’s Foundation and Great Ormond Street Hospital Children’s Charity. Dr Kaski is supported by a Medical Research Council–National Institute for Health Research Clinical Academic Research Partnership award. This work was financially supported by the Foundation for Paediatric Research of Finland (Dr Ojala). Dr Fernandez has received speaker fees from Sanofi-Genzyme. Dr Kubus is supported by MH CZ – DRO, Motol University Hospital (00064203). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Aims Sudden cardiac death (SCD) is the most common mode of death in paediatric hypertrophic cardiomyopathy (HCM). This study describes the implant and programming strategies with clinical outcomes following implantable cardioverter-defibrillator (ICD) insertion in a well-characterized national paediatric HCM cohort. Methods and results Data from 90 patients undergoing ICD insertion at a median age 13 (±3.5) for primary (n = 67, 74%) or secondary prevention (n = 23, 26%) were collected from a retrospective, longitudinal multi-centre cohort of children ( Conclusion In a large national cohort of paediatric HCM patients with an ICD, device and programming strategies varied widely. No particular strategy was associated with inappropriate therapies, missed/delayed therapies, or lead complications.

The annual meeting of the European Association of Cardiovascular Imaging, EuroEcho 2019, was held in Vienna, Austria, in December 2019. In this article, we present a summary of the ‘Highlights’ session.

Background: Maximal left ventricular wall thickness (MLVWT) is a risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). In adults, the severity of left ventricular hypertrophy has a nonlinear relationship with SCD, but it is not known whether the same complex relationship is seen in childhood. The aim of this study was to describe the relationship between left ventricular hypertrophy and SCD risk in a large international pediatric HCM cohort. Methods: The study cohort comprised 1075 children (mean age, 10.2 years [±4.4]) diagnosed with HCM (1–16 years) from the International Paediatric Hypertrophic Cardiomyopathy Consortium. Anonymized, noninvasive clinical data were collected from baseline evaluation and follow-up, and 5-year estimated SCD risk was calculated (HCM Risk-Kids). Results: MLVWT Z score was Z scores were associated with heart failure symptoms, unexplained syncope, left ventricular outflow tract obstruction, left atrial dilatation, and nonsustained ventricular tachycardia. One hundred twenty-two patients (71.3%) with MLVWT Z score ≥20 had coexisting risk factors for SCD. Over a median follow-up of 4.9 years (interquartile range, 2.3–9.3), 115 (10.7%) had an SCD event. Freedom from SCD event at 5 years for those with MLVWT Z scores Z score, peaking at Z score +23. The presence of coexisting risk factors had a summative effect on risk. Conclusions: In children with HCM, an inverted U-shaped relationship exists between left ventricular hypertrophy and estimated SCD risk. The presence of additional risk factors has a summative effect on risk. While MLVWT is important for risk stratification, it should not be used either as a binary variable or in isolation to guide implantable cardioverter defibrillator implantation decisions in children with HCM.

Aims: Children presenting with hypertrophic cardiomyopathy (HCM) in infancy are reported to have a poor prognosis, but this heterogeneous group has not been systematically characterized. This study aimed to describe the aetiology, phenotype, and outcomes of infantile HCM in a well-characterized multicentre European cohort. Methods and results: Of 301 children diagnosed with infantile HCM between 1987 and 2019 presenting to 17 European centres [male n=187 (62.1%)], underlying aetiology was non-syndromic (n=138, 45.6%), RASopathy (n=101, 33.6%), or inborn error of metabolism (IEM) (n=49, 16.3%). The most common reasons for presentation were symptoms (n=77, 29.3%), which were more prevalent in those with syndromic disease (n=62, 61.4%, P&nbsp

Purpose of the study The purpose was to explore the experience, information, support needs and decision-making of parents with congenital heart disease (CHD) during the COVID-19 crisis. Study design On-line survey design Setting An on-line survey with open/closed questions to explore the experiences of parents of children, as well as children and young people (CYP) with CHD during the COVID-19 crisis Patients Parents of children with CHD and CYP Results 184 parents and 36 CYP completed the survey. Parents worry about the virus (86.4%) vs. CYP (69.4%), whilst (89%) parents are vigilant for symptoms of the virus vs. CYP(69.4%). A thematic analysis of the qualitative comments covered 34 subthemes, forming eight-overarching themes: Virus:1)risk of infection, 2)information, guidance and advice, 3)change in health care provision, and 4)fears and anxieties; Lockdown and isolation: 5)psychological and social impact, 6)keeping safe under lockdown, 7)provisions and dependence on others, 8)employment and income. Conclusions Parents and CYP were worried about the virus, although CYP less so. Parents and children however, were frustrated with the lack of specific and paediatric focused information and guidance, expressing disappointment with the adult centric information available. Parents also felt alone, especially with their concerns around the implications of cardiac service suspension and the implication for their child’s health. In order to better support children and their families, resources need to be developed to address families’ and CYP concerns for their health during this pandemic. What is already known? People are feeling vulnerable and have heightened levels of stress during the COVID-19 pandemic Mental health and well-being of individuals has been adversely affected during this health crisis The COVID-19 pandemic has affected the mental health of parents due to uncertain financial circumstances, the closure of schools and suspension of educational services for children What this study adds? Parents worry about the virus and are vigilant for symptoms of the virus or cardiac symptoms. Parents felt abandonded, unsupported by their Government, local and specialist CHD clinical team, and wanted information specfic to their child’s cardiac diagnosis Families feel forgotten and that medical/ surgical procedures children had been waiting for were no longer prioritized, which added another level of abandonment

Introduction Sudden cardiac death is the most common cause of mortality in childhood onset hypertrophic cardiomyopathy. Identifying individuals at highest risk is therefore an essential part of clinical care but remains challenging. The 12 lead electrocardiogram (ECG) has been proposed as a useful tool for risk stratification and an ECG risk score has been proposed. However, this has not been independently validated and the ECG phenotype of childhood HCM has not been previously described. The aim of this study was to describe the ECG phenotype of childhood HCM in a large, international, multi-centre cohort and investigate its role in risk prediction for arrhythmic events. Methods Participants with an available baseline resting 12-lead ECG were identified from a large, international, multi-centre, retrospective cohort of patients aged less than 16 years fulfilling the diagnostic criteria for HCM (n=1029). Resting baseline ECG was evaluated and ECG variables were extracted. In addition, the ECG risk score based on 8 parameters (deviation in QRS axis, pathological T-wave inversion in limb or precordial leads, ST-segment depression, dominant S-wave in V4, limb-lead amplitude sum, 12-lead amplitude duration product and QTc) was calculated as previously described. The primary study endpoint was a composite outcome of major cardiac events (MACE) defined as SCD, resuscitated cardiac arrest, appropriate implantable cardioverter defibrillator therapy, or sustained ventricular tachycardia (VT) with haemodynamic compromise. The discriminatory performance of using an ECG risk score >5 to identify patients at increased risk of MACE at 5 years was determined using Harrell’s C-index. Results Of 356 patients with childhood HCM (68.9% male, mean age at presentation 10.1 ± 4.5 years), 347 (97.5%) had baseline ECG abnormalities such as: repolarization abnormalities (n=277, 77.8%), left ventricular hypertrophy (n=240. 67.6%), abnormal QRS axis (n=126, 35.4%) or QT prolongation (n=131, 36.8%). Over a median follow up of 3.9 years (IQR 2.0-7.7), 25 (7%) had an arrhythmic event, with an overall annual event rate of 1.38 (95% CI 0.93-2.04). No ECG variables were associated with 5-year MACE on univariable or multivariable Cox regression analysis. Of the 164 participants with an ECG score >5, 153 (93.3%) did not have a MACE within 5 years. Harrell’s C-index (the probability of correctly distinguishing between high and low risk patients using an ECG risk score threshold of >5) was 0.60 (95% CI 0.484-0.715) at 5 years. The corresponding positive and negative predictive values were 6.7% (95% CI 4.7 – 9.4%) and 96.9% (95% CI 94.2 – 98.4%). Conclusions In a large, international, multi-centre cohort of children with HCM, ECG abnormalities are common. No ECG characteristic, either in isolation or combined in the ECG risk score, was associated with 5-year MACE risk. This suggests that the role of the baseline ECG phenotype in improving risk stratification in childhood HCM is limited. Conflict of Interest None

