Left isomerism is characterized by bilateral left-sidedness and multiple associated cardiac and visceral anomalies. The clinical manifestation of left isomerism mainly depends upon the cardiac lesions. Occasionally an individual will have a normal heart and be presented with the extracardiac anomalies. A 3-year-old girl with a diagnosis of left isomerism was presented with pulmonary hypertension and intermittent hypoglycemia. Computerized tomography of the abdomen revealed absence of the portal vein and portosystemic shunt. The superior mesenteric and splenic veins joined as a common trunk, bypassed the liver and drained the left renal vein and hemiazygos vein. Her pulmonary hypertension was considered as a consequence of the portosystemic shunt. We report a case of left isomerism in association with absence of the portal vein and a review of literatures.
Abdomen ; Child, Preschool ; Diagnosis ; Female ; Heart ; Humans ; Hypertension, Pulmonary* ; Hypoglycemia ; Isomerism* ; Liver ; Portal Vein* ; Portasystemic Shunt, Surgical ; Renal Veins ; Splenic Vein ; Veins

PURPOSE: We evaluated Kawasaki disease(KD) in children in order to evaluate the clinical characteristics and coronary complication of infantile Kawasaki disease. METHODS: A total of 226 medical records of children with KD admitted to The Catholic University of Korea, St. Mary's Hospital, from 1994 to 2003 were retrospectively analyzed. RESULTS: The incidence of lymphadenopathy was statistically lower in infantile Kawasaki disease than in Kawasaki disease of children older than one year. The incidence of atypical Kawasaki disease in infant(32%) was greater than that of total patients(24%), but there was no statistically significant difference in two groups. The incidence of coronary complication in atypical Kawasaki disease in children younger than one year was 50%(6/12), which was greater than that of coronary complication in typical KD of children older than one year. CONCLUSION: Diagnostic difficulties in infantile Kawasaki disease are due to high incidence of atypical characteristics of Kawasaki disease. Yet, early treatment with IVGV and aspirin is necessary because of its high incidence of coronary complication.
Aspirin ; Child ; Humans ; Incidence ; Korea ; Lymphatic Diseases ; Medical Records ; Mucocutaneous Lymph Node Syndrome* ; Retrospective Studies

PURPOSE: The purpose of our study is to investigate the usefulness of B-type natriuretic peptide(BNP) assay as a biochemical marker of cardiovascular manifestations in children with Kawasaki disease(KD). METHODS: Blood was obtained to measure and compare plasma BNP concentrations in the acute phases of typical KD(n=56), atypical KD(n=25), viral febrile disease(n=30), Henoch-Shonlein purpura(n=20) and in the subacute phase of typical KD. Plasma BNP concentrations were measured using a commercial kit, Triage(R) BNP test kit. RESULTS: The mean BNP concentration in the acute phase of KD was significantly higher than in the acute phase of atypical KD, viral febrile disease and Henoch-Shonlein purpura(198.7+/-49.6 vs 55.9+/-10.4, 27.0+/-5.7, 13.7+/-3.1 pg/mL, P<0.001). In KD patients, the mean BNP concentration in the subacute phase was decreased significantly after the treatment with intravenous immunoglobulin and high-dose aspirin(78.0+/-23.5 pg/mL, P<0.001). The area under the Receiver Operating Characteristic curve using BNP level to differentiate KD from viral febrile disease was high: 0.826(95% CI, 0.735 to 0.918, P<0.001). A best cutoff of BNP concentrations for the differential diagnosis of Kawasaki disease was determined to be 30.8 pg/mL(sensitivity 76.8%, specificity 76.7%). CONCLUSION: This study shows the possibility that the rapid BNP assay is useful to detect cardiovascular manifestations associated with KD. Further studies are needed to clarify the mechanism by which the elevated levels of plasma BNP occur in the acute phase of KD.
Biomarkers* ; Child* ; Diagnosis, Differential ; Humans ; Immunoglobulins ; Mucocutaneous Lymph Node Syndrome* ; Natriuretic Peptide, Brain* ; Plasma ; ROC Curve ; Sensitivity and Specificity

