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A Case of Turner Syndrome with Coarctation and Subclavian Steal Syndrome by the Left Subclavian Artery from the Descending Aorta Below the Coarcted Segment.

Jang Won SEO ; Jae Hyun YU ; Hong Ryang KIL

Journal of the Korean Pediatric Cardiology Society.2005;9(2):405-408.

Juxtaductal coarctation is usually distal to the origin of the left subclavian artery, occasionally the orifice of the subclavian artery is involved in the coarctation and partially obstructed. An anomalous origin of the right subclavian artery from the descending aorta below the coarcted segment is also occasionally encountered. Reversed vertebral artery flow to a subclavian artery arising at or below a coarctation may produce the subclavian steal syndrome. Rarely both subclavian arteries arise beyond the coarctation. These abnormal subclavian arteries are important in clinical diagnosis and treatment. We report a case of Turner syndrome with coarctation, which present as juxtaductal type and the left subclavian artery from the descending aorta below the coarcted segment with reversed vertebral artery flow to a subclavian artery producing the subclavian steal syndrome. Resecton of coarctation segment and end-to-end anastomosis was successfully performed after transfer of left subclavian artery to distal segment of descending aorta.
Aorta, Thoracic* ; Aortic Coarctation ; Diagnosis ; Subclavian Artery* ; Subclavian Steal Syndrome* ; Turner Syndrome* ; Vertebral Artery

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A Case of Atypical Kawasaki Disease with Autoimmune Hemolytic Anemia.

Ja Hyun HONG ; Young Joo KWON ; Kyung Yil LEE ; Hong Ryang KIL ; Hyung Shin LEE ; Mi Hee LEE ; Byung Churl LEE

Journal of the Korean Pediatric Cardiology Society.2005;9(2):400-404.

Kawasaki disease(KD) is an acute febrile vasculitis with various clinical manifestations and the most common cause of acquired coronary arterial lesion in pediatric population. Occasionally, KD can be presented with some kind of unusual associations. of these, cases associated with autoimmune hemolytic anemia(AIHA) have been rarely reported worldwide. We report a case of KD associated with AIHA in the acute stage of disease and Parvovirus B19 infection, in a 4-month-old infant.
Anemia, Hemolytic, Autoimmune* ; Humans ; Infant ; Mucocutaneous Lymph Node Syndrome* ; Parvovirus ; Vasculitis

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Giant Coronary Aneurysm with Stenosis and Thrombus Formation due to Kawasaki Disease: Treatment with Graft Stent.

Woo Jung KIM ; Jong Geun SONG ; Byung Won YOO ; Yuria KIM ; Jae Young CHOI ; Jun Hee SUL ; Sung Kyu LEE

Journal of the Korean Pediatric Cardiology Society.2005;9(2):394-399.

Kawasaki disease is an acute systemic vasculitis of unknown origin. Coronary aneurysm or ectasia is one of the most serious complications of Kawasaki disease. The major complication of Kawasaki coronary disease is myocardial infarction caused by thrombus formation inside the aneurysm or by organic obstructive lesion following the regression of aneurysm. Percutaneous balloon angioplasty, rotational ablation, directional coronary artherectomy, stent insertion and coronary artery bypass graft can be used to treat coronary artery stenosis or occlusion. We describe a 6-year old boy who had an episode of Kawasaki disease with giant coronary artery aneurysm diagnosed at the age of 3 years. Surveillance echocardiogram showed giant coronary aneurysm with stenosis and large mural thrombus in the proximal portion of left main coronary artery. So we inserted two polytetrafluoroethylene(PTFE) covered graft stent without complication.
Aneurysm ; Angioplasty, Balloon ; Child ; Constriction, Pathologic* ; Coronary Aneurysm* ; Coronary Artery Bypass ; Coronary Disease ; Coronary Stenosis ; Coronary Vessels ; Dilatation, Pathologic ; Humans ; Male ; Mucocutaneous Lymph Node Syndrome* ; Myocardial Infarction ; Stents* ; Systemic Vasculitis ; Thrombosis* ; Transplants*

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A Case of Typical Kawasaki Disease with Vulvar Erythema and Edema in 21 Days Old Neonate.