ObjectiveHypertrophic cardiomyopathy (HCM) is an important predictor of long-term outcomes in Friedreich’s ataxia (FA), but the clinical spectrum and survival in childhood is poorly described. This study aimed to describe the clinical characteristics of children with FA-HCM.Design and settingRetrospective, longitudinal cohort study of children with FA-HCM from the UK.Patients78 children (InterventionAnonymised retrospective demographic and clinical data were collected from baseline evaluation and follow-up.Main outcome measuresThe primary study end-point was all-cause mortality (sudden cardiac death, atrial arrhythmia-related death, heart failure-related death, non-cardiac death) or cardiac transplantation.ResultsThe mean age at diagnosis of FA-HCM was 10.9 (±3.1) years. Diagnosis was within 1 year of cardiac referral in 34 (65.0%) patients, but preceded the diagnosis of FA in 4 (5.3%). At baseline, 65 (90.3%) had concentric left ventricular hypertrophy and 6 (12.5%) had systolic impairment. Over a median follow-up of 5.1 years (IQR 2.4–7.3), 8 (10.5%) had documented supraventricular arrhythmias and 8 (10.5%) died (atrial arrhythmia-related n=2; heart failure-related n=1; non-cardiac n=2; or unknown cause n=3), but there were no sudden cardiac deaths. Freedom from death or transplantation at 10 years was 80.8% (95% CI 62.5 to 90.8).ConclusionsThis is the largest cohort of childhood FA-HCM reported to date and describes a high prevalence of atrial arrhythmias and impaired systolic function in childhood, suggesting early progression to end-stage disease. Overall mortality is similar to that reported in non-syndromic childhood HCM, but no patients died suddenly.

Neonates requiring congenital cardiac surgery are at risk of acute kidney injury, associated with significant morbidity, mortality, and increased hospital length of stay; treatment may require renal replacement therapy. Data for single cardiac defect cohorts is important to stratify risk, but is lacking for transposition of the great arteries. Our study aimed at collecting data for this single lesion. A single-centre, retrospective analysis of 71 cases of arterial switch operation in neonates with isolated transposition of the great arteries, or transposition of the great arteries with ventricular septal defect, including length of stay, renal function and need for renal replacement therapy was performed. Acute kidney injury developed in 50.7%, and was associated with longer paediatric intensive care and hospital stays (p This study analyses renal outcomes in a cohort of neonates with transposition of the great arteries undergoing an arterial switch operation. Acute kidney injury is a significant complication, with accompanying need for renal replacement therapy. Development of acute kidney injury and a positive fluid balance were associated with increased length of stay. Initiation of renal replacement therapy was not associated with increased length of stay, and with some evidence from the literature that early or prophylactic peritoneal dialysis catheter insertion improves outcomes, these data report minimal complication rates which may be important when deciding to utilise peritoneal dialysis.

Background: Paediatric Multisystem Inflammatory Syndrome temporally associated with SARS-CoV-2 (PIMS-TS), first identified in April 2020, shares features of both Kawasaki disease (KD) and toxic shock syndrome (TSS). The surveillance describes the epidemiology and clinical characteristics of PIMS-TS in the United Kingdom and Ireland. Methods: Public Health England initiated prospective national surveillance of PIMS-TS through the British Paediatric Surveillance Unit. Paediatricians were contacted monthly to report PIMS-TS, KD and TSS cases electronically and complete a detailed clinical questionnaire. Cases with symptom onset between 01 March and 15 June 2020 were included. Findings: there were 216 cases with features of PIMS-TS alone, 13 with features of both PIMS-TS and KD, 28 with features of PIMS-TS and TSS and 11 with features of PIMS-TS, KD and TSS, with differences in age, ethnicity, clinical presentation and disease severity between the phenotypic groups. There was a strong geographical and temporal association between SARS-CoV-2 infection rates and PIMS-TS cases. Of those tested, 14.8% (39/264) children had a positive SARS-CoV-2 RT-PCR, and 63.6% (75/118) were positive for SARS-CoV-2 serology. In total 44·0% (118/268) required intensive care, which was more common in cases with a TSS phenotype. Three of five children with cardiac arrest had TSS phenotype. Three children (1·1%) died. Interpretation: the strong association between SARS-CoV-2 infection and PIMS-TS emphasises the importance of maintaining low community infection rates to reduce the risk of this rare but severe complication in children and adolescents. Close follow-up will be important to monitor long-term complications in children with PIMS-TS. Funding: PHE

Paediatric inflammatory multisystem syndrome temporally associated with COVID-19 (PIMS-TS) is a novel condition that was first reported in April, 2020. We aimed to develop a national consensus management pathway for the UK to provide guidance for clinicians caring for children with PIMS-TS. A three-phase online Delphi process and virtual consensus meeting sought consensus over the investigation, management, and research priorities from multidisciplinary clinicians caring for children with PIMS-TS. We used 140 consensus statements to derive a consensus management pathway that describes the initial investigation of children with suspected PIMS-TS, including blood markers to help determine the severity of disease, an echocardiogram, and a viral and septic screen to exclude other infectious causes of illness. The importance of a multidisciplinary team in decision making for children with PIMS-TS is highlighted throughout the guidance, along with the recommended treatment options, including supportive care, intravenous immunoglobulin, methylprednisolone, and biological therapies. These include IL-1 antagonists (eg, anakinra), IL-6 receptor blockers (eg, tocilizumab), and anti-TNF agents (eg, infliximab) for children with Kawasaki disease-like phenotype and non-specific presentations. Use of a rapid online Delphi process has made it possible to generate a national consensus pathway in a timely and cost-efficient manner in the middle of a global pandemic. The consensus statements represent the views of UK clinicians and are applicable to children in the UK suspected of having PIMS-TS. Future evidence will inform updates to this guidance, which in the interim provides a solid framework to support clinicians caring for children with PIMS-TS. This process has directly informed new PIMS-TS specific treatment groups as part of the adaptive UK RECOVERY trial protocol, which is the first formal randomised controlled trial of therapies for PIMS-TS globally.

