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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. [ABSTRACT FROM AUTHOR]

In this review, the clinical features and management of most commonly encountered acyanotic, left-to-right shunt lesions are discussed. Patients with small defects, especially in childhood, are usually asymptomatic while moderate to large defects in infancy may present with symptoms. Hyperdynamic precordium, widely split and fixed second heart sound, ejection systolic murmur at the left upper sternal border and a mid-diastolic flow rumble at the left lower sternal border are present in atrial septal defects, holosystolic murmur at the left lower border is characteristic for a ventricular septal defect whereas a continuous murmur at the left upper sternal border is distinctive for patent ductus arteriosus. Clinical diagnosis is not usually difficult and the diagnosis can be confirmed and quantitated by non-invasive echocardiographic studies. Whereas surgical intervention was used in the past, transcatheter methods are increasingly used for closure of atrial septal defect and patent ductus arteriosus. Small ventricular septal defects may not need to be closed whereas medium and large defects may require surgical closure. Transcatheter closure of both muscular and membranous ventricular septal defects is feasible by transcatheter methodology, but these techniques are experimental at the time of this writing. [ABSTRACT FROM AUTHOR]

In this review, the clinical features and management of most commonly encountered acyanotic obstructive cardiac lesions are discussed. Mild lesions, especially in children are usually asymptomatic while neonates and infants may present with symptoms. Ejection systolic murmurs in patients with pulmonic and aortic stenosis and decreased femoral pulses and blood pressure difference (>20 mmHg) between arms and leg in patients with aortic coarctation are usually seen. Clinical diagnosis is not difficult and the diagnosis can be confirmed and quantitated by non-invasive echocardiographic studies. Whereas surgical intervention was used in the past, balloon dilatation appears to be effective in the treatment of these lesions. [ABSTRACT FROM AUTHOR]

Fontan operation, since its original description, has undergone a number of modifications so that it is now a staged, total cavo-pulmonary connection with fenestration. Stage I is palliation, depending upon the pathophysiology of the defect complex in early life, Stage II is bidirectional Glenn at about the age of 6 mo and Stage III is transfer of inferior vena caval blood to the pulmonary circuit along with fenestration between 2 to 4 y. Any patient that has only one functioning ventricle is a candidate for Fontan surgery. The morbidity and mortality have remarkably improved since the institution of staged, total cavo-pulmonary connection with fenestration. Complications during follow up continue to occur, though diminished with the newer modifications, and should be promptly addressed.

The purpose of this review/editorial is to discuss how and when to treat the most common cyanotic congenital heart defects (CHDs); the discussion of acyanotic heart defects was presented in a previous editorial. By and large, the indications and timing of intervention are decided by the severity of the lesion. While some patients with acyanotic CHD may not require surgical or transcatheter intervention because of spontaneous resolution of the defect or mildness of the defect, the majority of cyanotic CHD will require intervention, mostly surgical. Total surgical correction is the treatment of choice for tetralogy of Fallot patients although some patients may need to be palliated initially by performing a modified Blalock-Taussig shunt. For transposition of the great arteries, arterial switch (Jatene) procedure is the treatment of choice, although Rastelli procedure is required for patients who have associated ventricular septal defect (VSD) and pulmonary stenosis (PS). Some of these babies may require Prostaglandin E1 infusion and/or balloon atrial septostomy prior to corrective surgery. In tricuspid atresia patients, most babies require palliation at presentation either with a modified Blalock-Taussig shunt or pulmonary artery banding followed later by staged Fontan (bidirectional Glenn followed later by extracardiac conduit Fontan conversion usually with fenestration). Truncus arteriosus babies are treated by closure of VSD along with right ventricle to pulmonary artery conduit; palliative banding of the pulmonary artery is no longer recommended. Total anomalous pulmonary venous connection babies require anastomosis of the common pulmonary vein with the left atrium at presentation. Other defects should also be addressed by staged correction or complete repair depending upon the anatomy/physiology. Feasibility, safety and effectiveness of treatment of cyanotic CHD with currently available medical, transcatheter and surgical methods are well established and should be performed at an appropriate age in order to prevent damage to cardiovascular structures.

