Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), also termed Bland-White-Garland syndrome, is a rare congenital cardiac anomaly characterized by myocardial ischemia which becomes symptomatic during early infancy leading to infarction, left heart failure or even death. More than 90% of patients can be dead within first year of life if untreated, so adult type ALCAPA without surgical correction is quitely rare. We present a case of a 20-year-old man with ALPACA syndrome whose diagnosis took several years to be made because he was mistaken to have organic mitral regurgitation.
Adult* ; Bland White Garland Syndrome ; Camelids, New World ; Coronary Vessels* ; Diagnosis ; Heart Failure ; Humans ; Infarction ; Mitral Valve Insufficiency ; Myocardial Ischemia ; Pulmonary Artery* ; Young Adult

PURPOSE: Kawasaki disease can cause cardiovascular complications if not properly treated from the beginning. Recently, serum amyloid A(SAA) was reported to be a predictive factor of cardiovascular diseases. Therefore, it was examined whether the existence of coronary artery abnormality in Kawasaki disease can be predicted in acute stage. METHODS: Forty nine patients who were diagnosed with Kawasaki disease between October, 2006 and May, 2007 at Yonsei University, Wonju College of Medicine were selected for this study. We reviewed results of CBC, AST, ALT, CK, LDH, total bilirubin, albumin, CRP, CK-MB, troponin-I, LDL, HDL, SAA, ESR. We divided the patients into two groups: Group A consisting of patients with coronary artery lesions, and group B consisting of patients without coronary artery lesions. RESULTS: CRP was significantly higher in group A (group A 11.0+/-7.0 mg/dL vs group B 5.3+/-5.3 mg/dL, P=0.030). SAA was slightly higher in group A but did not show any statistical significance (group A 283.8+/-357.3 microgram/mL vs group B 133.2+/-293.4 microgram/mL, P=0.128). Binary regression analysis was used to identify the significance of SAA as a predictor of coronary artery abnormality but did not find any significance (SAA OR=1.000, 95% CI=0.998-1.002, P=0.950). CONCLUSION: SAA are not significant predictors of coronary artery abnormality in Kawasaki disease but are non specific factors which increase in the acute stage.
Amyloid ; Bilirubin ; Cardiovascular Diseases ; Coronary Vessels* ; Gangwon-do ; Humans ; Mucocutaneous Lymph Node Syndrome* ; Serum Amyloid A Protein* ; Troponin I

PURPOSE: Transcatheter closure of patent foramen ovale (PFO), atrial septal defect (ASD) and patent ductus arteriosus (PDA) is a new and less traumatic technique than open heart surgery. One of the more popular occluding devices is the Amplatzer(R) septal occluder which is made of nitinol. The present study was undertaken to evaluate the safety and release of nickel after implantation of Amplatzer(R) occluder in patients with PFO, ASD and PDA. METHODS: Random blood samples were obtained from 25 patients with Amplatzer(R) PFO, ASD, PDA occluder during 4-year and 7-month post closure period. The nickel content in the specimens was determined using atomic absorption spectrometer. RESULTS: All patients showed satisfactory clinical improvements and there was no echocardiographic evidence of complications. During the post closure, concentrations of nickel in serum were within normal range with values 0.2 ug/dL. CONCLUSION: Nickel seems to be released from Amplatzer(R) occluder. The dissolusion of nickel from Amplatzer(R) occluder is minimal and systemic rise in serum levels of nickel are within normal range. However, further studies are needed to evaluate biological effects in patients with nickel hypersensitivity.
Absorption ; Ductus Arteriosus, Patent ; Echocardiography ; Foramen Ovale, Patent ; Heart Septal Defects, Atrial ; Humans ; Hypersensitivity ; Nickel* ; Reference Values ; Septal Occluder Device ; Thoracic Surgery