Aims The 12-lead electrocardiogram (ECG) is routinely performed in children with hypertrophic cardiomyopathy (HCM). An ECG risk score has been suggested as a useful tool for risk stratification, but this has not been independently validated. This aim of this study was to describe the ECG phenotype of childhood HCM in a large, international, multi-centre cohort and investigate its role in risk prediction for arrhythmic events. Methods and results Data from 356 childhood HCM patients with a mean age of 10.1 years (±4.5) were collected from a retrospective, multi-centre international cohort. Three hundred and forty-seven (97.5%) patients had ECG abnormalities at baseline, most commonly repolarization abnormalities (n = 277, 77.8%); left ventricular hypertrophy (n = 240, 67.7%); abnormal QRS axis (n = 126, 35.4%); or QT prolongation (n = 131, 36.8%). Over a median follow-up of 3.9 years (interquartile range 2.0–7.7), 25 (7%) had an arrhythmic event, with an overall annual event rate of 1.38 (95% CI 0.93–2.04). No ECG variables were associated with 5-year arrhythmic event on univariable or multivariable analysis. The ECG risk score threshold of >5 had modest discriminatory ability [C-index 0.60 (95% CI 0.484–0.715)], with corresponding negative and positive predictive values of 96.7% and 6.7% Conclusion In a large, international, multi-centre cohort of childhood HCM, ECG abnormalities were common and varied. No ECG characteristic, either in isolation or combined in the previously described ECG risk score, was associated with 5-year sudden cardiac death risk. This suggests that the role of baseline ECG phenotype in improving risk stratification in childhood HCM is limited.

Background: Paediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2 (PIMS-TS), first identified in April 2020, shares features of both Kawasaki disease (KD) and toxic shock syndrome (TSS). Methods: Public Health England initiated prospective national surveillance of PIMS-TS through the British Paediatric Surveillance Unit. Paediatricians were contacted monthly to report PIMS-TS, KD and TSS cases electronically and complete a detailed clinical questionnaire. Cases with symptom onset between 01 March and 15 June 2020 were included. Findings: There were 216 cases with features of PIMS-TS alone, 13 with features of both PIMS-TS and KD, 28 with features of PIMS-TS and TSS and 11 with features of PIMS-TS, KD and TSS, with differences in age, ethnicity, clinical presentation and disease severity between the three phenotypic groups. There was a strong geographical and temporal association between SARS-CoV-2 infection rates and PIMS-TS cases. Of those tested, 14·8% (39/264) children had a positive SARS-CoV-2 RT-PCR, and 63·6% (75/118) were positive for SARS-CoV-2 serology. In total 118 children (44·0%) required intensive care, which was more common in cases with a TSS phenotype: 89/216 (41·2%) with PIMS-TS only vs. 19/28 (67·9%) with PIMS-TS/TSS and 6/11 (54·5%) with PIMS-TS/KD/TSS. Three of five children with cardiac arrest had PIMS-TS/TSS phenotype. Three children (1·1%) died. Interpretation: The strong association between SARS-CoV-2 infection and PIMS-TS emphasises the importance of maintaining low community infection rates to reduce the risk of this rare but severe complication in children and adolescents. Close follow-up will be important to monitor long-term complications in children with PIMS-TS. Funding: PHE Declaration of Interests: None to declare. Ethics Approval Statement: PHE has legal permission under Regulation 3 of The Health Service (Control of Patient Information) Regulations 2002, to conduct national surveillance of communicable diseases in England and, as such, individual patient consent is not required. Public Health Wales, through the established order legislation, is required to conduct surveillance of communicable diseases in Wales and, as such, individual patient consent is not required. The surveillance protocol was approved by the Public Benefit and Privacy Panel for Health and Social Care in Scotland (Ref: 2021-0041, 19th May 2020).

Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2020., The coronavirus disease 2019 (COVID-19) pandemic has created new and unpredictable challenges for modern medicine and healthcare systems. Preliminary reports have demonstrated that older age, previous cardiovascular disease, diabetes, and hypertension are risk factors for increased mortality. Data on the cardiac affinity of the virus and its potential to harm the cardiovascular system and the mechanisms by which this occurs are sparse. A systemic infection generally increases demand on the heart, and can exacerbate underlying cardiac conditions. When the lungs are heavily involved, as seen in COVID-19 patients, this may have a major impact on cardiac function, particularly that of the right ventricle. Finally, COVID-19 may have direct effects on the heart, as may some drugs being used in its treatment.

Background Irbesartan, a long acting selective angiotensin-1 receptor inhibitor, in Marfan syndrome might reduce aortic dilatation, which is associated with dissection and rupture. We aimed to determine the effects of irbesartan on the rate of aortic dilatation in children and adults with Marfan syndrome. Methods We did a placebo-controlled, double-blind randomised trial at 22 centres in the UK. Individuals aged 6–40 years with clinically confirmed Marfan syndrome were eligible for inclusion. Study participants were all given 75 mg open label irbesartan once daily, then randomly assigned to 150 mg of irbesartan (increased to 300 mg as tolerated) or matching placebo. Aortic diameter was measured by echocardiography at baseline and then annually. All images were analysed by a core laboratory blinded to treatment allocation. The primary endpoint was the rate of aortic root dilatation. This trial is registered with ISRCTN, number ISRCTN90011794. Findings Between March 14, 2012, and May 1, 2015, 192 participants were recruited and randomly assigned to irbesartan (n=104) or placebo (n=88), and all were followed for up to 5 years. Median age at recruitment was 18 years (IQR 12–28), 99 (52%) were female, mean blood pressure was 110/65 mm Hg (SDs 16 and 12), and 108 (56%) were taking β blockers. Mean baseline aortic root diameter was 34·4 mm in the irbesartan group (SD 5·8) and placebo group (5·5). The mean rate of aortic root dilatation was 0·53 mm per year (95% CI 0·39 to 0·67) in the irbesartan group compared with 0·74 mm per year (0·60 to 0·89) in the placebo group, with a difference in means of −0·22 mm per year (−0·41 to −0·02, p=0·030). The rate of change in aortic Z score was also reduced by irbesartan (difference in means −0·10 per year, 95% CI −0·19 to −0·01, p=0·035). Irbesartan was well tolerated with no observed differences in rates of serious adverse events. Interpretation Irbesartan is associated with a reduction in the rate of aortic dilatation in children and young adults with Marfan syndrome and could reduce the incidence of aortic complications. Funding British Heart Foundation, the UK Marfan Trust, the UK Marfan Association.