Sin Weon YUN ; Kang Won RHEE ; Jong Hoon KIM ; Soo Ahn CHAE ; In Seok LIM ; Dong Keun LEE ; Eung Sang CHOI ; Byoung Hoon YOO

Journal of the Korean Pediatric Cardiology Society.2005;9(2):387-393.

Kawasaki diseases occur frequently in children beyond the age of 4 years, but classic Kawasaki disease in newborns are rare and have not been reported in Korea yet. We report a case of classic Kawasaki disease in neonate who is 21 days old girl and has no response to gamma-globulin although early diagnosis and treatment. Her clinical features met typical Kawasaki disease, but unusually she had a non suppurative labia major erythema and severe edema instead of cervical lymphadenitis. She had persistent fever and progressive carditis with coronary dilatation in spite of 3 doses of gamma globulin. Her fever was subsided after 3 doses of intravenous methyl prednisolone. In the subacute stage her other symptoms subsided and appeared typical desquamation of fingers and toe. Finally she had mild mitral regurgitation and diffuse coronary dilatation when she was discharged. Since one year follow up, with continuous aspirin medication, now she is very healthy, and her coronary dilatation was normalized and not any more valvular regurgitation.
Aspirin ; Child ; Dilatation ; Early Diagnosis ; Edema* ; Erythema* ; Female ; Fever ; Fingers ; Follow-Up Studies ; gamma-Globulins ; Humans ; Infant, Newborn* ; Korea ; Lymphadenitis ; Mitral Valve Insufficiency ; Mucocutaneous Lymph Node Syndrome* ; Myocarditis ; Prednisolone ; Toes

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Efficacy of Dexamethasone Therapy for Coronary Lesion after Immunoglobulin-retreated Kawasaki Disease.

Ji Yeon LEE ; Hee Joon KIM ; Yeong Sun JEONG ; Jo Won JUNG

Journal of the Korean Pediatric Cardiology Society.2005;9(2):379-386.

PURPOSE: To evaluate the outcome of coronary lesions for efficacy of dexamethasone therapy after additional immunoglobulin(IVIG) retreated in initial IVIG-resistant Kawasaki disease(KD). METHODS: Retrospective studies were performed on 438 cases of KD treated with one or two episode of high-dose IVIG and 24 cases with dexamethasone therapy after additional immunoglobulin(IVIG) retreated in initial IVIG-resistant Kawasaki disease(KD) at this hospital from June 2000 to January 2004. 2D echocardiogram was done at admission, 2 months later and every 2 or 3 months when coronary lesion had improved more than 2 months later. RESULTS: In 108(24.7%) of 438 cases with IVIG therapy only, 10(41.7%) of 24 cases with dexamethasone therapy after additional immunoglobulin(IVIG) retreated in initial IVIG-resistant Kawasaki disease(KD), coronary abnormality had been shown by initial 2D-echocardiogram examined at acute stage. In 10(2.3%) of 438 patients with IVIG therapy only, and in 3(12.5%) of 24 cases with additional IVIG retreatment, coronary lesions were still remained at follow-up echocardiogram. Even though it had tendency to increase the coronary lesions in the group with dexamethasone therapy after additional immunoglobulin(IVIG) retreated in initial IVIG-resistant Kawasaki disease(KD), there was no significant differences about the incidence of coronary lesions between two groups. There was no significant differences in age, sex, and other clinical findings between two groups. CONCLUSION: The combination therapy with high doses of IVIG and aspirin in KD is the standard treatment but not always effective. The dexamethasone therapy may be another treatment of choice after additional immunoglobulin(IVIG) retreated in IVIG- resistant Kawasaki disease(KD).
Aspirin ; Dexamethasone* ; Follow-Up Studies ; Humans ; Immunoglobulins, Intravenous ; Incidence ; Mucocutaneous Lymph Node Syndrome* ; Retreatment ; Retrospective Studies

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Plasma Brain Natriuretic Peptide Concentration and Tissue Doppler Imaging for Acute Febrile Phase in Patients with Kawasaki Disease.

