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Journal of the Korean Pediatric Cardiology Society.2006;10(4):385-390.

Disparate results for human coronavirus as causative agent likely correspond to the concept that the inflammations associated with Kawasaki disease does not result from a single infectious trigger but rather a final common inflammatory pathway, in susceptible individuals, following a variety of infectious or environmental triggers. It is hoped that there will be a change in the current diagnostic criteria, which do not apply in cases of incomplete disease. So detailed algorithm and new diagnostic criteria may be needed. In patients whose Kawasaki disease is resistant or refractory to IVIG, treatment with tumor necrosis factor (TNF-alpha) blockade and steroids seems to be safe and promising deserving of future study. Echocardiography is important in confirming the diagnosis and should be performed in all suspected cases. New non-invasive tools including MRA and MSCT are of great value for the follow-up of aneurysm progress and outcome.
Aneurysm ; Coronavirus ; Diagnosis ; Echocardiography ; Follow-Up Studies ; Hope ; Humans ; Immunoglobulins, Intravenous ; Inflammation ; Mucocutaneous Lymph Node Syndrome* ; Steroids ; Tumor Necrosis Factor-alpha ; Infliximab

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Surgical Treatment for Kawasaki Disease.

Ji Hyuk YANG ; Tae Gook JUN ; Young Tak LEE

Journal of the Korean Pediatric Cardiology Society.2006;10(4):373-384.

Kawasaki disease is characterized by a variety of symptoms and signs resulted from systemic vasculitis. Although the etiology of the disease remains uncertain, its serious coronary sequelae have been proved to cause ischemic heart disease in children. Coronary artery aneurysms or ectasia develop in approximately 20% of untreated children with the disease and may lead to coronary ischemia. Although the incidence of severe coronary arterial stenosis is low (2-3%), but once myocardial infarction occurs in children, the mortality is quite high (22% at the first infarction). Children with coronary aneurysms should be assessed carefully with periodic stress testing for reversible ischemia. Coronary artery bypass surgery is indicated when there is an evidence of myocardial ischemia even in small children. However, one should keep in mind that the ability to recanalize or develop collateral vessels in children is very high and recanalization is frequently noted in the right coronary artery. Internal thoracic arteries are the graft of choice. We performed coronary bypass grafting in 5 patients with Kawasaki disease since 2001. The mean age of the patients was 18.8+/-11.2 (range, 2-30) year. The mean number of anastomosis was 1.8+/-1.1. All patients survived and have revealed no evidence of myocardial ischemia during follow-up.
Aneurysm ; Child ; Constriction, Pathologic ; Coronary Aneurysm ; Coronary Artery Bypass ; Coronary Artery Disease ; Coronary Vessels ; Dilatation, Pathologic ; Exercise Test ; Follow-Up Studies ; Humans ; Incidence ; Ischemia ; Mammary Arteries ; Mortality ; Mucocutaneous Lymph Node Syndrome* ; Myocardial Infarction ; Myocardial Ischemia ; Systemic Vasculitis ; Transplants

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Interventions in Coronary Lesions Associated with Kawasaki Disease.

Jae Hyeong PARK ; Jae Hwan LEE

Journal of the Korean Pediatric Cardiology Society.2006;10(4):367-372.

The incidence of coronary artery disease requiring coronary intervention in patients with Kawasaki disease is high. Because coronary artery lesions in Kawasaki disease commonly involve severe calcification and aneurysmal changes which can progress with time, in contrast with adult atherosclerotic coronary artery lesions, the indication or technique of catheter intervention for adult patients cannot be directly applied. However, the experience of coronary intervention in Kawasaki disease is extremely limited compared to that with intervention in adults, which provides satisfactory therapeutic results. There are several kinds of percutaneous coronary intervention techniques in Kawasaki disease including balloon angioplasty, stent implantation, rotational ablation, and directional coronary atherectomy. Satisfactory acute results for coronary balloon angioplasty can be obtained in patients in a relatively short interval from the onset of disease, especially within 6 years. However, the incidence of restenosis after angioplasty is still high. Stent implantation acquires larger luminal area, less restenosis rate and less aneurysmal formation than balloon angioplasty. Rotational ablation is a good interventional option with high success rate for longstanding Kawasaki disease with severe calcification. Intravascular ultrasound imaging provides valuable information for the selection of the appropriate interventional procedure and the assessment of postprocedural outcomes. To obtain good result and optimal decision making, cooperation between pediatric and adult cardiologists is essential. Postprocedural anticoagulation or antiplatelet regimens are required for proper long-term management.
Adult ; Aneurysm ; Angioplasty ; Angioplasty, Balloon ; Angioplasty, Balloon, Coronary ; Atherectomy, Coronary ; Catheters ; Coronary Artery Disease ; Coronary Vessels ; Decision Making ; Humans ; Incidence ; Mucocutaneous Lymph Node Syndrome* ; Percutaneous Coronary Intervention ; Phenobarbital ; Stents ; Ultrasonography

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Direct Visualization of Coronary Artery and Flow using Transthoracic Doppler Echocardiography.