Summary Objective Infants with congenital heart disease (CHD) often experience growth failure prior to surgery, which is associated with increased paediatric-intensive-care unit length of stay (PICU-LOS) and post-operative complications. This study assessed the impact of a pre-operative, consensus-based nutritional pathway (including support from a multi-disciplinary team) on growth and clinical outcome. Design Single-centre prospective pilot study. Setting Tertiary paediatric cardiology surgical centre. Patients Infants with CHD. Intervention Infants with CHD were followed for up to 4-months-of-age before cardiac surgery and then to 12-months-of-age following the implementation of the consensus-based nutritional-pathway (Intervention group: November 2017–August 2018), with outcomes compared to a historic control group. The nutrition pathway involved a dietitian contacting parents of infants with the highest risk of growth failure weekly; reviewing weight gain and providing feeding support. Main outcome measure Growth (weight-for-age, WAZ, and height-for-age-z-score, HAZ) at 4 and 12 months-of-age. Results 44 infants in the intervention group were compared to 38 in the control group. Median (inter quartile range) change in WAZ from birth to 4 months-of-age (−0.9 (−1.5, 0.7)) and from birth to 12 months-of-age (−0.09 (−1.3, 1.1)) in the intervention group compared to the control group (−1.5 (−2.0, −0.4) (p = 0.04)) at 4 months-of age and at 12 months-of-age (−0.4 (1.9, 0.2) (p = 0.03)). HAZ at 4 months-of-age was −0.7 (−1.4, −0.1) vs. −1.0 (−1.9, −0.3) (p = 0.6) in the intervention and control groups respectively, and at 12 months-of-age HAZ was −0.7 (−1.9, −0.07) in the intervention group vs.-1.6 (−2.6, −0.4) in the control group (p = 0.04). Duration of PICU-LOS was 8.2 ± 11.6 days intervention vs. 18.3 ± 24.0 days control (p = 0.006). Conclusion Overall weight was well maintained and growth improved in infants who followed the pre-operative nutritional-pathway. The duration of PICU-LOS was significantly lower in the intervention group, which may be due to improved nutritional status, although this requires further investigation.

Introduction:Infants with CHD often experience growth failure. Ensuring optimal growth before surgery is associated with improved outcomes and has emerged as a significant cause of parental stress. Parents have reported a perceived lack of accessible feeding information for infants with CHD. To address this gap, the aim of this study was to develop feeding information to better support parents.Materials and methods:A search for existing material on six electronic databases and an internet search for unpublished (grey) literature on feeding information for infants with CHD were carried out. Following the development of feeding information, semi-structured interview(s) with parents/health-care professionals were completed, focusing on whether the information was easy to understand, relevant, provided sufficient information around feeding/feeding difficulties, and whether there were any information gaps. Iterative changes were made to the information following each interview. The process was completed until thematic saturation was achieved.Results:A total of 23 unique articles were identified of which 5 studies were included. From the grey literature, four web pages were reviewed. A total of 22 parents and 25 health-care professionals were interviewed. All parents/health-care professionals felt that the feeding information developed provided sufficient information; however, many wanted information on how to introduce complementary food, particularly if weaning was delayed.Conclusions:This study describes the development of feeding information for infants with CHD. From parent interviews, gaps identified focused on the introduction of complementary foods and uncertainty regarding the feeding journey beyond surgery.

The annual meeting of the European Association of Cardiovascular Imaging, EuroEcho-Imaging, was held in Milan, Italy, in December 2018. In the present paper, we report a summary of the 'Highlights' session.

Accepted manuscript version, licensed CC BY-NC-ND 4.0. Background - Right ventricular (RV) function is a major determinant of survival in hypoplastic left heart syndrome (HLHS). However, the relation of RV geometry to myocardial mechanics and their relation to transplant-free survival are incompletely characterized. Methods - We retrospectively studied 48 HLHS patients from the Hospital for Sick Children, Toronto, (median age, 2.2; interquartile range, 3.62 years) at different surgical stages. Patients were grouped by the presence (n = 23) or absence (n = 25) of RV “apical bulging” defined as a sigmoid-shaped septum with the RV leftward apical segment contiguous with the left ventricular (LV) lateral wall. Regional and global RV strain were measured using speckle-tracking echocardiography, and regional strains were analyzed for patterns and peak values. These were compared between HLHS anatomical subtypes and between patients with versus without apical bulging. We further investigated the association between RV geometry and dysfunction with the outcomes of heart failure, death, or transplant. Results - RV global (–7.3% ± 2.8% vs –11.2% ± 4.4%; P = .001), basal septal (–3.8% ± 3.2% vs –11.4% ± 5.8%; P = .0001) and apicolateral (–5.1% ± 3.5% vs –8.0% ± 5.8%, P = .001) longitudinal strain were lower in patients with versus without apical bulging, respectively. Apical bulging was equally prevalent in all HLHS anatomical variants. Twenty of 22 (91%) patients with apical bulging displayed hypertrophy of the LV apical and lateral segments. Death or transplantation were approximately equal in both groups but related to reduced RV global strain in patients with (seven of seven) and not in those without apical bulging (two of eight; P = .022). Conclusions - These results suggest that the finding of apical bulging is related to the presence of a hypertrophied hypoplastic LV, with a negative impact on regional and global RV function. Therefore, analysis of RV and LV geometry and mechanics may aid in the assessment and prognostication of this high-risk population.

Aims Sudden cardiac death (SCD) is the most common cause of death in children with hypertrophic cardiomyopathy (HCM). The European Society of Cardiology (ESC) recommends consideration of an implantable cardioverter-defibrillator (ICD) if two or more clinical risk factors (RFs) are present, but this approach to risk stratification has not been formally validated. Methods and results Four hundred and eleven paediatric HCM patients were assessed for four clinical RFs in accordance with current ESC recommendations: severe left ventricular hypertrophy, unexplained syncope, non-sustained ventricular tachycardia, and family history of SCD. The primary endpoint was a composite outcome of SCD or an equivalent event (aborted cardiac arrest, appropriate ICD therapy, or sustained ventricular tachycardia), defined as a major arrhythmic cardiac event (MACE). Over a follow-up period of 2890 patient years (median 5.5 years), MACE occurred in 21 patients (7.5%) with 0 RFs, 19 (16.8%) with 1 RFs, and 3 (18.8%) with 2 or more RFs. Corresponding incidence rates were 1.13 [95% confidence interval (CI) 0.7–1.73], 2.07 (95% CI 1.25–3.23), and 2.52 (95% CI 0.53–7.35) per 100 patient years at risk. Patients with two or more RFs did not have a higher incidence of MACE (log-rank test P = 0.34), with a positive and negative predictive value of 19% and 90%, respectively. The C-statistic was 0.62 (95% CI 0.52–0.72) at 5 years. Conclusions The incidence of MACE is higher for patients with increasing numbers of clinical RFs. However, the current ESC guidelines have a low ability to discriminate between high- and low-risk individuals.

