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.

Impact of liver fibrosis and nodules formation on hemodynamics in young adults after total cavopulmonary connection. A magnetic resonance study.

Background: The aim of this study was to analyze the relation between the hepatic fibrosis markers, liver morphology and hemodynamics assessed by magnetic resonance imaging (MRI) after total cavopulmonary connection (TCPC).

Materials And Methods: Adult patients after TCPC performed in childhood between 1993 and 2003 are the subjects of this observational study. The follow-up protocol consisted of clinical and echocardiographic examination, liver elastography, cardiopulmonary exercise test, MRI hemodynamics and liver morphology assessment and direct enhanced liver fibrosis (ELF) test.

Identification and in-silico characterization of splice-site variants from a large cardiogenetic national registry.

Splice-site variants in cardiac genes may predispose carriers to potentially lethal arrhythmias. To investigate, we screened 1315 probands and first-degree relatives enrolled in the Canadian Hearts in Rhythm Organization (HiRO) registry. 10% (134/1315) of patients in the HiRO registry carry variants within 10 base-pairs of the intron-exon boundary with 78% (104/134) otherwise genotype negative.

Angiotensin receptor blockers and β blockers in Marfan syndrome: an individual patient data meta-analysis of randomised trials.

Background: Angiotensin receptor blockers (ARBs) and β blockers are widely used in the treatment of Marfan syndrome to try to reduce the rate of progressive aortic root enlargement characteristic of this condition, but their separate and joint effects are uncertain. We aimed to determine these effects in a collaborative individual patient data meta-analysis of randomised trials of these treatments.

Methods: In this meta-analysis, we identified relevant trials of patients with Marfan syndrome by systematically searching MEDLINE, Embase, and CENTRAL from database inception to Nov 2, 2021.

Clinical Question Influence on Radiation Dose of Cardiac CT Scan in Children.

Background: To assess the impact of different clinical questions on radiation doses acquired during cardiac computed tomography in children. Methods: A total of 116 children who underwent cardiac CT on a third-generation dual-source CT scanner were included. The clinical questions were divided into three main categories: the extent of scanning in the z-axis, coronary artery assessment and cardiac function assessment.

Impact and Modifiers of Ventricular Pacing in Patients With Single Ventricle Circulation.

Background: Palliation of the single ventricle (SV) circulation is associated with a burden of lifelong complications. Previous studies have identified that the need for a permanent ventricular pacing system (PPM) may be associated with additional adverse long-term outcomes.

Objectives: The goal of this study was to quantify the attributable risk of PPM in patients with SV, and to identify modifiable risk factors.

The Detection, Evaluation, and Management of Dyslipidemia in Children and Adolescents: A Canadian Cardiovascular Society/Canadian Pediatric Cardiology Association Clinical Practice Update.

Atherosclerosis begins in youth and is directly linked with the presence and severity of cardiovascular risk factors, including dyslipidemia. Thus, the timely identification and management of dyslipidemia in childhood might slow atherosclerotic progression and decrease the risk of cardiovascular disease in adulthood. This is particularly true for children with genetic disorders resulting in marked dyslipidemia, including familial hypercholesterolemia, which remains frequently undiagnosed.

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.

The Critical Transfer From Paediatrics to Adult Care in Patients With Congenital Heart Disease: Predictors of Transfer and Retention of Care.

Background: Congenital heart disease is the most common congenital birth defect and presents with differing degrees of complexity. Patients require lifelong specialized care. The transfer from paediatric to adult care is a time of risk that may result in lapses or loss of care.

Repair of a remote pericommissural anomalous left coronary artery from the pulmonary artery.

We demonstrate a classic trapdoor technique to correct a late-presenting remote pericommissural anomalous left coronary artery from the pulmonary artery. The merits of the technique compared to alternative techniques are explained. The impact of late diagnosis on left ventricular function as well as on papillary muscle ischemia-induced mitral regurgitation is evident.

Burden of sudden cardiac death in persons aged 1-40 years in the Czech Republic.

Objectives: The aim of the study was to ascertain the incidence, circumstances and causes of sudden cardiac death in persons aged 1-40 years in the Czech Republic.

Methods: De-identified autopsy reports of all individuals who died suddenly between the ages of 1-40 years during the period 2014-2019 inclusive in a selected area of the Czech Republic were analysed retrospectively. Persons with substantial cardiovascular pathology defined by histopathological criteria and those with a negative autopsy were included in the study.

Echocardiographic scores for biventricular repair risk prediction of congenital heart disease with borderline left ventricle: a review.