Ho Joong YOUN

Journal of the Korean Pediatric Cardiology Society.2006;10(4):354-366.

With advancements of high frequency transducer, transthoracic Doppler echocardiography (TTE) is emerging as a promising method for evaluating coronary artery. In addition to visualizing the images for detection of stenosis of proximal and distal coronary artery and various kinds of coronary artery anomaly, functional assessment through measurement of coronary flow reserve using TTE have become a valuable and additive tool for coronary angiography defining only the epicardial coronary arteries. Further efforts for development of new techniques including real time 3D echocardiography in the anatomic and functional assessment of coronary artery disease should be undertaken.
Constriction, Pathologic ; Coronary Angiography ; Coronary Artery Disease ; Coronary Circulation ; Coronary Vessels* ; Echocardiography ; Echocardiography, Doppler* ; Echocardiography, Three-Dimensional ; Transducers

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Immunopathogenesis of Kawasaki Disease.

Jeong Kyu PARK

Journal of the Korean Pediatric Cardiology Society.2006;10(4):349-353.

Kawasaki disease (KD) is an acute, self-limiting, small-vessel vasculitis with an unknown cause that affects children between the ages of 6 months and 5 years. It is the most common cause of acquired coronary artery disease in childhood. Acute myocardial infarction and coronary artery aneurysm are major complications. Although an infectious agent is highly suspected, the etiology of KD is unknown. Significant progress has been, however, toward understanding the natural history of this disease, and therapeutic interventions have been developed that halt the immune-mediated destruction of the vascular system. The pathology of the necrotizing vaculitis of KD suggests a primary role for monocytes-macrophages and T lymphocytes in the acute vascular injury observed. KD fits nicely in the spectrum between an infectious disease and a true autoimmune disease, with an infectious trigger leading to a prolonged self-directed immune response. This review focuses on recent data concerning the immunopathogenesis of vascular damage, and the involvement of CD4+CD25+ regulatory T cells (Treg) in KD.
Aneurysm ; Autoimmune Diseases ; Child ; Communicable Diseases ; Coronary Artery Disease ; Coronary Vessels ; Humans ; Mucocutaneous Lymph Node Syndrome* ; Myocardial Infarction ; Natural History ; Pathology ; T-Lymphocytes ; T-Lymphocytes, Regulatory ; Vascular System Injuries ; Vasculitis

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A Case of Three-Dimensional Image Reconstruction of Pulmonary Venous Return .

Hye Kyung LEE ; Chang Hwi KIM ; Yun Seoup JUNG

Journal of the Korean Pediatric Cardiology Society.2000;4(1):78-80.

A case of supracardiac type of total anomalous pulmonary venous return into the superior vena cava is reported which was successfully diagnosed using spiral CT and three-dimensional image reconstruction. Spiral CT with three-dimensional reconstruction is non-invasive method of visualizing the vascular system and in some instances can replace conventional angiography in pediatric patients.
Angiography ; Humans ; Imaging, Three-Dimensional* ; Scimitar Syndrome ; Tomography, Spiral Computed ; Vena Cava, Superior

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The Study of Selective Decreased Serum IgG4 In Acute Phase of Kawasaki Disease.

Min Shik KIM ; Yoon Hwa CHA ; Kyung Sook KIM

Journal of the Korean Pediatric Cardiology Society.2000;4(1):71-77.

PURPOSE: The study was performed to investigate the IgG subclasses in acute febrile phase of Kawasaki disease(KD) to find correlation between selective IgG4 deficiency and incidence of KD patients. METHODS: The patients group consisted of 64 cases in acute phase of KD hospitalized from Jan. 1996 to Jul. 1999, and control group consisted of age matched 36 cases who admitted without KD. The obtained sera were measured the level of total IgG, IgM, IgA and IgG subclasses(IgG1, IgG2, IgG3, IgG4) by using Enzyme Immuno Assay and Single Radial Immuno-Diffusion method. RESULTS: The sex ratio of male to female was 1.4:1.0, and febrile days before admission was 4.6+/-2.4 days. Total levels of IgG, IgM and IgA were normal range in both groups. The levels of IgG1, IgG2 and IgG3 were not specific changes but significantly decreased the level of IgG4 in patients group as compared with control group(P<0.05). CONCLUSION: Selective decreased the levels of serum IgG4 can be one of factors to increasing the incidence of KD.
Female ; Humans ; Immunoglobulin A ; Immunoglobulin G* ; Immunoglobulin M ; Incidence ; Male ; Mucocutaneous Lymph Node Syndrome* ; Reference Values ; Sex Ratio

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Clinical Study on Supraventricular Tachycardia in Children.