Background Classical management of small infants with tetralogy of Fallot has involved placement of a Blalock-Taussig shunt followed later by complete repair, rather than primary complete repair which is the strategy adopted in larger infants. Some advantages of early complete repair compared to a staged strategy have been shown. We sought to review our institutional outcomes. Methods Patients under 3-months old undergoing complete surgical repair of tetralogy of Fallot in our institution between 2005 and 2015 were retrospectively reviewed and compared with an older control group matched by anatomical diagnosis and outflow tract intervention. Results Fourteen index cases (group A) and 14 controls (group B) were identified. At surgery, the median age was 43 days and weight 4.2 kg in group A, and 130 days and 6.1 kg in group B. Nine of 14 in group A were admitted for surgery as emergencies compared to none in group B. Peak inotrope score (22.3 vs. 12.8, p = 0.02) and intensive care unit stay (4.4 vs. 2.6 days, p = 0.02) were higher in group A. Bypass and crossclamp times, duration of intubation, and total length of stay did not differ. Conclusions We conclude that although babies undergoing early repair of tetralogy of Fallot have an increased need for intensive care support in the early postoperative period, the total length of stay is not prolonged despite more emergency admissions. As it is known that early repair may reduce long-term morbidity, we propose consideration of earlier complete repair of tetralogy of Fallot.

ObjectiveGrowth among infants with CHD is poor, and is multifactorial with multiple contributing factors. Unexplained hypophosphataemia has been reported among infants and children with complex medical needs consuming amino acid infant formula as the sole source of nutrition. The aim of this audit was therefore to review the incidence of hypophosphataemia among infants with CHD.MethodsThe use of an electronic patient record search for “amino acid infant formula”, “CHD”, and “cardiac” yielded 136 infants 1 year of age (n=11) and infants with a structurally normal heart (n=31) were excluded from the study. The remaining 66 infants with CHD were given amino acid infant formula.Measurements and main resultsIn all, 1059 serum phosphate measures were available. After the introduction of amino acid infant formula, significantly more infants with CHD had episodes of hypophosphataemia: 15% (n=10/66) before treatment versus 29% (n=19/66) after treatment (p=0.049). Mean serum phosphate levels were significantly lower in infants with CHD following consumption of amino acid infant formula (2.0±0.5 versus 1.5±0.5 mmol/L following treatment (pConclusionAfter the introduction of an amino acid formula, weight gain was significantly lower among those infants with low phosphate levels. There was a significantly higher prevalence of hypophosphataemia among infants with CHD after the introduction of amino acid infant formula. Lower phosphate levels were associated with lower weight-for-age z scores. Infants with CHD are susceptible to poor weight gain; it is therefore, crucial the nutritional status of infants prescribed amino acid infant formula is more closely monitored to ensure adequate growth.

IntroductionDespite improvements in the medical and surgical management of infants with CHD, growth failure before surgery in many infants continues to be a significant concern. A nutritional pathway was developed, the aim of which was to provide a structured approach to nutritional care for infants with CHD awaiting surgery.Materials and methodsThe modified Delphi process was development of a nutritional pathway; initial stakeholder meeting to finalise draft guidelines and develop questions; round 1 anonymous online survey; round 2 online survey; regional cardiac conference and pathway revision; and final expert meeting and pathway finalisation.ResultsPaediatric Dietitians from all 11 of the paediatric cardiology surgical centres in the United Kingdom contributed to the guideline development. In all, 33% of participants had 9 or more years of experience working with infants with CHD. By the end of rounds 1 and 2, 76 and 96% of participants, respectively, were in agreement with the statements. Three statements where consensus was not achieved by the end of round 2 were discussed and agreed at the final expert group meeting.ConclusionsNutrition guidelines were developed for infants with CHD awaiting surgery, using a modified Delphi process, incorporating the best available evidence and expert opinion with regard to nutritional support in this group.

Background: Children with cardiomyopathy (CM) are at risk of sudden cardiac death (SCD), but the incidence and risk factors for this outcome are not clear.Objectives: This study sought to d...

Importance: Sudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM), but there is no validated algorithm to identify those at highest risk.Objective: To develop and validate an SCD risk prediction model that provides individualized risk estimates.Design, Setting, and Participants: A prognostic model was developed from a retrospective, multicenter, longitudinal cohort study of 1024 consecutively evaluated patients aged 16 years or younger with HCM. The study was conducted from January 1, 1970, to December 31, 2017.Exposures: The model was developed using preselected predictor variables (unexplained syncope, maximal left-ventricular wall thickness, left atrial diameter, left-ventricular outflow tract gradient, and nonsustained ventricular tachycardia) identified from the literature and internally validated using bootstrapping.Main Outcomes and Measures: A composite outcome of SCD or an equivalent event (aborted cardiac arrest, appropriate implantable cardioverter defibrillator therapy, or sustained ventricular tachycardia associated with hemodynamic compromise).Results: Of the 1024 patients included in the study, 699 were boys (68.3%); mean (interquartile range [IQR]) age was 11 (7-14) years. Over a median follow-up of 5.3 years (IQR, 2.6-8.3; total patient years, 5984), 89 patients (8.7%) died suddenly or had an equivalent event (annual event rate, 1.49; 95% CI, 1.15-1.92). The pediatric model was developed using preselected variables to predict the risk of SCD. The model's ability to predict risk at 5 years was validated; the C statistic was 0.69 (95% CI, 0.66-0.72), and the calibration slope was 0.98 (95%, CI 0.59-1.38). For every 10 implantable cardioverter defibrillators implanted in patients with 6% or more of a 5-year SCD risk, 1 patient may potentially be saved from SCD at 5 years.Conclusions and Relevance: This new, validated risk stratification model for SCD in childhood HCM may provide individualized estimates of risk at 5 years using readily obtained clinical risk factors. External validation studies are required to demonstrate the accuracy of this model's predictions in diverse patient populations.

Background Right ventricular (RV) dysfunction and tricuspid regurgitation (TR) are associated with adverse outcome in hypoplastic left heart syndrome (HLHS) but remain poorly understood. The aim of this study was to assess RV longitudinal strain and mechanical dyssynchrony in HLHS in relation to TR. The hypothesis was that inhomogeneous RV contraction and mechanical dyssynchrony around the tricuspid valve apparatus may be associated with TR in some patients with HLHS. Methods Echocardiograms of children aged 0 to 2 years with HLHS at all stages of surgical palliation were retrospectively reviewed for anatomic subtype and severity of TR. RV peak strain and dyssynchrony were assessed by vector velocity imaging. Results Sixty echocardiograms of patients with a median age of 0.54 years (interquartile range, 0.04–1.63 years) and a median weight 6.40 kg (interquartile range, 3.70–10.45 kg) demonstrated TR, which was absent or trivial in 25 (42%), mild in 20 (33%), moderate in five (8%), and severe in 10 (17%). The difference in peak longitudinal strain between the RV free wall and the septum or left-sided RV wall was significantly higher in patients with moderate or severe TR compared with no, trivial, or mild TR (2.93 ± 6.03% vs 0.16 ± 6.6%, P = .04). The difference in time to peak longitudinal strain between walls was significantly longer in moderate or severe TR compared with no, trivial, or mild TR (57.4 ± 145.1 vs 15.8 ± 75.9 msec, P = .04). There was a significant difference in anatomic subtype between patients with the most difference in peak strain compared with those with the least. Conclusions RV mechanical dyssynchrony and inhomogeneous contraction are worse in patients with clinically important TR and HLHS.

