Kawasaki disease: a review with emphasis on cardiovascular complications

Kawasaki disease (KD) is an acute systemic vasculitis that is currently the leading cause of acquired heart disease in childhood in the United States. Cardiovascular complications are the major cause of morbidity, are responsible for virtually all deaths from KD and should be evaluated as soon as possible after the acute phase to establish the baseline status, in order to predict disease progression and determine adequate treatment. In selected patients, electrocardiography (ECG)-gated cardiac computed tomography (CT) and magnetic resonance (MR) imaging are valuable non-invasive techniques that can be used to help diagnose the cardiovascular complications associated with KD. In this article, we review the epidemiology, aetiology and pathogenesis, histopathology, clinical features, cardiovascular complications and imaging, focusing on the role of cardiac CT and MR on the initial assessment and follow-up of the cardiovascular complications of KD

Tetralogy of Fallot

Tetralogy of Fallot is a congenital cardiac malformation that consists of an interventricular communication, also known as a ventricular septal defect, obstruction of the right ventricular outflow tract, override of the ventricular septum by the aortic root, and right ventricular hypertrophy

A Multicenter, Long-Term Study on Arrhythmias in Children with Ebstein Anomaly

To assess the prevalence, history, and treatment of arrhythmias, in particular preexcitation and Wolff–Parkinson–White (WPW) syndrome, in patients with Ebstein anomaly (EA) during childhood and adolescence, we performed a multicenter retrospective study of all consecutive live-born patients with EA, diagnosed, and followed by pediatric cardiologists between 1980 and 2005 in The Netherlands. During a follow-up after EA diagnosis of 13 years 3 months (range: 6 days to 28 years 2 months), 16 (17%) of the 93 pediatric EA patients exhibited rhythm disturbances. Nine patients showed arrhythmic events starting as of the neonatal period. Supraventricular tachycardia was noted in 11 patients. One patient died in the neonatal period due to intractable supraventricular tachycardia resulting in heart failure and one patient died at 5 weeks of age most probably due to an arrhythmic event. The 14 surviving patients all show preexcitation, albeit 4 of them intermittently, and all have a right-sided accessory pathway location. Nine patients underwent catheter ablation of an accessory pathway. Only four patients are currently on antiarrhythmic drugs. The 17% prevalence of rhythm disturbances in pediatric EA patients, most commonly supraventricular arrhythmias, is significantly lower than in adult EA patients. Life-threatening rhythm disturbances are not frequent early in life. Symptomatic patients are well treated with radiofrequency catheter ablation

Unambiguous molecular detections with multiple genetic approach for the complicated chromosome 22q11 deletion syndrome

<p>Abstract</p> <p>Background</p> <p>Chromosome 22q11 deletion syndrome (22q11DS) causes a developmental disorder during the embryonic stage, usually because of hemizygous deletions. The clinical pictures of patients with 22q11DS vary because of polymorphisms: on average, approximately 93% of affected individuals have a de novo deletion of 22q11, and the rest have inherited the same deletion from a parent. Methods using multiple genetic markers are thus important for the accurate detection of these microdeletions.</p> <p>Methods</p> <p>We studied 12 babies suspected to carry 22q11DS and 18 age-matched healthy controls from unrelated Taiwanese families. We determined genomic variance using microarray-based comparative genomic hybridization (array-CGH), quantitative real-time polymerase chain reaction (qPCR) and multiplex ligation-dependent probe amplification (MLPA).</p> <p>Results</p> <p>Changes in genomic copy number were significantly associated with clinical manifestations for the classical criteria of 22q11DS using MPLA and qPCR (<it>p </it>< 0.01). An identical deletion was shown in three affected infants by MLPA. These reduced DNA dosages were also obtained partially using array-CGH and confirmed by qPCR but with some differences in deletion size.</p> <p>Conclusion</p> <p>Both MLPA and qPCR could produce a clearly defined range of deleted genomic DNA, whereas there must be a deleted genome that is not distinguishable using MLPA. These data demonstrate that such multiple genetic approaches are necessary for the unambiguous molecular detection of these types of complicated genomic syndromes.</p

Intracardiac Devices for Stroke Prevention

Stroke is a significant cause of serious disability and death worldwide. A substantial proportion of strokes are related to an underlying cardiac embolic source, most commonly in association with atrial arrhythmias (fibrillation/flutter). Atrial fibrillation is considered a major risk factor for stroke. Although long-term prophylactic oral anticoagulation has been shown to be very effective in reducing stroke in patients with atrial fibrillation, it has a number of major limitations and is not feasible in all patients. In such cases, the use of percutaneously (transvenous) implanted left atrial appendage occlusive devices or surgical appendage obliteration is being explored. Similarly, the presence of a patent foramen ovale, especially in the presence of an atrial septal aneurysm, is now recognized as an important potential mediator of paradoxical cardiogenic embolism. Percutaneous patent foramen ovale closure is becoming increasingly established as a safe and effective means of preventing recurrent strokes in the presence of a patent foramen ovale. In this account, the authors discuss the intracardiac devices and techniques available and the relative merits of their use for stroke prevention