Diagnosis and management of cyanotic congenital heart disease: part I.

Most commonly encountered cyanotic cardiac lesions in children, namely, tetralogy of Fallot, transposition of the great arteries and tricuspid atresia are reviewed. Pathology, pathophysiology, clinical features, non-invasive and invasive laboratory studies and management are discussed. The clinical and non-invasive laboratory features are sufficiently characteristic for making the diagnosis and invasive cardiac catheterization and angiographic studies are not routinely required and are needed either to define features, not clearly defined by non-invasive studies or as a part of catheter-based intervention. Surgical correction or effective palliation can be undertaken with relatively low risk. However, residual defects, some requiring repeat catheter or surgical intervention, are present in a significant percentage of patients and therefore, continued follow-up after surgery is mandatory.

Current perspectives on Kawasaki disease.

The etiology of Kawasaki disease (KD) remains unknown despite several years of dedicated research in this direction. Recently coronavirus infection and genetic polymorphisms have been implicated. Since first description of the disease there have been few changes in the diagnostic criteria except for newer recommendations of fever of at least 4 instead of 5 days duration. Recently, Echocardiography Criteria and Laboratory Criteria have been added to aid in the diagnosis of incomplete KD where all the historical diagnostic criteria are not present; this is now called the "incomplete form of KD" as opposed to "atypical form of KD". The word "atypical" is reserved for unusual presentations of KD such as those with hemophagocytic syndrome or nerve palsy. The treatment of KD includes infusion of high dose immunoglobulin. Patients non-responsive to immunoglobulin therapy are labeled as having "immunoglobulin resistant KD". The treatment of immunoglobulin resistant KD can be challenging and new therapies that have tried with some success. Late outcomes after 4 decades of treating these patients have recently been published. There has been some concern about increased risk for premature atherosclerosis in patients with childhood KD who had coronary artery abnormalities.

Recent advances in pediatric cardiology--electrophysiology, transcatheter and surgical advances.

Whereas the medical advances were reviewed in the previous paper, electrophysiological, transcatheter and surgical advances will be the focus in this review. Greater understanding of the arrhythmias, development of non-pharmacological treatment, namely catheter ablation and internal cardioverter-defibrillator (ICD) and miniaturization pacemakers and IDs have occurred in the last decade so that the methods could be applied to smaller and more complex patient population. Surgery has been the traditional treatment option for palliation and correction of congenital and acquired heart defects in infants and children. During the last one to one and one-half decades, a remarkable number of transcatheter methods were developed and refined. These developments during the last decade were reviewed and include long-term results of balloon dilatation procedures, transcatheter closure of atrial septal defects, patent ductus arteriosus, and ventricular septal defect, percutaneous valve replacement, intravascular stents to manage vascular obstructive lesions that can not be satisfactorily balloon-dilated, catheter completion of Fontan procedure, myocardial reduction in hypertrophic cardiomyopathy and other miscellaneous procedures. Recent advances in the transcatheter modes of therapy have added a new dimension to the management of neonates, infants and children with heart disease. They should now be added to the armamentarium available to the Pediatrician and Pediatric Cardiologist in the management of cardiac problems in the pediatric patient. Surgical methods and concepts have been greatly refined such that surgery can be undertaken even in the sickest and most complex patient. The majority of congenital heart defects can be corrected by open heart surgery; some require prior palliation and others can be operated without prior palliation. Recent advances in various defects were reviewed.

What is new in pediatric cardiology.

Enormous advances in the diagnosis and management of heart disease in pediatric patient have taken place during the last-four decades. In this review the authors will concentrate on the developments within the last five to ten years. It will deal with what may be called medical advances. Recent advances in molecular genetics and defining the familial patterns have led to finding that certain genetic and molecular factors are linked to congenital heart disease and arrythmia, thus providing opportunity for improved genetic counseling and future gene therapy. Medical treatment of congenital heart disease targets not only the augmentation of ventricular contractility (positive inotropy) but also addresses the neuro-humoral derangement associated with it. The ultrasound technology for the evaluation of the heart has come a long way from the early A-mode and M-mode capabilities to color Doppler, 2-dimentional and 3-dimentional capabilities.