The aim of this review is to highlight the strengths and limitations of major echocardiographic biventricular repair (BVR) prediction models for borderline left ventricle (LV) in complex congenital heart disease (CHD). A systematic search in the National Library of Medicine for Medical Subject Headings and free text terms including echocardiography, CHD, and scores, was performed. The search was refined by adding keywords for critical aortic stenosis (AS), borderline LV, complex left ventricular outflow tract (LVOT) obstruction, hypoplastic left heart syndrome/complex (HLHS/HLHC), and unbalanced atrio-ventricular septal defects (uAVSD).

Paediatric and adult congenital cardiology education and training in Europe.

Background: Limited data exist on training of European paediatric and adult congenital cardiologists.

Methods: A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries.

Results: Delegates from 30 countries (91%) responded.

Physical Activity in Paediatric Long QT Syndrome Patients.

Background: Physical activity (PA) is important for cardiovascular health as well as social and emotional well-being of children. Patients with long QT syndrome (LQTS) often face PA restrictions and are often prescribed beta-blockers for disease management. The aim of this study was to determine if PA levels were lower in patients with LQTS compared with healthy controls.

A pharmacogenomic investigation of the cardiac safety profile of ondansetron in children and pregnant women.

Background: Ondansetron is a highly effective antiemetic for the treatment of nausea and vomiting. However, this medication has also been associated with QT prolongation. Pharmacogenomic information on therapeutic response to ondansetron exists, but no investigation has been performed on genetic factors that influence the cardiac safety of this medication.

Cardiac Resynchronization Therapy Using Single Site Left Ventricular Pacing in a Tricuspid Atresia Patient With Left Bundle Branch Block.

Cardiac resynchronization therapy (CRT) is typically achieved by pacing both ventricles. However, left ventricular-only pacing has been shown to be noninferior to biventricular pacing in patients with left bundle branch block and normal atrioventricular conduction. However, there is no evidence in favour of CRT with single-site pacing for patients with single-ventricle physiology.

Massive Saddle Pulmonary Embolism in a Preterm Neonate With Successful Emergent Open Embolectomy.

Pulmonary embolism in the neonate is a rare, life-threatening emergency. Risk factors for neonatal pulmonary embolism (PE) include sepsis, asphyxia, prematurity, and vascular catheterisation. We report the case of a preterm neonate with a massive saddle pulmonary thrombosis of unidentified etiology.

Does COVID-19 pose a threat for patients after univentricular palliation? Thrombosis of the Fontan tunnel.

A 6-year-old boy, born with hypoplastic left heart syndrome, underwent total cavopulmonary connection and later presented in a significantly deteriorated condition. A CT scan revealed multiple thrombi in the extracardiac conduit, although the patient was maintained on an effective anticoagulant therapy. Further examination revealed anamnestic antibodies suggesting that the patient had gone through a clinically inapparent COVID-19 infection, which we conclude most likely contributed to his hypercoagulable state and led to the formation of significant thrombi impairing the patient's haemodynamics.

The aorto-left ventricular tunnel from a fetal perspective: Original case series and literature review.

Introduction: Aorto-left ventricular tunnel (ALVT) accounts for <0.1% of congenital heart defects. Evidence on the prognosis from a fetal perspective is limited.

Training and mentoring of the congenital heart surgeon for India.

Congenital heart surgery is one of the most demanding subspecialities in surgery. To become a competent surgeon, a lot of investment of time, in-depth study, training under a committed mentor, acquisition of the necessary fine surgical skills, and development of a three-dimensional appreciation of corrections are needed to be cultivated. These make it not only a speciality of skills, but also a cerebral speciality.

Exercise capacity after total cavopulmonary anastomosis: a longitudinal paediatric and adult study.

Aims: Fontan palliation is a surgical strategy for patients with complex congenital heart disease, in whom biventricular circulation cannot be achieved. Long-term survival is negatively affected by the absence of sub-pulmonary ventricle and increased systemic venous pressure. Exercise capacity is a known predictor of overall survival and quality of life in congenital heart defects.

An International Multicenter Cohort Study on β-Blockers for the Treatment of Symptomatic Children With Catecholaminergic Polymorphic Ventricular Tachycardia.

Background: Symptomatic children with catecholaminergic polymorphic ventricular tachycardia (CPVT) are at risk for recurrent arrhythmic events. β-Blockers decrease this risk, but studies comparing individual β-blockers in sizeable cohorts are lacking. We aimed to assess the association between risk for arrhythmic events and type of β-blocker in a large cohort of symptomatic children with CPVT.