The purpose of this review/editorial is to discuss how and when to treat the most common acyanotic congenital heart defects (CHD); the discussion of cyanotic heart defects will be presented in a subsequent editorial. By and large, the indications and timing of intervention are decided by the severity of the lesion. Balloon pulmonary valvuloplasty is the treatment of choice for valvar pulmonary stenosis and the indication for intervention is peak-to-peak systolic pressure gradient >50 mmHg across the pulmonary valve. For aortic valve stenosis, balloon aortic valvuloplasty appears to be the first therapeutic procedure of choice; the indications for balloon dilatation of aortic valve are peak-to-peak systolic pressure gradient across the aortic valve in excess of 70 mmHg irrespective of the symptoms or a gradient ≥ 50 mmHg with either symptoms or electrocardiographic ST-T wave changes indicative of myocardial perfusion abnormality. The indications for intervention in coarctation of the aorta are significant hypertension and/or congestive heart failure along with a pressure gradient in excess of 20 mmHg across the coarctation; the type of intervention varies with age at presentation and the anatomy of coarctation: surgical intervention for neonates and young infants, balloon angioplasty for discrete native coarctation in children, and stents in adolescents and adults. Long segment coarctations or those associated with hypoplasia of the isthmus or transverse aortic arch require surgical treatment in younger children and stents in adolescents and adults. For post-surgical aortic recoarctation, balloon angioplasty in young children and stents in adolescents and adults are treatment options. Transcatheter closure methods are currently preferred for ostium secundum atrial septal defects (ASDs); the indications for occlusion are right ventricular volume overload by echocardiogram. Ostium primum, sinus venosus and coronary sinus ASDs require surgical closure. For all ASDs elective closure around age 4 to 5 y is recommended or as and when detected beyond that age. For the more common perimembraneous ventricular septal defects (VSDs) of large size, surgical closure should be performed prior to 6 to 12 mo of age. Muscular VSDs may be closed with devices. Patent ductus arteriosus (PDA) may be closed with Amplatzer Duct Occluder if they are moderate to large and Gianturco coils if they are small. Surgical and video-thoracoscopic closure are the available options at some centers. In the presence of pulmonary hypertension appropriate testing to determine suitability for closure should be undertaken. The treatment of acyanotic CHD with currently available medical, transcatheter and surgical methods is feasible, safe and effective and should be performed at an appropriate age in order to prevent damage to cardiovascular structures.

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.

Necrotizing fasciitis can be observed in a wide variety of clinical settings in the pediatric age group. But necrotizing fasciitis as a complication of chickenpox is rare. Five cases of necrotizing fasciitis following chickenpox were treated in the authors' institutions. Clinical signs like fever, tachycardia, pallor, erythema, marked edema of the affected area and patchy areas of discoloration or gangrene of the skin were present and were the important clues to the seriousness of infection. Patients were treated with aggressive surgical debridement, frequent dressings, broad-spectrum antibiotics, nutritional support, and split skin grafting. Early diagnosis and aggressive surgical therapy are critical. Literature search showed necrotizing fasciitis following chickenpox has not been reported in India. We feel early suspicion and aggressive management can decrease the morbidity and mortality in this rare complication.

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.

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.

Achalasia is rare in children, more so familial. We report two siblings with familial achalasia who presented in their infancy with vomiting and failure to thrive. Achalasia can be misdiagnosed as upper gastrointestinal obstruction as happened in one of our siblings. Esophageal contrast roentgenography is diagnostic. Both the children were treated successfully by transabdominal esophagomyotomy with fundoplication.

Congenital coronary artery abnormalities are rare and account for approximately 0.1 to 2% of congenital heart defects. They may pose significant risk of mortality or morbidity to the patient. The pediatrician and the pediatric cardiologist should be aware of their subtle but very serious presentations and diagnostic steps to be undertaken to pinpoint the diagnosis. Prevention of serious complications from these abnormalities can be achieved by making the appropriate diagnosis and performing timely surgical intervention. This review will discuss the most common congenital coronary artery abnormalities and their management.

Chronic chest pain is a complaint that frequently prompts referral to pediatric cardiology clinics although very few pediatric patients with this symptom will be found to have cardiac disease. This review will discuss the common non-cardiac and cardiac causes of chest pain. Guidelines on the office management of this group of patients will be presented as well as the indications for referring patients for evaluation and treatment by the subspecialist.

Surgical treatment for congenital heart disease has become available over the last five decades. Palliative procedures have been designed to improve physiologic abnormalities, for example systemic artery (or venous) to pulmonary artery shunts of various types to increase the pulmonary blood flow, pulmonary artery constriction (banding) to decrease the pulmonary blood flow, and surgical or transcatheter atrial septostomy to augment intracardiac mixing. These can be performed with a low mortality. The majority of congenital heart defects can be corrected by open heart surgical techniques; some require prior palliation and others can be operated without prior palliative surgery. Recent surgical advances include early total surgical correction for tetralogy of Fallot, arterial switch procedure for transposition of the great arteries, Fontan operation and its modifications for tricuspid atresia and single ventricle, new operations for hypoplastic left heart syndrome, newer prosthetic valves, myocardial preservation and cardiac transplantation.