PURPOSE: Tissue Doppler Imaging(TDI) is a modern echocardiographic technique used for quantitative assessment of myocardial tissue velocities. The purpose of this study is to estimate the left ventricular function by TDI in Kawasaki disease(KD). METHODS: TDI and conventional echocardiography were performed in 33 patients(3.2+/-2.4 years) in acute phase, 33 patients(4.2+/-4.0 years) in subacute phase of KD and 60 children(5.4+/-3.9 years) in controls. Systolic velocity, systolic integral, E' velocity, E' integral, A' velocity and A' integral at the base, mid-septum and the apex were measured. Ejection fraction(EF) and myocardial performance index(MPI) were estimated by conventional echocardiography. Myocardial function was compared in KD patients of the acute and subacute phase with controls by TDI and conventional echocardiography. RESULTS: E' integral at the base(4.59+/-3.87 cm vs 6.91+/-4.29 cm, P<0.05), mid septum(3.52+/-3.13 cm vs 5.49+/-3.47 cm, P<0.05), apex(2.52+/-2.40 cm vs 3.80+/-2.48 cm, P<0.05), A' integral at the mid septum(1.24+/-1.19 cm vs 2.11+/-1.64 cm, P<0.05) and apex(1.14+/-1.32 cm vs 1.90+/-1.59 cm, P<0.05) were lower in the acute phase patients than in the controls. Systolic integral at base, mid septum and apex were significantly lower in KD patients of the acute phase than in the controls. There was significant negative correlation with right coronary artery diameter and systolic integral, E' integral, A' integral at mid septum, E' integral, A' integral at apex in the acute phase of KD. EF and MPI were not significantly decreased in KD. CONCLUSION: Myocardial velocity was not different, but systolic and diastolic integrals at the left ventricular septum were significantly decreased in the acute phase of KD by TDI. The data will need to be compared with febrile controls.
Coronary Vessels ; Echocardiography ; Echocardiography, Doppler* ; Humans ; Mucocutaneous Lymph Node Syndrome* ; Ventricular Function ; Ventricular Function, Left* ; Ventricular Septum

PURPOSE: Obesity is a risk factor for cardiovascular morbidity and is frequently associated with coronary artery disease, hypertension, and diabetes mellitus. Conventional Doppler technique is limited by the absence of an adequate apical window to assess the transmitral flow in obese patients. Tissue Doppler imaging(TDI) and strain rate imaging(SRI) were performed to assess the influence of obesity on left ventricular systolic function. METHODS: In 13 obese and 15 normal adolescents aged 16 to 17 years, height, weight, body mass index(BMI), and obesity index(OI) were measured. Fat mass, body fat percent, and abdominal fat percent were estimated by bioelectrical impedance. Ejection fraction(EF) and myocardial performance index(MPI) were estimated by conventional echocardiography to evaluate left ventricular systolic function. Systolic myocardial velocity and strain rate were estimated by TDI and SRI. RESULTS: EF(63.8+/-6.4% vs 55.7+/-3.4%) was significantly lower in obese adolescents than normal controls. MPI(0.34+/-0.03 vs 0.48+/-0.06) was significantly higher in obese adolescents than normal controls. Systolic myocardial velocity and strain rate were significantly lower in obese adolescents than normal controls. Strain rate showed a negative correlation with arm circumference(r=-0.558, P<0.05) and BMI(r=-0.332, P<0.05). Strain rate was positively correlated with EF(r=0.557, P<0.05) at the base of left ventricle by SRI CONCLUSION: Significant decrease in left ventricular systolic function was noted in the moderate degree of obesity. SRI may be a more useful diagnostic tool in evaluating systolic dysfunction in patients with moderate degree of obesity.
Abdominal Fat ; Adipose Tissue ; Adolescent* ; Arm ; Body Weight ; Coronary Artery Disease ; Diabetes Mellitus ; Echocardiography ; Electric Impedance ; Heart Ventricles ; Humans ; Hypertension ; Obesity ; Risk Factors

PURPOSE: Myocarditis is an insidious inflammatory disorder of the myocardium. We investigated clinical characteristics, laboratory data, prognosis, and outcomes in patients with acute myocarditis. METHODS: We retrospectively analyzed the medical records of 52 myocarditis patients who were admitted to the SNUCH from 1985 to 2005. We compared progressed group (included mortality and dilated cardiomyopathy) with recovery group. RESULTS: The median age was 4.12 years. The median follow-up duration was 2.84 years. Sixteen patients(30.8%) recovered myocardial function. Twelve patients(23.1%) died; ten of them died because of fulminant myocarditis. Sixteen patients(30.8%) progressed to severe dilated cardiomyopathy. Twenty-one patients were treated with intravenous immunoglobulin(IVIG), and six patients took oral prednisone. Oral prednisone and IVIG showed no significant treatment effects(P=0.284, P=0.695). Six patients underwent temporary pacemaker due to complete atrioventricular block. Three patients underwent extracorporeal membrane oxygenation(ECMO), and 1 patient survived. Recently, survival rate for children with myocarditis was increased due to early intensive care and non-pharmacologic therapy(ECMO, ventricular assist device). CONCLUSIONS: Steroid and IVIG were ineffective treatments in acute myocarditis. Patients with myocarditis needed an early intensive care. We expect that early aggressive treatments could improve patients' outcomes.
Atrioventricular Block ; Cardiomyopathy, Dilated ; Child* ; Follow-Up Studies ; Humans ; Immunoglobulins ; Immunoglobulins, Intravenous ; Critical Care ; Medical Records ; Membranes ; Mortality ; Myocarditis* ; Myocardium ; Prednisone ; Prognosis ; Retrospective Studies ; Survival Rate