Many, if not most, of the controversies regarding the description of the congenitally malformed heart have been resolved over the turn of the 20th century. A group of lesions that remains contentious is the situation in which both arterial trunks, in their greater part, are supported by the morphologically right ventricle. It was considered, for many years, that presence of bilateral infundibulums, or conuses, was a necessity for such a diagnosis. It has now been appreciated that this suggestion founders on many counts. In the first instance, such bilateral infundibulums are to be found in patients with other ventriculo-arterial connections, including the otherwise normal heart. In the second instance, it is clear that such an approach abrogates the important principle now known as the morphological method. This states that entities should be defined in terms of their intrinsic morphology and not on the basis of other variable features. It is now also clear that, when assessed simply on the basis of the ventricular origin of the arterial trunks, a significant number of patients fulfil the criteria for so-called “200%” origin of the trunks from the right ventricle when there is fibrous continuity between the leaflets of the atrioventricular and arterial valves. In this review, we show how attention to the morphology of the channel between the ventricles now provides the key to accurately diagnose the ventriculo-arterial connection in patients with suspected double-outlet right ventricle.This is because, when both arterial trunks arise exclusively or predominantly from the morphologically right ventricle, the outlet septum, of necessity, is itself a right ventricular structure. The channel between the ventricles, therefore, is roofed by the inner heart curvature, whether that structure is fibrous or muscular. Our observations then confirm that it is the attachment of the outlet septum, which itself can be muscular or fibrous, which determines the commitment of the interventricular communication to the subarterial outlets. The interventricular communication itself, when directly committed to the ventricular outlets, opens between the limbs of the septomarginal trabeculation or septal band. The defect is subaortic when the outlet septum is attached to the cranial limb of the trabeculation, subpulmonary when attached to the caudal limb, and doubly committed when attached to the inner heart curvature in the roof of the defect. Non-committed defects are no longer positioned within the limbs of the septomarginal trabeculation. Although readily demonstrable by a skilled echocardiographer, we show how these anatomical features are more easily demonstrated with added accuracy when using CT data sets.

Cardiorespiratory collapse can have a number of causes in the neonate, and echocardiography is an important diagnostic tool in this population. While speed of diagnosis may be important, it is also critically important that the cardiologist is aware of the limitations and potential pitfalls of echocardiography. Echocardiographic evaluation must include a full and accurate assessment of anatomy and haemodynamics, and the cardiologist must also correctly interpret findings. We present a case that highlights the principles of interpretation.

An 8-week-old baby girl was diagnosed with aortopulmonary (AP) window after presenting to her local hospital with failure to thrive and respiratory distress. Echocardiogram also identified a right-sided aortic arch. Unfortunately, there was incomplete imaging of the arch branching pattern prior to surgery. Her early post-operative recovery period was complicated by two cardiac arrests, she remained in hospital for a long time, and after discharge continued to fail to thrive with frequent respiratory infections. Further investigations revealed a right-sided aortic arch with aberrant left subclavian artery forming a vascular ring. A vascular ring is a rare congenital anomaly of the great vessels, and the severity of symptoms is related to the degree of compression of the trachea and oesophagus. Some varieties of vascular ring are highly associated with other congenital cardiac defects. Currently there is no gold standard diagnostic imaging for vascular ring; however, computed tomography angiography (CTA) and cardiac magnetic resonance imaging (MRI) are superseding echocardiography because of their ability to provide detailed anatomy and relationship to other structures. A symptomatic untreated vascular ring carries significant risk of mortality and diagnosis is often delayed due to attribution of the respiratory symptoms to more common paediatric disorders.

Aims The multifactorial mechanisms of tricuspid valve (TV) insufficiency in patients with hypoplastic left heart syndrome (HLHS) include structural anomalies of TV leaflets and ventricular dilatation. We hypothesized that 2-D echocardiography underestimates the importance of TV structural abnormalities, whereas surgical assessment underestimates the importance of motion abnormalities, and compared echocardiographic assessment with surgical description. Methods and results Two independent experts retrospectively reviewed echocardiograms of all patients who had staged single-ventricular palliation and TV repair during January 1998–December 2008, and compared with case-matched controls who did not require TV repair. Primary and secondary mechanisms of TV insufficiency were categorized, and surgical findings ascertained from operation records. There were 32 patients with a median age of 5.9 months (0.3–140) and 32 matched controls. On echocardiographic review, an abnormality of at least one leaflet was noted in every patient (100%) vs. in only 14 controls 14 (44%) ( P < 0.001). Leaflet prolapse was described in 22 (69%), and the restriction of a leaflet in 20 (69%). Agreement between the experts was excellent ( κ = 0.64–0.88). On surgical inspection, annular dilatation was found in 17 (53%), and leaflet dysplasia in 14 (44%). Agreement between echocardiographic and surgical assessment was poor ( κ < 0.6). Conclusion Important structural abnormalities are common in patients with HLHS and TV insufficiency, some readily identified by 2-D echocardiography. However, there are significant discrepancies between echocardiographic and surgical findings. Echocardiographic assessment is sensitive to detect leaflet motion abnormalities, but not leaflet structural abnormalities. Both echocardiographers and surgeons should be aware of these limitations when planning surgical interventions.

Aims: Pressure gradient is used for timing of balloon aortic valvuloplasty for aortic stenosis (AS) in children, but does not correlate well with outcome and is limited if ventricular function is poor. In adults, effective orifice area (EOA) is used to assess AS severity, but EOA by continuity equation or 2D echo is unreliable in children. Three-dimensional echocardiography (3DE) may reliably assess EOA but has not been studied in children. We assessed measurement of aortic valve EOA by 3DE in children with AS before and after balloon aortic valvuloplasty and compared results with change in aortic valve gradient. Methods: 3DE was performed at time of catheterization before and after balloon aortic valvuloplasty. Using 3DE multiplanar review mode, valve annulus diameter, area, and EOA were measured and compared with change in aortic gradient and degree of aortic insufficiency. Results: Twenty-four 3DE studies in 12 children (mean age 4.4 ± 5.0 years) were analyzed. EOA was measurable in all. Catheter peak gradient decreased from 45 ± 10 to 26 ± 17 mmHg (P = 0.0018). 3DE EOA increased after balloon aortic valvuloplasty (0.59 ± 0.52 cm2 vs 0.80 ± 0.70 cm2; P = 0.03), without change in valve diameter. EOA change correlated with change in peak (r = 0.77; P = 0.005) and mean (r = 0.60; P = 0.03) aortic valve gradient post balloon aortic valvuloplasty. Conclusion: 3DE facilitates EOA measurement in pediatric AS and correlates with change in aortic valve gradient after balloon valvuloplasty. (Echocardiography 2012;29:484-491)

