2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients.

In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences.

Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11).

Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code () is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11).

Characteristics of Interstage Death After Discharge from Stage I Palliation.

Background: Interstage mortality (IM) remains high for patients with single-ventricle congenital heart disease (SVCHD) in the period between Stage 1 Palliation (S1P) and Glenn operation. We sought to characterize IM.

Methods: This was a descriptive analysis of 2184 patients with SVCHD discharged home after S1P from 60 National Pediatric Cardiology Quality Improvement Collaborative sites between 2008 and 2015.

Belhassen Ventricular Tachycardia in a Child.

First reported in 1981, idiopathic left ventricular tachycardia (VT) of the Belhassen type is characterized during electrocardiography (ECG) by a right bundle branch pattern and left axis deviation. We report the case of a 15-year-old Hispanic male who, during a routine evaluation ECG to support sports participation, was found to have nonsustained monomorphic VT. Prior to his exercise treadmill test, his physical examination and echocardiogram were normal.

Aortic root aneurysm repair in a neonate with Loeys-Dietz syndrome.

Loeys-Dietz syndrome is a connective tissue disorder known to cause aggressive aortopathy in paediatric patients, but it is extremely rare for cardiovascular events to present during infancy. We report the first successful aortic repair in a neonate with LDS presenting in extremis with an early onset, massive aortic aneurysm.

Covid-19 in an infant with systemic to pulmonary artery shunt dependent functionally univentricular physiology.

We report a case of two-month old with a functionally univentricular heart and parallel circulation who presented to the emergency department with Covid-19 and subsequently developed acute respiratory distress syndrome. The course of illness, clinical values, and laboratory markers are characterized in this report.

Pulmonary hypertension and mitral regurgitation in an infant with an anatomically normal mitral valve.

A full-term, female presented on her date of birth with severe pulmonary hypertension (PH) and mitral regurgitation (MR), requiring veno-arterial extracorporeal membrane oxygenation. After the treatment, her PH and MR were resolved with no anatomic abnormality present. We propose a positive feedback loop of PH causing right ventricular dilation and interventricular septal shifts, worsening MR, and elevated left atrial, and potentially pulmonary, pressures.

Effects of Vasopressin Infusion After Pediatric Cardiac Surgery: A Meta-analysis.

Vasopressin has been used to augment blood pressure; however, cardiovascular effects after cardiac surgery have not been well established. The primary objective of this study was to survey the current literature and quantify the pooled effect of vasopressin on hemodynamic parameters in children after pediatric cardiac surgery. A systematic review was conducted to identify studies characterizing the hemodynamic effects of vasopressin after pediatric cardiac surgery.

Congenitally Abnormal Aortic Valve Causing Coronary Obstruction and Cardiac Arrest in Infancy.

Many instances of coronary artery anomalies are documented in the literature; however, the detection and treatment of an asymmetric, large aortic cusp causing obstruction of a coronary ostium in a symptomatic infant remains unreported. We present a case of a 2-month-old infant with an enlarged right coronary cusp obstructing the left coronary ostium, requiring emergent repair by relocating the left coronary button and reconstructing the sinus of Valsalva with autologous pericardium. This procedure preserved native aortic valve function, and the child remains asymptomatic months after discharge.

The quality of general movements in infants with complex congenital heart disease undergoing surgery in the neonatal period.

Background: Advances in diagnostic technologies, surgical management, and perioperative care have increased survival for neonates with complex congenital heart disease (CCHD). The success of these advances exposed a heightened risk of brain injury and developmental disabilities. The General Movements Assessment, a non-invasive method, may detect early neurodevelopmental impairments in high-risk infants.

Pulmonary Cusp Positioning of a Right Ventricular Outflow Tract Ventricular Tachycardia in a Pediatric Patient Identified Using Intracardiac Echocardiography.

Ventricular premature beats originating from the right ventricular outflow tract can have myocardial extensions to the pulmonary valve and pulmonary artery. Treatment may consist of catheter ablation combined with the use of three-dimensional mapping to determine the exact location of ectopy. The location of ectopy relative to the pulmonary valve may be hard to ascertain.

Loss of ventricular preexcitation during noninvasive testing does not exclude high-risk accessory pathways: A multicenter study of WPW in children.

Background: Abrupt loss of ventricular preexcitation on noninvasive evaluation, or nonpersistent preexcitation, in Wolff-Parkinson-White syndrome (WPW) is thought to indicate a low risk of life-threatening events.

Objective: The purpose of this study was to compare accessory pathway (AP) characteristics and occurrences of sudden cardiac arrest (SCA) and rapidly conducted preexcited atrial fibrillation (RC-AF) in patients with nonpersistent and persistent preexcitation.

