Characterising the lipid profile of paediatric patients with anomalous aortic origins of a coronary artery.

Background: Lipid levels in paediatric patients with anomalous aortic origin of a coronary artery (AAOCA) have not previously been explored. Patients with CHD have an increased risk of atherosclerotic cardiovascular disease later in life compared to the general population. We aim to characterise the lipid profiles in paediatric patients with AAOCA and explore its relation to diagnosis, race/ethnicity, and exercise.

Mitral Annular Disjunction Associated with Ventricular Dilation in Pediatric Marfan Syndrome: A Cardiovascular Magnetic Resonance Study.

Background: Mitral annular disjunction (MAD) has increasingly been recognized as a marker for adverse cardiovascular events in Marfan syndrome (MFS). As recent adult data links MFS with left ventricular (LV) dilation and reduced ejection fraction (LVEF), we hypothesized that MAD may be associated with LV dilation in pediatric MFS patients.

Methods: A retrospective analysis was performed among MFS patients < 19 years old at initial cardiac MRI (CMR).

Perceptions of exercise behavior and well-being in anomalous aortic origin of coronary arteries.

Unlabelled: Anomalous aortic origin of a coronary artery (AAOCA) is associated with sudden death in the young. Risk stratification and management decision-making remain challenging. Data addressing post-diagnosis perceptions of exercise behavior and safety are lacking.

Wall motion assessment by feature tracking in pediatric patients with coronary anomalies undergoing dobutamine stress CMR.

Background: Left ventricular (LV) wall motion assessment is an important adjunct in addition to perfusion defects in assessing ischemic changes. This study aims to investigate the feasibility and utility of performing feature tracking (FT) in pediatric patients with coronary anomalies undergoing dobutamine stress CMR to assess wall motion abnormalities (WMA) and perfusion defects.

Method: This is a retrospective study where 10 patients with an inducible first-pass perfusion (FPP) defect and 10 without were selected.

Evidence of cardiomyopathy associated with Marfan syndrome in children.

Objective: Marfan syndrome (MFS)-associated cardiomyopathy, defined as ventricular dilation and dysfunction unexplained by volume loading, is not well defined in children. This study evaluated ventricular size and function in paediatric MFS using cardiac MRI (cMRI).

Methods: This retrospective cohort study examined patients with MFS <19 years old at first cMRI.

Managing Bilateral Discontinuous Pulmonary Arteries of Ductal Origin in Single-Ventricle Anatomy.

Background: We describe outcomes and management strategies for single-ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus.

Methods: We reviewed 22 patients with aforementioned anatomy and PA centralization from 1995 to 2023, excluding those with biventricular repair.

Results: Median age at centralization was 9 days (minimum-maximum, 0 days-2 years).

Mitral annular disjunction and its progression during childhood in Marfan syndrome.

Aims: Data on mitral annular disjunction (MAD) in children with Marfan syndrome (MFS) are sparse. To investigate the diagnostic yield of MAD by echocardiography and cardiac magnetic resonance imaging (CMR), its prevalence and progression during childhood.

Methods And Results: We included patients <21 years old with MFS, defined by 2010 Ghent criteria and a pathogenic FBN1 variant or ectopia lentis.

Long-Term Outcomes of Ascending Sliding Arch Aortoplasty.

Coarctation of the aorta can be associated with significant hypoplasia of the aortic arch. In contrast to patch aortoplasty, ascending sliding arch aortoplasty uses viable autologous tissue for potential growth in children. We reviewed the mid- to long-term outcomes of this technique.

Labyrinthine cor triatriatum sinister in fetal hypoplastic left heart syndrome is associated with poor outcomes.

Objectives: A subset of hypoplastic-left-heart-syndrome (HLHS) fetuses have a complex cor-triatriatum sinister that we named "labyrinthine-cor (L-cor)". We sought to determine the prevalence of L-cor in HLHS fetuses and hypothesized that it is associated with increased mortality.

Methods: This single-center retrospective cohort study included all HLHS fetuses from January 2010-December 2020.

Creating a flow restrictor in ductal stenting: a novel technique.

Ductal stenting has transformed the care of neonates with ductal-dependent critical CHD, especially in low-income countries. In small infants, a 3.5- or 4-mm stent may lead to too much pulmonary blood flow resulting in pulmonary oedema.

Comparing palliation strategies for single-ventricle anatomy with transposed great arteries and systemic outflow obstruction.

Objective: Patients with complex single-ventricle anatomy with transposed great arteries and systemic outflow obstruction (SV-TGA-SOO) undergo varied initial palliation with ultimate goal of Fontan circulation. We examine a longitudinal experience with multiple techniques, including the largest published cohort following palliative arterial switch operation (pASO), to describe outcomes and decision-making factors.

Methods: Neonates with SV-TGA-SOO who underwent initial surgical palliation from 1995 to 2022 at a single institution were retrospectively reviewed.

Abnormal Left Ventricular Strain Correlates with Left Ventricular Dysfunction but not Aortic Pathology in Marfan Syndrome in Children.

Cardiomyopathy is a complication in adults with Marfan syndrome (MFS). Early recognition of MFS patients at high risk of cardiomyopathy could impact monitoring and treatment. Abnormal ventricular strain has been associated with impaired ventricular function among adults with MFS but remains understudied in children.

Perforated posterior mitral valve leaflet in mitral annular disjunction and infective endocarditis.

We herein presented a 17-year-old female with history of mild mitral valve prolapse who was admitted for methicillin-sensitive endocarditis and diagnosed with mitral annular disjunction and perforated posterior mitral valve leaflet on two-dimensional and three-dimensional echocardiography (P-P). A perforation in the posterior leaflet was confirmed and repaired during surgical intervention. This is a rare presentation of leaflet perforation in the area of mitral annular disjunction.

Imaging and guiding intervention for tricuspid valve disorders using 3-dimensional transesophageal echocardiography in pediatric and congenital heart disease.

In the pediatric and congenital heart disease (CHD) population, tricuspid valve (TV) disorders are complex due to the variable TV morphology, its sophisticated interaction with the right ventricle as well as associated congenital and acquired lesions. While surgery is the standard of care for TV dysfunction in this patient population, transcatheter treatment for bioprosthetic TV dysfunction has been performed successfully. Detailed and accurate anatomic assessment of the abnormal TV is essential in the preoperative/preprocedural planning.

Clinical Presentation and Medium-Term Outcomes of Children With Anomalous Aortic Origin of the Left Coronary Artery: High-Risk Features Beyond Interarterial Course.

Background: Anomalous aortic origin of the left coronary artery (AAOLCA) confers a rare, but significant, risk of sudden cardiac death in children. Surgery is recommended for interarterial AAOLCA, and other subtypes considered benign. We aimed to determine the clinical characteristics and outcomes of 3 AAOLCA subtypes.

Ischemia in Anomalous Aortic Origin of a Right Coronary Artery: Large Pediatric Cohort Medium-Term Outcomes.

Background: Anomalous aortic origin of a right coronary artery may cause myocardial ischemia and sudden death in the young. Data on myocardial ischemia or longitudinal outcomes are sparse in pediatric anomalous aortic origin of a right coronary artery population.

Methods: Patients <21 years with anomalous aortic origin of a right coronary artery were prospectively enrolled.