Background A potential genetic basis has been identified in around 30% of all childhood dilated cardiomyopathy (DCM). Despite this, the long-term outcomes for children with familial DCM have not been well characterized. This study examines the presentation, outcomes and echocardiographic findings for children with familial DCM enrolled in the National Australian Childhood Cardiomyopathy Study. Methods NACCS is a longitudinal, population based study which includes all children aged 0–10years who were diagnosed with primary cardiomyopathy between 1987 and 1996. Details of clinical status at diagnosis, medical therapy and late outcomes were recorded. Serial echocardiographic indices of LV size, fractional shortening and ejection fraction were expressed as Z scores, based on BSA and age. Familial cardiomyopathy was defined as the presence of an affected first or second degree relative with a similar cardiomyopathy. All available echocardiograms were interpreted by a single observer. Results There were 175 subjects with DCM. Children with familial DCM (27 cases) were younger at diagnosis than those with sporadic DCM (median age 3.2 vs. 8.4months ( p =.004) and were less likely to have congestive heart failure and to be hospitalised at presentation ( p ≤.002 for both). Familial DCM subjects had a higher mortality (62.9% vs. 36.5%; p =.02) and were less likely to be free of medical therapy at latest follow-up (7.4% vs. 52.0%; p p =.006) and a lower FS Z score (−7.35 vs. −3.40; p =.002) score at latest follow-up. Conclusions Familial DCM subjects are younger and are less likely to have symptomatic heart failure at the time of diagnosis. Despite this they have a higher mortality and worse ventricular function at late follow-up. This study underlines the differing presentations and outcomes among children with familial and sporadic DCM.

IntroductionWe aimed to assess the ability of the multiplanar review modality of three-dimensional echocardiography to examine the dynamic morphology and the functional characteristics of malformed tricuspid valves in patients previously identified as having Ebstein’s malformation. Based on these characteristics, we attempted to differentiate Ebstein’s malformation from tricuspid valvar dysplasia.MethodsUsing three-dimensional multiplanar review, analysed with either Qlab 6.0 or Tomtech Image Arena 3.0, we studied 23 patients, aged from 1 day to 70 years, previously diagnosed using cross-sectional echocardiography as having Ebstein’s malformation.ResultsUsing the features of rotational abnormality, and the orientation, of the effective tricuspid valvar orifice as diagnostic features of Ebstein’s malformation, we reclassified 11 patients (48 per cent) as exhibiting tricuspid valvar dysplasia. In addition, we studied the dynamic morphology as well as the function of the tricuspid valve. Surgical treatment was undertaken on 10 patients, revealing good correlation with the findings obtained using three-dimensional multiplanar review. In those with Ebstein’s malformation, we found varying degrees of rotation, with the effective valvar orifice always directed towards the right ventricular outflow tract. The opening of the orifice of dysplastic tricuspid valves, in contrast, was towards the apex of the right ventricle. The degree of delamination, and abnormalities of subcordal apparatus, were similar in the two groups.DiscussionThree-dimensional multiplanar review permits accurate definition of the dynamic morphology of Ebstein’s malformation, permitting clear differentiation from tricuspid valvar dysplasia.

Background Three-dimensional echocardiography offers new insights into valvar function in atrioventricular septal defects (AVSDs). The aim of this study was to identify a morphological marker to predict the functional outcomes of left atrioventricular valves (AVVs) following the repair of AVSDs. Methods Twenty-nine consecutive patients were evaluated preoperatively using 2-dimensional and 3-dimensional echocardiography. The angle of the AVV relative to the crux of the heart was measured in multiplanar review mode. Results The severity of postoperative left AVV regurgitation was correlated with preoperative valvar angle, being more acute in patients with moderate or severe regurgitation (mean, 57 ± 13° vs 83 ± 9° in patients with no or mild regurgitation; P = .002). Angles ≤ 59° predicted severe regurgitation with 79% specificity. Conclusions Multiplanar review of 3-dimensional data sets is valuable for the assessment of the functional morphology of AVSD valves. Using this technique, more acute AVV angles predicted increased likelihood of severe regurgitation following surgical repair.

IntroductionThe mitral valvar complex is difficult to visualise accurately in only two dimensions. Three-dimensional echocardiography gives new insight into the dynamic changes of intra-cardiac structures during the cardiac cycle. The aim of this study was to study the mitral annulus in systole and diastole in normal children using three-dimensional echocardiography, and to analyse the effect of regurgitation on annular function.Materials and methodsThree-dimensional echocardiographic datasets, acquired in 11 consecutive subjects with mitral regurgitation, and 20 normal subjects, were analysed offline using simultaneous multiplanar review.ResultsThe mitral valvar annular area decreased in diastole, and increased in systole, in both groups. The annulus in patients with mitral regurgitation is dilated compared to normal subjects, the systolic value for those with regurgitation having a mean of 6.79 plus or minus 2.55 centimetres2/metres2, and the diastolic value a mean of 5.01 plus or minus 1.78 centimetres2/metres2, as opposed to a systolic mean value of 5.28 centimetres2/metres2 plus or minus 1.68, p = 0.091, and diastolic mean value of 3.05 centimetres2/metres2 plus or minus 0.90, in normal subjects (p less than 0.0001). The proportional change in mitral valvar annular area from systole to diastole showed a trend towards being smaller in those with mitral regurgitation, although this did not reach significance (24.8% versus 41.13%, p equal to 0.249). Analysis of subgroups of patients with moderate or severe mitral regurgitation showed mitral excursion, expressed as percentage of left ventricular length, to be significantly less than in normal subjects, at 12.78 plus or minus 5.10% versus 15.84 plus or minus 4.23% (p equal to 0.012).ConclusionsMitral valvar annular area in children decreases in diastole, and increases in systole. In those with mitral regurgitation, the annulus is dilated and the dynamic annular function is depressed.

Aims Associated left ventricular structures may play a role in progression and recurrence of discrete subaortic stenosis. The availability of a new 3D echocardiography tool, multiplanar review (MPR), allows comprehensive analysis of datasets in infinite planes, and detailed examination of anatomy. We sought to evaluate the role of MPR in defining the morphology of subaortic stenosis. Methods Consecutive patients underwent detailed 2 and 3D echocardiographic examination using MPR. Results Sixteen patients aged 0.7–15.9 years (median 4.57) with diagnosis as follows: isolated subaortic stenosis in nine, additional defects in seven (coarctation of aorta, VSD, mitral, or aortic stenosis). Position and extent of subaortic stenosis was clearly described by multiplanar revew in all patients. Additional MPR findings were: abnormalities of mitral valve leaflet or chordal apparatus attachments (14 patients), abnormal ventricular muscle band (11), abnormal increased aorto-mitral separation (two). The aortoseptal angle was significantly decreased in subaortic stenosis, mean 141 ± 12°, vs. normal subjects, mean 153 ± 6°, P = 0.02. Surgical findings correlated well with MPR findings. Conclusions MPR analysis of 3D datasets is a sensitive and accurate mode for delineation of morphological details of discrete subaortic stenosis, providing additional information to 2D echocardiography.

Neonatal diabetes mellitus (hyperglycaemia within the first month of life, with an insulin requirement) may be transient or permanent. Management is complex, due to lack of subcutaneous fat and the need for small doses of insulin, and may be complicated by additional medical problems. Three cases are presented, the first of which was treated with conventional insulin therapy. The latter two were successfully treated with subcutaneous insulin pump therapy. We present suggested guidelines for treatment of neonatal diabetes, using this novel approach to management.