Methods: Patients 21 years or younger with WPW and invasive electrophysiology study (EPS) data, SCA, or RC-AF were identified from multicenter databases.

Developmental outcomes after early surgery for complex congenital heart disease: a systematic review and meta-analysis.

Aim: (1) To systematically review the literature on developmental outcomes from infancy to adolescence of children with complex congenital heart disease (CHD) who underwent early surgery; (2) to run a meta-regression analysis on the Bayley Scales of Infant Development, Second Edition Mental Developmental Index and Psychomotor Developmental Index (PDI) of infants up to 24 months and IQs of preschool-aged children to adolescents; (3) to assess associations between perioperative risk factors and outcomes.

Method: We searched pertinent literature (January 1990 to January 2019) in PubMed, Embase, CINAHL, and PsycINFO. Selection criteria included infants with complex CHD who had primary surgery within the first 9 weeks of life.

The effect of milrinone on hemodynamic and gas exchange parameters in children.

Milrinone is a drug frequently used for hemodynamic support in children during critical illness. Although the hemodynamic changes induced by milrinone in children may appear similar to those of adults, the physiologic contributors of these changes remain vastly unknown. A systematic review was conducted to identify studies characterising the hemodynamic effects of milrinone in children during critical illness for hemodynamic support for various medical conditions.

Noninvasive Neurally Adjusted Ventilatory Assist Versus High Flow Cannula Support After Congenital Heart Surgery.

Background: Extubation failure rates for critical patients in pediatric intensive care units (ICUs) range from 5% to 29%. Noninvasive (NIV) ventilation has been shown to decrease extubation failure. We compared reintubation rates and outcomes of patients supported with NIV neurally adjusted ventilation assist (NAVA) versus historical controls supported with high-flow nasal cannula (HFNC).

Repair of Superior Sinus Venosus Atrial Septal Defect Using a Modified Two-Patch Technique.

Background: Surgical repair of superior sinus venosus atrial septal defect associated with partial anomalous pulmonary venous connection is usually performed with either a two-patch technique or the Warden procedure. However, both techniques can lead to superior vena cava or pulmonary vein stenosis, as well as sinus node dysfunction. To prevent these complications, we have utilized a modified two-patch technique.

Bleeding and Thrombosis With Pediatric Extracorporeal Life Support: A Roadmap for Management, Research, and the Future From the Pediatric Cardiac Intensive Care Society: Part 1.

Objectives: To make practical and evidence-based recommendations on improving understanding of bleeding and thrombosis with pediatric extracorporeal life support and to make recommendations for research directions.

Data Sources: Evaluation of literature and consensus conferences of pediatric critical care and extracorporeal life support experts.

Study Selection: A team of 10 experts with pediatric cardiac and extracorporeal membrane oxygenation experience and expertise met through the Pediatric Cardiac Intensive Care Society to review current knowledge and make recommendations for future research to establish "best practice" for anticoagulation management related to extracorporeal life support.

Saline Contrast Transesophageal Echocardiography in Fontan Patients: Assessment of the Presence, Type, and Size of Right to Left Shunts.

Right to left (R-L) shunts resulting in cyanosis or systemic embolization occur after the Fontan procedure. The primary modality of diagnosing these is angiography. Successful delineation of these shunts in Fontan patients using selective saline contrast transesophageal echocardiography (SCTEE) may allow for reduced radiation and contrast exposure.

Chronotropic incompetence in paediatric heart transplant recipients with prior congenital heart disease.

Background: Cardiopulmonary exercise testing has been used to measure functional capacity in children who have undergone a heart transplant. Cardiopulmonary exercise testing results have not been compared between children transplanted for a primary diagnosis of CHD and those with a primary diagnosis of cardiomyopathy despite differences in outcomes. This study is aimed to compare cardiopulmonary exercise testing performance between these two groups.

Peritoneal Dialysis Vs Diuretics in Children After Congenital Heart Surgery.

Background: This study sought to evaluate outcomes of patients undergoing congenital heart surgery who underwent peritoneal dialysis (PD) vs a diuretic regimen.

Methods: This study conducted a comprehensive search in Medline, EMBASE, Scopus, the Cochrane Central Register of Controlled Trials, and the Cochrane Database of Systematic Reviews from the databases' inception through April 24, 2018. Independent reviewers selected studies and extracted data.

Assessing the criteria for definition of perimembranous ventricular septal defects in light of the search for consensus.

Background: Discussions continue as to whether ventricular septal defects are best categorized according to their right ventricular geography or their borders. This is especially true when considering the perimembranous defect. Our aim, therefore, was to establish the phenotypic feature of the perimembranous defect, and to establish the ease of distinguishing its geographical variants.