Top Papers for 2018 - Pediatric Cardiology

This review article will guide the reader through the basics of echocardiographic assessment of congenital left to right shunts in both paediatric and adult age groups. After reading this article, the reader will understand the pathology and clinical presentation of atrial septal defects (ASDs), ventricular septal defects (VSDs), atrioventricular septal defects (AVSDs) and patent arterial duct. Echocardiography is the mainstay in diagnosis and follow-up assessment of patients with congenital heart disease. This article will therefore describe the echocardiographic appearances of each lesion, and point the reader towards specific features to look for echocardiographically...

Pediatric heart failure (HF) represents an important cause of morbidity and mortality in childhood. There is an overlapping relationship of HF, congenital heart disease, and cardiomyopathy. The goal of treatment of HF in children is to maintain stability, prevent progression, and provide a reasonable milieu to allow somatic growth and optimal development. Current management and therapy for HF in children are extrapolated from treatment approaches in adults. There are significant barriers in applying adult data to children because of developmental factors, age variation from birth to adolescence, and differences in the genetic expression profile and β-adrenergic signaling...

Rheumatic fever (RF) is an autoimmune disease associated with group A β-hemolytic streptococcal infection, in the course of which the patient develops carditis, arthritis, chorea, subcutaneous nodules and erythema marginatum. Rheumatic fever diagnosis is based on the Jones criteria, developed in 1944, then revised twice by the American Heart Association (AHA), in 1992 and recently in 2015. The last revision of the Jones criteria consists mainly in the supplementation of the major criteria with echocardiographic examination, the introduction of a concept of subclinical carditis and the isolation of low, medium and high risk populations among the patients...

The population of patients with congenital heart disease (CHD) is continuously increasing with more and more patients reaching adulthood. A significant portion of these young adults will suffer from arrhythmias due to the underlying congenital heart defect itself or as a sequela of interventional or surgical treatment. The medical community will encounter an increasing challenge as even most of the individuals with complex congenital heart defects nowadays become young adults. Within the past 20 years, management of patients with arrhythmias has gained remarkable progress including pharmacological treatment, catheter ablation, and device therapy...

This review describes the different surgical options for transposition of the great arteries, ventricular septal defect (VSD), and left ventricular outflow tract obstruction. When the pulmonary valve can be used, an arterial switch operation with VSD closure and resection of pulmonary stenosis may be possible. This is not the scope of our review: we focus on the Rastelli, REV (Réparation à l'Etage Ventriculaire), and Nikaidoh techniques, and we also describe the "en bloc rotation" technique. Each of these procedures has a different history, and these techniques have not been used uniformly around the world...

Echocardiography is the mainstay in screening for pulmonary hypertension (PH). International guidelines suggest echocardiographic parameters for suspecting PH, but these may not apply to many adults with congenital heart disease (ACHD). PH is relatively common in ACHD patients and can significantly affect their exercise capacity, quality of life, and prognosis. Identification of patients who have developed PH and who may benefit from further investigations (including cardiac catheterization) and treatment is thus extremely important...

Functional echocardiography has become an invaluable tool in the pediatric and neonatal intensive care unit. "Point-of-care," "target," or "focus" echocardiography allows bedside cardiac ultrasound evaluation of the hemodynamic status of the patient, helps in directing treatment, thus improves patients care. In order to be able to perform functional echocardiography, it is essential to understand the principles of ultrasound, to know the echocardiographic equipment and settings necessary to acquire the images...

Congenitally corrected transposition of the great arteries (ccTGA) can occur in isolation, or in combination with other structural cardiac anomalies, most commonly ventricular septal defect, pulmonary stenosis and tricuspid valve disease. Clinical recognition can be challenging, so echocardiography is often the means by which definitive diagnosis is made. The tricuspid valve and right ventricle are on the systemic arterial side of the ccTGA circulation, and are therefore subject to progressive functional deterioration...

Arrhythmias in the neonatal period are not uncommon, and may occur in neonates with a normal heart or in those with structural heart disease. Neonatal arrhythmias are classified as either benign or nonbenign. Benign arrhythmias include sinus arrhythmia, premature atrial contraction, premature ventricular contraction, and junctional rhythm; these arrhythmias have no clinical significance and do not need therapy. Supraventricular tachycardia, ventricular tachycardia, atrioventricular conduction abnormalities, and genetic arrhythmia such as congenital long-QT syndrome are classified as nonbenign arrhythmias...

PURPOSE OF REVIEW: The past couple of decades have brought tremendous advances to the field of pediatric and adult congenital heart disease (CHD). Percutaneous valve interventions are now a cornerstone of not just the congenital cardiologist treating patients with congenital heart disease, but also-and numerically more importantly-for adult interventional cardiologists treating patients with acquired heart valve disease. Transcatheter pulmonary valve replacement (tPVR) is one of the most exciting recent developments in the treatment of CHD and has evolved to become an attractive alternative to surgery in patients with right ventricular outflow tract (RVOT) dysfunction...

The original version of the article unfortunately contained an alignment error in Table 1. The correct version of Table 1 is given below.

No abstract text is available yet for this article.

BACKGROUND AND PURPOSE: The diverse causes of right-sided heart failure (RHF) include, among others, primary cardiomyopathies with right ventricular (RV) involvement, RV ischemia and infarction, volume loading caused by cardiac lesions associated with congenital heart disease and valvular pathologies, and pressure loading resulting from pulmonic stenosis or pulmonary hypertension from a variety of causes, including left-sided heart disease. Progressive RV dysfunction in these disease states is associated with increased morbidity and mortality...

The care of children with hypoplastic left heart syndrome is constantly evolving. Prenatal diagnosis of hypoplastic left heart syndrome will aid in counselling of parents, and selected fetuses may be candidates for in utero intervention. Following birth, palliation can be undertaken through staged operations: Norwood (or hybrid) in the 1st week of life, superior cavopulmonary connection at 4-6 months of life, and finally total cavopulmonary connection (Fontan) at 2-4 years of age. Children with hypoplastic left heart syndrome are at risk of circulatory failure their entire life, and selected patients may undergo heart transplantation...

In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up...

Kawasaki disease (KD) has become the most common form of pediatric systemic vasculitis. Although patients with KD received intravenous immunoglobulin (IVIG) therapy, coronary arterial lesions (CALs) still occurred in 5%-10% of these patients during the acute stage. CALs may persist and even progress to stenosis or obstruction. Therefore, CALs following KD are currently the leading cause of acquired heart diseases in children. The etiology of CALs remains unknown despite more than four decades of research. Two unsolved problems are IVIG unresponsiveness and the diagnosis of incomplete KD...

Digoxin has been associated with reduced interstage mortality after Norwood procedure. We sought to determine its association with survival and change in weight-for-age Z-score (WAZ) before the superior cavopulmonary connection (SCPC) surgery and at 14 months in a heterogeneous group of single ventricle infants. We performed a post-hoc analysis of the Pediatric Heart Network Infant Single Ventricle public use dataset to determine associations between digoxin and survival, transplant-free survival, and change in WAZ pre-SCPC and at 14 months...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

The definition and classification of ventricular septal defects have been fraught with controversy. The International Society for Nomenclature of Paediatric and Congenital Heart Disease is a group of international specialists in pediatric cardiology, cardiac surgery, cardiac morphology, and cardiac pathology that has met annually for the past 9 years in an effort to unify by consensus the divergent approaches to describe ventricular septal defects. These efforts have culminated in acceptance of the classification system by the World Health Organization into the 11th Iteration of the International Classification of Diseases...