It is frequent, in the current era, to encounter congenital cardiac malformations described in terms of “cor triatriatum”. But can hearts be truly found with three atrial chambers? The morphological method, emphasised by Van Praagh et al, states that structures within the heart should be defined on the basis of their most constant components. In the atrial chambers, it is the appendages that are the most constant components, and to the best of our knowledge, hearts can only possess two appendages, which can be of either right or left morphology. The hearts described on the basis of “cor triatriatum”, nonetheless, can also be analysed on the basis of division of either the morphologically right or the morphologically left atriums. In this review, we provide a description of cardiac embryology, showing how each of the atrial chambers possesses part of the embryological body, along with an appendage, a vestibule, a venous component, and a septum that separates them. We then show how it is, indeed, the case that the hearts described in terms of “cor triatriatum” can be readily understood on the basis of division of these atrial components. In the right atrium, it is the venous valves that divide the chamber. In the left atrium, it is harder to provide an explanation for the shelf that produces atrial division. We also contrast the classic examples of the divided atrial chambers with the vestibular shelf that produces supravalvar stenosis in the morphologically left atrium, showing that this form of obstruction needs to be distinguished from the fibrous shelves producing intravalvar obstruction.

OBJECTIVES: Transposition of the great arteries (TGA) and left ventricular outflow tract obstruction (LVOTO) with or without ventricular septal defect have multiple surgical treatment options. We sought to identify pre- and intraoperative factors that determine the timing of repair, procedure type and subsequent LVOT outcome. METHODS: Twenty-eight (8.2% of all TGA) patients with TGA with LVOTO (double outlet ventricle, n= 5, TGA/intact septum, n =1 ) between 2000 and 2012 were reviewed. Anatomical factors were identified by prerepair echocardiography. LVOTO complexity was characterized by the degree of obstruction (0 = none, 0.33 = mild, 0.66 moderate and 1 = severe) at various levels: pulmonary valve (PV) dysplasia/hypoplasia, posterior deviation of the infundibular septum, fibromuscular ridge, tissue tag and abnormal chordal attachment. Summation of the obstruction score, at each level, yielded the LVOT complexity score. The descriptive analysis of intraoperative decisionmaking at late repair was performed. RESULTS: Operations: early arterial switch operation (ASO) + LVOT resection (n= 9, 32%), late ASO + LVOT resection (n= 3, 10%), Nikaidoh (n= 8, 29%), Rastelli (n= 6, 21%), single-ventricle palliation (n= 2, 7%). The primary LVOT obstruction mechanism was posterior deviation of the infundibular septum (n= 16, 57%) and PV dysplasia (n= 6, 21%). The early ASO group had a lower PV complexity score (0.42 ± 0.22 vs 0.96 ± 0.55, P= 0.007), tissue tag score (0.03 ± 0.15 vs 0.26 ± 0.34, P= 0.018) and LVOT complexity score (2.11 ± 0.86 vs 3.2 ± 0.96, P= 0.006). The LVOT complexity score in the Nikaidoh group was higher than in the late ASO group (P= 0.019). Of 16 candidates for the Nikaidoh procedure, 6 patients underwent a Rastelli operation due to coronary artery patterns (single coronary, n= 3, 1RL-2Cx, n= 2 or an abnormal left anterior descending coronary artery course, n= 1). Two patients underwent single-ventricle palliation due to the interference of essential chordae. All patients survived the operation. The 3-year survival was 96%. One patient who underwent late ASO required re-LVOT resection. CONCLUSIONS: A newly developed scoring system, the LVOT complexity score, helped to quantify the LVOT complexity and was correlated with our choice of the surgical procedure of TGA with LVOTO. The current strategy achieved reasonable survival and LVOT outcome with three quarters of the patients having an anatomically aligned LVOT. The coronary anatomy pattern was the primary determinant in the decision-making between the Nikaidoh procedure and the Rastelli operation.

Pressure gradient is used for timing of balloon aortic valvuloplasty for aortic stenosis (AS) in children, but does not correlate well with outcome and is limited if ventricular function is poor. In adults, effective orifice area (EOA) is used to assess AS severity, but EOA by continuity equation or 2D echo is unreliable in children. Three-dimensional echocardiography (3DE) may reliably assess EOA but has not been studied in children. We assessed measurement of aortic valve EOA by 3DE in children with AS before and after balloon aortic valvuloplasty and compared results with change in aortic valve gradient.3DE was performed at time of catheterization before and after balloon aortic valvuloplasty. Using 3DE multiplanar review mode, valve annulus diameter, area, and EOA were measured and compared with change in aortic gradient and degree of aortic insufficiency.Twenty-four 3DE studies in 12 children (mean age 4.4 ± 5.0 years) were analyzed. EOA was measurable in all. Catheter peak gradient decreased from 45 ± 10 to 26 ± 17 mmHg (P = 0.0018). 3DE EOA increased after balloon aortic valvuloplasty (0.59 ± 0.52 cm(2) vs 0.80 ± 0.70 cm(2) ; P = 0.03), without change in valve diameter. EOA change correlated with change in peak (r = 0.77; P = 0.005) and mean (r = 0.60; P = 0.03) aortic valve gradient post balloon aortic valvuloplasty.3DE facilitates EOA measurement in pediatric AS and correlates with change in aortic valve gradient after balloon valvuloplasty.

Introduction Better data regarding the incidence and risk factors for sudden cardiac death (SCD) in children with cardiomyopathy (CM) is critical in defining appropriate primary prevention strategies. Methods The National Australian Childhood Cardiomyopathy Study is a prospective cohort study, including all children in Australia with primary CM diagnosed at 0 – 10 years of age, between 1987–1997. SCD was defined as sudden and unexpected death in children who were not hospitalized and not in congestive heart failure at the time of death. Nine subjects with sudden death as presenting symptom were excluded. Indexed echocardiographic measurements at latest follow-up were compared between subjects with SCD and survivors. Results Study criteria were met by 291 children. Mean duration of follow-up was 9.2 years. The incidence of sudden death relative to each CM type, for all cases and as a proportion of deaths, is shown in the Table : Incidence of SCD by CM type. SCD incidence was significantly associated with CM type, for all cases ( p = 0.006) and when only those subjects who died were considered ( p = 0.005), with LVNC and RCM having up to 4 times the risk of other CM types. Children with familial DCM had a significantly higher rate of SCD than subjects with non-familial CM (12% vs 3%; p = 0.028), however, familial CM was not a risk factor in other CM types. DCM SCD subjects had larger LVEDd Z score than survivors (median 5.53 vs 1.16; p p = 0.0025). HCM SCD subjects had thicker LVPW dimension Z scores than survivors (median 4.63 vs 1.18; p = 0.007). Twelve subjects (2 DCM, 8 HCM and 2 LVNC) underwent ICD implantation (8/12 for primary prevention). Conclusions: This population based study defines new risk factors for sudden death in children with CM. RCM is well known to have a high incidence of SCD. In addition, children with LVNC and those with DCM who have severe dilatation, systolic dysfunction or familial DCM are at increased risk of sudden death.