PURPOSE: This study sought to analyze the safety, efficacy, and follow-up results of percutaneous closure with Amplatzer septal occulder (ASO) for secundum atrial septal defect (ASD) in less than 10 years old by comparing to those of adult. METHODS: We divided 49 enrolled subjects to two groups as childhood group (25 patients, 10< years) and adult group (24 patients, 20> years). We evaluated hemodynamic changes, including Qp/Qs, systolic right ventricular pressure, pulmonary artery pressure in pre- and post-treatment. The residual shunt rate and complications were investigated. Then, we compared the results between two groups. RESULTS: The success rate of two groups was not statistically different (100% vs 95.8%). The hemodynamic changes, including Qp/Qs, systolic right ventricular pressure, pulmonary artery pressure were statistically significant between pre- and post-treatment (all P<0.05). However, hemodynamic change difference between children and adult group was not statistically significant (all P>0.2). The residual shunt rate and complications occurrence rate were not statistically significant between two groups. CONCLUSION: The cure rate and complications by percutaneous closure of ASD with ASO were not different between in children and in adult. This procedure was an effective treatment modality for ASD in children like in adult.
Adult* ; Child* ; Follow-Up Studies ; Heart Septal Defects, Atrial* ; Hemodynamics ; Humans ; Pulmonary Artery ; Septal Occluder Device* ; Ventricular Pressure

PURPOSE: This study was aimed to assess the growth of neo-aorta and its implication to neo-aortic insufficiency in children with complete transposition of great arteries (TGA) after arterial switch operation (ASO). METHOD: We retrospectively reviewed medical records, echocardiograms and cardiac catheterization of 40 patients who had underwent ASO at Samsung Medical Center from 1995 through 2001. Pulmonary artery and aorta were evaluated regarding its stenosis and regurgitation, respectively. The growth of the neo-aortic valve, sinus of neo-aortic Valsalva, and the site of aortic anastomosis were evaluated by measuring the change of the diameter. RESULT: The mean duration of follow-up after ASO was 17.2+/-12.4 months (range 1.2-67 months). Aortic insufficiency (AI) developed in 45%, in which all were mild. The neo-aortic annulus (originally pulmonary annulus) had grown as normal pulmonic valve does do (diameter of pulmonary valve annulus preoperatively, 8.9+/-1.22 mm; postoperatively at more than 6 months, 12.8+/-2.2mm). The anastomotic site of neo-aorta showed a growth curve equivalent to that of sinotubular junction of normal aorta (preoperative diameter, 7.7+/-1.4 mm; postoperatively at more than 6months, 12.7+/-3.1mm). However, the growth rate of sinus of Valsalva showed a extremely higher compared to that of normal aorta (preoperative diameter, 10.5+/-1.2 mm; postoperatively at more than 6 months, 18.8+/-2.6 mm). No significant relations could be revealed between the change of dimension of aortic root and aortic insufficiency. CONCLUSION: Aortic insufficiency was not uncommon but mild aortic dilatation was not significantly associated with AI. Although the short term result is encouraging, long-term surveillance for aortic root dilatation and aortic insufficiency remains necessary.
Aorta ; Cardiac Catheterization ; Cardiac Catheters ; Child ; Constriction, Pathologic ; Dilatation ; Follow-Up Studies ; Humans ; Medical Records ; Pulmonary Artery ; Pulmonary Valve ; Retrospective Studies ; Sinus of Valsalva ; Transposition of Great Vessels*

PURPOSE: Resveratrol (trans-3,4',5-trihydroxystilbene), abundant in skin of grapes and red wines, has been known to protect heart cells from hypoxia/ischemia injury through its anti-oxidant properties and may also exert its cardioprotective action. There, to date, are no reports about the relationship with nitric oxide (NO)-mediated mechanism. Therefore, we investigated whether resveratrol can regulate the expression of NO synthase (NOS) in an in vitro hypoxic model of cultured H9c2 cardiomyoblasts. METHODS: The cultured H9c2 cardiomyoblasts were divided into four groups: a normal control group, a hypoxic group, two groups each treated with resveratrol before and after hypoxic insult. The control cells were placed in 5% CO2 incubator, and the hypoxic and resveratrol-treated groups were placed in 1% O2 incubator. NO activity was determined for all three isoforms of NOS; induced NOS (iNOS), endothelial NOS (eNOS), and neuronal NOS (nNOS) using real-time PCR. RESULTS: The expressions of iNOS and eNOS were decreased in the hypoxic group compared to the control group, whereas the expression of nNOS was greater in the hypoxic group than in the control group. In contrast, the group treated with resveratrol before hypoxic insult showed increased expressions of iNOS and eNOS as compared to the hypoxic group. CONCLUSION: The results of the present study demonstrate that activation of iNOS and eNOS, but not nNOS, may be one of the mechanisms involved in the protective effect on resveratrol against hypoxic myocardial injury.
Anoxia ; Heart ; Incubators ; Neurons ; Nitric Oxide Synthase* ; Nitric Oxide* ; Protein Isoforms* ; Real-Time Polymerase Chain Reaction ; Skin ; Vitis ; Wine