Hyeon Seung LEE ; Byung Ju KIM ; Jae Sook MA

Journal of the Korean Pediatric Cardiology Society.2000;4(1):62-70.

PURPOSE: Supraventricular tachycardia(SVT) is the most common symptomatic tachyarrhythmia in pediatric age group. Causes, types, and clinical characteristics of this arrhythmia in children are different from those in adults. This study was undertaken to investigate the clinical features of SVT in children. METHODS: A retrospective study was done on 36 patients with SVT, who were diagnosed at Department of Pediatrics, Chonnam National University Hospital from January, 1991 through August, 1999. The types of SVT, age of onset, associated cardiovascular abnormalities, and response to treatment were analyzed. RESULTS: Atrioventricular reentrant tachycardia(AVRT) using an accessory pathway was found in 63.9%. Primary atrial tachycardia and AV nodal reentrant tachycardia(AVNRT) were noted in 25.0% and in 5.6%, respectively. SVT occurred in over 50% of the total patients before 1 year of age : during the neonatal period, 22.2% ; during the infant period, 33.3%. Primary atrial tachycardia usually developed in the infant period. AVRT mostly occurred in the infant period, appeared to decline in the preschool period and then to increase again in the school age. AVNRT occurred only after 1 year of age. Associated cardiovascular abnormalities were found in 22.2%. Successful conversion to sinus rhythm was possible in 91.7%. Initially, normal sinus rhythm was achieved in 55.9% of the 34 children treated with adenosine. Radiofrequency catheter ablation(RFCA) was successfully performed in all 10 patients without complication and there was no recurrence of SVT during the follow-up period. CONCLUSION: We conclude that the mechanism of SVT appeared to have an age-dependent distribution, AVRT was the most common mechanism and adenosine was effective drug for restoration of normal sinus rhythm in the initial treatment of SVT. RFCA seemed to be effective and safe method for the treatment of SVT in children.
Adenosine ; Adult ; Age of Onset ; Arrhythmias, Cardiac ; Cardiovascular Abnormalities ; Catheters ; Child* ; Follow-Up Studies ; Humans ; Infant ; Jeollanam-do ; Pediatrics ; Recurrence ; Retrospective Studies ; Tachycardia ; Tachycardia, Supraventricular*

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Establishment of Cardiac Perfusion System using Personal Computer for in vitro Electrophysiologic Study.

Seung Hee MOK ; Heon Seok HAN

Journal of the Korean Pediatric Cardiology Society.2000;4(1):54-61.

PURPOSE: After Langendorff made aortic perfusion system with the extracted animal heart for the experimental study regarding cardiac mechanical action and rhythm, this system has been modified by various methods for the hemodynamic studies. The authors developed cardiac perfusion system using personal computer for mechanical cardiac contraction and electrophysiologic studies without the effects of autonomic nervous system and hemodynamic influences from various causes. METHODS: This system consisted of perfusion system, electrical pulse generator, and data acquisition system. the perfusion system has reservoir containing physiologic solution, heat exchange system, and organ chamber. The pulse generator composed of personal computer and digital/analog converter can produce electrical pulses with adjustable amplitude and cycle length. The mode of stimulations were as follows; maintenance, programmed, and burst mode. The data acquisition system was composed of amplifier, analog/digital converter, and AcqKnowledge program that are provided by Biopac system. The data were sampled at 1kHz as a digitized form, and the noises wee reduced by filtering at band width 30-500Hz. Krebs' solution was used as perfusate through the aorta of isolated heart with mean pressure of 40mmHg. RESULTS: With this system, AH and HV interval, refractory period of AV node and His-Purkinje system, and Wenckebach cycle length were measured. CONCLUSION: Studies for cardiac mechanical contraction as well as electrophysiologic properties in vitro with or without specific cardiac drugs would be possible with this system in isolated experimental heart without autonomic nervous effects.
Animals ; Aorta ; Atrioventricular Node ; Autonomic Nervous System ; Electrophysiology ; Heart ; Hemodynamics ; Hot Temperature ; Humans ; Microcomputers* ; Noise ; Perfusion*

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Management and Outcome of Atrial Isomerism.

Jong Kyun LEE

Journal of the Korean Pediatric Cardiology Society.2000;4(1):45-53.

No Abstract available.
Isomerism*

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