Cardiac Rhabdomyomas Presenting with Critical Cardiac Obstruction in Neonates and Infants: Treatment Strategies and Outcome, A Single-Center Experience.

Cardiac rhabdomyomas are the most common benign pediatric heart tumor in infancy, which are commonly associated with tuberous sclerosis complex (TSC). Most rhabdomyomas are asymptomatic and spontaneously regress over time. However, some cases especially in neonates or small infants can present with hemodynamic instability.

Proteomic analysis of extracellular vesicle cargoes mirror the cardioprotective effects of rivaroxaban in patients with venous thromboembolism.

Background: Venous thromboembolism (VTE) remains a significant cause of morbidity and mortality worldwide. Rivaroxaban, a direct oral factor Xa inhibitor, mediates anti-inflammatory and cardiovascular-protective effects besides its well-established anticoagulant properties; yet, these remain poorly characterized. Extracellular vesicles (EVs) are considered proinflammatory messengers regulating a myriad of (patho)physiological processes and may be highly relevant to the pathophysiology of VTE.

Using Ribonucleoprotein-based CRISPR/Cas9 to Edit Single Nucleotide on Human Induced Pluripotent Stem Cells to Model Type 3 Long QT Syndrome (SCN5A).

Human induced pluripotent stem cells (hiPSCs) have been widely used in cardiac disease modelling, drug discovery, and regenerative medicine as they can be differentiated into patient-specific cardiomyocytes. Long QT syndrome type 3 (LQT3) is one of the more malignant congenital long QT syndrome (LQTS) variants with an SCN5A gain-of-function effect on the gated sodium channel. Moreover, the predominant pathogenic variants in LQTS genes are single nucleotide substitutions (missense) and small insertion/deletions (INDEL).

Octreotide for Acquired Chylothorax in Pediatric Patients Post-Cardiothoracic Surgery for Congenital Heart Disease: A Systematic Review.

Chylothorax is a life-threatening complication post-corrective congenital heart surgery. Octreotide is used for treatment of refractory chylothoraces, with no standardized treatment protocol and a paucity of literature describing its efficacy. Our aim was to provide an update on the safety and efficacy of octreotide for the treatment of refractory chylothoraces in neonatal and pediatric patients' post-corrective congenital heart surgery.

Severe Restriction of a VSD and Development of Pulmonary Atresia in a Patient with Transposition of the Great Arteries: Fetal Diagnosis.

Pulmonary atresia with an intact ventricular septum typically occurs in patients with concordant atrioventricular and ventriculoarterial connections. When it does occur in patients with discordant connections, it is most frequently seen in association with congenitally corrected transposition. We present a rare case of transposition of the great arteries with a ventricular septal defect (VSD) detected in fetal life which evolved throughout pregnancy resulting in the development of pulmonary atresia and severe restriction of the VSD.

Planning Transition of Care for Adolescents Affected by Congenital Heart Disease: The Irish National Pathway.

At some point in their life, adolescent patients with a congenital heart disease (CHD) transition from paediatric services to adult care facilities. The process is not without any risks, as it is often linked with a significantly progressive deterioration in adolescents' health and loss of follow-up. In fact, transition patients often encounter troubles in finding a care giver who is comfortable managing their condition, or in re-establishing trust with the new care provider.

Improving the Quality of Paediatric ECG Interpretation.

Aims Our aim was to complete an audit loop and identify whether implementing a paediatric ECG checklist improved the documentation and therefore the quality of paediatric ECG interpretation. We designed a paediatric ECG and education proforma in a Paediatric Emergency Department and incorporated it into daily practice. Methods We audited the medical records of children presenting with clinical indications for ECG.

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.

Rockwood Clinical Frailty Scale as a predictor of adverse outcomes among older adults undergoing aortic valve replacement: a protocol for a systematic review.

Introduction: Frailty is associated with adverse outcomes relating to cardiac procedures. It has been proposed that frailty scoring should be included in the preoperative assessment of patients undergoing aortic valve replacement. We aim to examine the Rockwood Clinical Frailty Scale (CFS), as a predictor of adverse outcomes following aortic valve replacement.