Echocardiography is a useful, safe and noninvasive method and is the cornerstone in diagnosis and management of children with heart disease1). Although, today more technologically advanced echocardiographic methods are used in this area, transthoracic echocardiography still has been an ideal tool for cardiac assessment, as it is noninvansive, portable, and efficacious in providing detailed anatomic, hemodynamic, and physiologic information about the pediatric heart2). And the two-dimensional echocardiography with Doppler echocardiography is the essential part of the transthoracic echocardiography. Various images should be displayed in pediatric congenital heart disease, because there is a wide spectrum of anomalies. So, standard echocardiographic images and several echocardiographic findings of heart disease in children based on two-dimensional and Doppler echocardiography in transthoracic echocardiography will be briefly presented in this paper.
Child* ; Diagnosis ; Echocardiography* ; Echocardiography, Doppler ; Heart Defects, Congenital ; Heart Diseases* ; Heart* ; Hemodynamics ; Humans

A complete history and physical examination for heart diseases in children, especially neonates and infants, enables the pediatrician to compile an appropriate differential diagnosis, order tests such as chest radiography and electrocardiogram in a suitable manner, and efficiently care for the patients. The ability to obtain an accurate history and to perform an excellent physical examination is very important and provides the basis for best management of children with heart diseases. As such, we are reviewed the components of the history and the physical examination that are critical in assessing the cardiovascular system.
Cardiovascular System ; Child* ; Diagnosis, Differential ; Electrocardiography ; Heart Diseases* ; Heart* ; Humans ; Infant ; Infant, Newborn ; Physical Examination* ; Radiography ; Thorax

The prevalence of obesity and overweight among children and adolescents has increased during the past decade. Obesity during the children and adolescents constitutes the major components of metabolic syndrome (MS) and is considered as a significant independent predictor of cardiovascular risk. The MS is a grouping of clinical characteristics including insulin resistance, abdominal obesity, impaired glucose tolerance. elevated blood pressure, elevated triglycerides, and reduced high-density lipoprotein cholesterol (HDL-cholesterol). Other common manifestations include systemic inflammation and a prothrombotic state. Studies suggest that a substantial percentage of overweight children and adolescents may be affiliated the metabolic syndrome because many have 1 or more of the following: an elevated triglyceride level, a low HDL-cholesterol level, and high blood pressure. Many overweight children also have elevated insulin levels. So, clinicians should assess overweight children for manifestations of the MS. The metabolic and cardiovascular consequences of the MS are well demonstrated and have a major impact on the development of atherosclerosis and lifetime cardiovascular risk. Despite the degree to which the MS has been established, it is not without controversy in point of several definitions, physiologic accuracy, and its value as risk factor of atherosclerotic cardiovascular disease. This review will summarize recent data about the metabolic syndrome in the pediatric age group.
Adolescent ; Atherosclerosis ; Blood Pressure ; Cardiovascular Diseases ; Child* ; Cholesterol ; Glucose ; Humans ; Hypertension ; Inflammation ; Insulin ; Insulin Resistance ; Lipoproteins ; Obesity ; Obesity, Abdominal ; Overweight ; Prevalence ; Risk Factors ; Triglycerides

Infants with congenital heart diseases are prone to malnutrition due to low calory intake and high energy requirement. Energy imbalance affects outcome of cardiac surgery and perioperative mortality. Most infants with congenital heart disease have normal weight for gestational age at birth but develop nutritional disturbance in early infancy. The extent of growth failure does not always correlated with the severity of cardiac lesion, but infants with cyanotic heart lesions reveal more decrease in weight and height compared to healthy infants. Significant improvements of weight and height may occur within months after corrective surgery. Delayed surgical correction for congenital heart lesion lead to malnutrition and operative outcome. Hence, early corrective surgery is recommended for critical congenital heart lesions in symptomatic neonates and infants. Aggressive preoperative nutritional support with proper assessment of growth status, and high calory and high protein formula feeding is required to minimize perioperative risks but also maximize catch-up growth after cardiac surgery.
Child* ; Gestational Age ; Heart ; Heart Defects, Congenital ; Heart Diseases* ; Humans ; Infant* ; Infant, Newborn ; Malnutrition ; Mortality ; Nutritional Support ; Parturition ; Thoracic Surgery