Sex Differences in Children and Adolescents With Hypertrophic Cardiomyopathy.

Background: Sex differences have been described in adults with hypertrophic cardiomyopathy (HCM), but it is unknown if similar differences exist in childhood-onset disease.

Objectives: This study aimed to investigate the influence of biological sex on the clinical characteristics and outcomes of children with HCM.

Methods: An international retrospective cohort of patients diagnosed with nonsyndromic HCM ≤16 years was formed.

Management of aortic disease in children with FBN1-related Marfan syndrome.

Marfan syndrome (MFS) is a hereditary connective tissue disorder with an estimated prevalence of 1:5000-1:10 000 individuals. It is a pleiotropic disease characterized by specific ocular, cardiovascular, and skeletal features. The most common cardiovascular complication is aortic root dilatation which untreated can lead to life-threatening aortic root dissection, mainly occurring in adult patients.

Double aortic arch: a comparison of fetal cardiovascular magnetic resonance, postnatal computed tomography and surgical findings.

Background: In double aortic arch (DAA), one of the arches can demonstrate atretic portions postnatally, leading to diagnostic uncertainty due to overlap with isolated right aortic arch (RAA) variants. The main objective of this study is to demonstrate the morphological evolution of different DAA phenotypes from prenatal to postnatal life using three-dimensional (3D) fetal cardiac magnetic resonance (CMR) imaging and postnatal computed tomography (CT)/CMR imaging.

Methods: Three-dimensional fetal CMR was undertaken in fetuses with suspected DAA over a 6-year period (January 2016-January 2022).

Early superior cavopulmonary connection with preoperative computer tomography shows comparable outcomes for infants following Norwood palliation.

Objectives: There is increasing evidence that performing superior cavopulmonary connection (SCPC) at 3 months reduces mortality: reducing the risky 'interstage' period, volume off-loading the ventricle and reducing coronary steal, hopefully preserving ventricular function. Our objective was to describe our experience of early SCPC with preoperative computer tomography (CT) assessment compared to later connection at 6 months.

Methods: Patients undergoing Norwood procedure from 2005 to 2020 were divided into 2 eras were described and compared.

Relationship Between Maximal Left Ventricular Wall Thickness and Sudden Cardiac Death in Childhood Onset Hypertrophic Cardiomyopathy.

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.

Cardiac Function After Cardiotoxic Treatments for Childhood Cancer-Left Ventricular Longitudinal Strain in Screening.

The majority of childhood cancer survivors (CCSs) have been exposed to cardiotoxic treatments and often present with modifiable cardiovascular risk factors. Our aim was to evaluate the value of left ventricular (LV) longitudinal strain for increasing the sensitivity of cardiac dysfunction detection among CCSs. We combined two national cohorts: neuroblastoma and other childhood cancer survivors treated with anthracyclines.

Clinical presentation and long-term outcomes of infantile hypertrophic cardiomyopathy: a European multicentre study.

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.

Dilated ascending aorta in the fetus.

Introduction: Prenatal recognition of dilated aortic root is extremely rare and there are significant challenges in counselling these patients. The primary aim of this case series is to describe the prevalence, associations and outcome of dilated ascending aorta diagnosed during fetal life.

Methods: This is a retrospective cohort study from two tertiary fetal cardiology centres.

Clinical outcomes and programming strategies of implantable cardioverter-defibrillator devices in paediatric hypertrophic cardiomyopathy: a UK National Cohort Study.

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.

Multimodality cardiac evaluation in children and young adults with multisystem inflammation associated with COVID-19.

Aims: Following the peak of the UK COVID-19 epidemic, a new multisystem inflammatory condition with significant cardiovascular effects emerged in young people. We utilized multimodality imaging to provide a detailed sequential description of the cardiac involvement.

Methods And Results: Twenty consecutive patients (mean age 10.

EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Cardiovascular MRI and CT in congenital heart disease.

Cardiovascular MRI and CT are useful imaging modalities complimentary to echocardiography. This review article describes the common indications and consideration for the use of MRI and CT in the management of congenital heart disease.

Application of the Boston Technical Performance Score to intraoperative echocardiography.