Generation and characterization of three induced pluripotent stem cell lines (NUIGi046-A, NUIGi046-B, NUIGi046-C) from a 51-year-old healthy individual.

Skin punch biopsy was donated by a healthy 51-year-old Caucasian male and the dermal fibroblasts were reprogrammed into human induced pluripotent stem cell (hiPSC) lines by using non-integrative Sendai viruses expressing OCT4, SOX2, KLF4 and c-MYC. Three iPSC lines (NUIGi046-A, NUIGi046-B, NUIGi046-C) highly expressed the pluripotent markers and were capable of differentiating into cells of endodermal, mesodermal, and ectodermal origin. These iPSCs can be offered as controls and in combination with genome-editing and three-dimensional (3D) system.

Derivation and characterization of two human induced pluripotent stem cell lines (NUIGi004-A) and (NUIGi012-A) from two patients with LQT2 disease.

Long QT syndrome type 2 (LQT2) is associated with KCNH2, which encodes the α subunit of the ion channel that controls the K current in the heart. Mutations of KCNH2 cause loss of Kv11.1 channel function by disrupting subunit folding, assembly, or trafficking of the channel to the cell surface.

Nonvalvular atrial fibrillation patients anticoagulated with rivaroxaban compared with warfarin exhibit reduced circulating extracellular vesicles with attenuated pro-inflammatory protein signatures.

Background: Rivaroxaban, a direct oral factor Xa inhibitor, mediates anti-inflammatory and cardiovascular-protective effects besides its well-established anticoagulant properties; however, these remain poorly characterized. Extracellular vesicles (EVs) are important circulating messengers regulating a myriad of biological and pathological processes and may be highly relevant to the pathophysiology of atrial fibrillation as they reflect alterations in platelet and endothelial biology. However, the effects of rivaroxaban on circulating pro-inflammatory EVs remain unknown.

Use of Flecainide in Stable Coronary Artery Disease: An Analysis of Its Safety in Both Nonobstructive and Obstructive Coronary Artery Disease.

Background: Flecainide is a class IC antiarrhythmic drug that is contraindicated in patients who have a history of myocardial infarction, but its effect on mortality and risk of proarrhythmia in patients with stable obstructive and nonobstructive epicardial coronary artery disease (CAD) has not been assessed.

Objective: We sought to compare the safety of flecainide administration in patients who had angiographic evidence of either no or minimal CAD versus nonobstructive CAD, and those who underwent nuclear stress testing with perfusion defects versus those without perfusion defects.

Methods: We conducted a retrospective chart review of 348 patients who were treated with flecainide for at least 1 year duration and underwent evaluation for CAD with coronary angiography or myocardial perfusion imaging (MPI) stress testing within 3 months of initiating flecainide.

Generation and characterization of three induced pluripotent stem cell lines (NUIGi047-A, NUIGi047-B, NUIGi047-C) from a 7-year-old healthy Caucasian male.

We report the generation of three human induced pluripotent stem cell (hiPSC) lines (NUIGi047-A, NUIGi047-B, NUIGi047-C) from a healthy 7-year-old boy using non-integrational Sendai re-programming method expressing OCT4, SOX2, KLF4 and C-MYC. Stem cell characterization was confirmed through morphology, immunofluorescence staining and RT-qPCR. Differentiation potential in vitro was demonstrated to all three germ layers with STR lineage verification and normal molecular karyotyping through the process of re-programming.

The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy.

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.

Safety of Conscious Sedation for Transcatheter Aortic Valve Replacement Without an Anesthetist.

Objectives: To compare the safety of performing transfemoral transcatheter aortic valve replacement (TAVR) under conscious sedation without an anesthetist present (TAVR-NA) vs TAVR performed with an anesthetist supervising sedation (TAVR-A).

Background: In almost all United States and European centers, TAVR-A represents the standard of care. There are limited data on the safety of TAVR-NA.