PURPOSE: Ebstein's anomaly is characterized by ongoing tricuspid regurgitation and right ventricular dysfunction. It is difficult to decide the proper time of surgical correction. The purpose of this study is to evaluate the advantage of early surgical correction of Ebstein's anomaly. METHODS: The clinical records of the sixteen patients, who had undergone tricuspid valvuloplasty with plication of the atrialized RV and followed-up for more than five years, were reviewed. Surgical outcome was analysed as regards year of surgery, age of the patients, echocardiographic grade of severity, Carpentier type, associated cardiac defects, and type of surgery. RESULTS: Of sixteen patients, eight patients underwent tricuspid valve repair before 5 years(8 months to 5 years, mean 2.4 years) of age(group A), and eight patients underwent tricuspid valve repair after 5 years(6 to 23 years, mean 12.9 years) of age(group B). Immediately after the operation, tricuspid incompetence regressed significantly in 5 of group A, and 5 of group B, and reduction in heart size was usual. After short and mid- term follow-up period, four of group A showed mild tricuspid valve regurgitation and right ventricular dilatation and relatively well preserved right ventricular function. However, all of group B showed severe right ventricular dilatation with severe tricuspid regurgitation and both ventricular dysfunction. All of group A were in New York Heart Association class I. However, in group B, only three were in class I. Two of group B died of right heart failure 10 and 16 years after the surgical correction, respectively. The year of surgery, mean follow-up periods, associated cardiac defects, and the preoperative echocardiographic grade of the severity were not different significantly between two groups. But there was a difference between two groups in the mean age of the patients, 12.7+/-5.2 years in group A, 21.7+/-5.5 years in group B. The surgical outcome was not closely related to Carpentier types of Ebstein's anomaly. CONCLUSION: Early repair of tricuspid valve may be advantageous to prevent progressive dilatation of the right side heart and to preserve right ventricular function after surgical correction. But the aging factor was not corrected in our study and it is still necessary to follow-up for a longer period in larger patients group.
Aging ; Dilatation ; Ebstein Anomaly* ; Echocardiography ; Follow-Up Studies ; Heart ; Heart Failure ; Humans ; Tricuspid Valve ; Tricuspid Valve Insufficiency ; Ventricular Dysfunction ; Ventricular Dysfunction, Right ; Ventricular Function, Right

PURPOSE: Doubly committed juxtaarterial ventricular septal defect(DCJA VSD) is relatively more frequent among Asians with VSD. The potential impairments of growth of pulmonic annulus and function of pulmonary valve are possible in postoperative patients due to the anatomic location of defect. But there are few reports regarding those impairments. So we evaluated growth of the pulmonic annulus and function of pulmonic valve after an operation. METHODS: Between Jan. 1995 and Dec. 1996, twenty seven patients with DCJA VSD were operated in Dong-A University Hospital. To compare with the study group, we selected 14 perimembranous VSD patients with similar ages and body weights who were operated during same period. We reviewed medical records and video tapes for 2 years-postoperative echocardiographic findings retrospectively. Main pulmonary artery annulus and aortic annulus ratio(MPA/Ao ratio) was used to evaluate the relative growth of pulmonary artery, and we also checked the severity of pulmonic stenosis related to valvar dysfunction. RESULTS: Two years after the operation, the MPA/Ao ratio decreased significantly in DCJA VSD patients(P value 0.001, and the decrease showed positive correlation with the defect size which is standardized by defect size/aortic annulus ratio. The mean gradient through pulmonic valve was 15.68+/-5.87, but it was not influenced by defect size or severity of growth retardation of MPA. CONCLUSION: The repair of DCJA VSD can induce the growth retardation of pulmonic annulus and valvar stenosis of pulmonary artery.
Asian Continental Ancestry Group ; Body Weight ; Constriction, Pathologic ; Echocardiography ; Heart Septal Defects, Ventricular* ; Humans ; Medical Records ; Pulmonary Artery* ; Pulmonary Valve ; Pulmonary Valve Stenosis ; Retrospective Studies

No abstract available.
Adolescent* ; Humans ; Hypertension*

No abstract available.
Adolescent* ; Humans ; Hypertension*