Background: The Technical Performance Score (TPS) developed by Boston Children's Hospital showed surgical outcomes correlate with adequacy of technical repair when implemented on pre-discharge echocardiograms. We applied this scoring system to intraoperative imaging in a tertiary UK congenital heart surgical centre.

Methods: After a period of training, intraoperative TPS (epicardial and/or transesophageal echocardiography) was instituted.

Development of a Novel Risk Prediction Model for Sudden Cardiac Death in Childhood Hypertrophic Cardiomyopathy (HCM Risk-Kids).

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.

A validation study of the European Society of Cardiology guidelines for risk stratification of sudden cardiac death in childhood hypertrophic cardiomyopathy.

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.

Three-dimensional visualisation of the fetal heart using prenatal MRI with motion-corrected slice-volume registration: a prospective, single-centre cohort study.

Background: Two-dimensional (2D) ultrasound echocardiography is the primary technique used to diagnose congenital heart disease before birth. There is, however, a longstanding need for a reliable form of secondary imaging, particularly in cases when more detailed three-dimensional (3D) vascular imaging is required, or when ultrasound windows are of poor diagnostic quality. Fetal MRI, which is well established for other organ systems, is highly susceptible to fetal movement, particularly for 3D imaging.

Visualization of coronary arteries in paediatric patients using whole-heart coronary magnetic resonance angiography: comparison of image-navigation and the standard approach for respiratory motion compensation.

Aims: To investigate the use of respiratory motion compensation using image-based navigation (iNAV) with constant respiratory efficiency using single end-expiratory thresholding (CRUISE) for coronary magnetic resonance angiography (CMRA), and compare it to the conventional diaphragmatic navigator (dNAV) in paediatric patients with congenital or suspected heart disease.

Methods: iNAV allowed direct tracking of the respiratory heart motion and was generated using balanced steady state free precession startup echoes. Respiratory gating was achieved using CRUISE with a fixed 50% efficiency.

Clinical presentation and survival of childhood hypertrophic cardiomyopathy: a retrospective study in United Kingdom.

Aims: Understanding the spectrum of disease, symptom burden and natural history are essential for the management of children with hypertrophic cardiomyopathy (HCM). The effect of changing screening practices over time has not previously been studied. This study describes the clinical characteristics and outcomes of childhood HCM over four decades in a well-characterized United Kingdom cohort.

Improved coronary magnetic resonance angiography using gadobenate dimeglumine in pediatric congenital heart disease.

Background: CMRA in pediatrics remains challenging due to the smaller vessel size, high heart rates (HR), potential image degradation caused by limited patient cooperation and long acquisition times. High-relaxivity contrast agents have been shown to improve coronary imaging in adults, but limited data is available in children. We sought to investigate whether gadobenate dimeglumine (Gd-BOPTA) together with self-navigated inversion-prepared coronary magnetic resonance angiography (CMRA) sequence design improves coronary image quality in pediatric patients.

Atrioventricular block: an unusual complication of Graves' disease.

We report the case of a 10-year-old girl treated with atenolol and carbimazole for tachycardia and hypertension associated with Graves' disease who developed symptomatic 2:1 heart block. 2:1 heart block resolved following cessation of atenolol, reduction in carbimazole dose and treatment of suspected tonsillitis. First-degree atrioventricular block persisted, but gradually normalised following improvement in thyroid status.

Cardiac manifestations of inherited metabolic disease in children.

Inborn errors of metabolism (IEM) are responsible for around 5% of all cases of cardiomyopathy (CM) and for 15% of non-idiopathic cases. Storage disorders such as Pompe disease (glycogen storage disease type II) typically cause hypertrophic CM, whereas the accumulation of toxic metabolites, as seen in the organic acidurias, is associated with dilated cardiomyopathy (DCM). Mixed pathology is also possible, particularly in late presentations.

Coronary artery size and origin imaging in children: a comparative study of MRI and trans-thoracic echocardiography.

Background: The purpose of this study was to see how coronary magnetic resonance angiography (CMRA) compared to echocardiography for the detection of coronary artery origins and to compare CMRA measurements for coronary dimensions in children with published echocardiographic reference values.

Methods: Enrolled patients underwent dual cardiac phase CMRA and echocardiography under the same anesthetic. Echocardiographic measurements of the right coronary artery (RCA), left anterior descending (LAD) and left main (LM) were made.