Hee Joon KIM ; Yeong Sun JEONG ; Jo Won JUNG

Journal of the Korean Pediatric Cardiology Society.2005;9(2):371-378.

PURPOSE: The purpose of this study is to evaluate the role of plasma brain natriuretic peptide(BNP) concentraion in Kawasaki disease(KD) and to evaluate the relationship of BNP and Tissue doppler imaging(TDI) in ventricular function. METHODS: Plasma BNP concentration was obtained in the acute phases of KD(n=49) and non-KD(n=36). TDI and conventional echocardiography were performed in 49 patients in acute phase of KD. E' velocity, A' velocity at the lateral mitral annulus, interventricular septum, lateral tricuspid annulus were measured. RESULTS: The mean plasma BNP concentration in patients with KD in the acute phase was significantly higher than non-KD(61.1+/-100.9 pg/mL vs 13.2+/-9.5 pg/mL, P<0.05). There was significant negative correlation with BNP and E' velocity, E/E' ratio at lateral mitral annulus in the aucte phase of KD. CONCLUSION: The level of plasma BNP significantly increased in aucte phase of KD. But, there was no definite level of plasma BNP to diagnosis of KD. Diatolic ventricular function was decreased in acute phase of KD which BNP is elevated by TDI.
Brain* ; Diagnosis ; Echocardiography ; Humans ; Mucocutaneous Lymph Node Syndrome* ; Natriuretic Peptide, Brain* ; Plasma* ; Ventricular Function

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The Risk Factors of Carditis in Children with Kawasaki Disease.

Jang Won SEO ; Myong Sun RYU ; Hong Ryang KIL

Journal of the Korean Pediatric Cardiology Society.2005;9(2):365-370.

PURPOSE: The most common complication of Kawasaki disease(KD) is the development of coronary artery aneurysm. Other cardiac complication include myocarditis, pericarditis, mitral or aortic insufficiency, congestive failure, and arrhythmia. These non- coronary cardiac problems have important role on clinical course in acute phase of KD. Extensive researches have been performed about the pathogenesis, natural course and treatment of coronary artery lesion. But the studies about non-coronary cardiac complication have been limited, so we aimed to study the manifestation, incidence, and clinical predictor of carditis. METHODS: The retrospective studies was performed to the 48 KD patients diagnosed in Chungnam National University Hospital from May 1998 to September 2004. The patients were divided into two groups. Patients with carditis(11 patients) and without carditis(37 patients) before receiving the IVGG infusion were analyzed. RESULTS: There were no differences in age and sex between two groups. Patients with long duration of fever and high Harada score had the significant increase in the incidence of carditis before the initial IVGG infusion. No significant differences were not shown between the two groups in terms of WBC count, hemoglobin, hematocrit, platelet count, total protein, AST/ALT, LDH, CPK, troponin I, total cholesterol, and triglyceride before the initiL IVGG infusion. The incidence of carditis was high in patient with high CRP, low albumin, and high cholesterol and triglyceride. CONCLUSION: The possibility of significant carditis should be considered before the infusion of high dose IVIG, which may worse the caridac dysfunction. Some predictors of significant carditis such as long duration of fever, high Harada score, high levels of CRP, and low levels of albumin can be suggested.
Aneurysm ; Arrhythmias, Cardiac ; Child* ; Cholesterol ; Chungcheongnam-do ; Coronary Vessels ; Estrogens, Conjugated (USP) ; Fever ; Hematocrit ; Humans ; Immunoglobulins, Intravenous ; Incidence ; Mucocutaneous Lymph Node Syndrome* ; Myocarditis* ; Pericarditis ; Platelet Count ; Retrospective Studies ; Risk Factors* ; Triglycerides ; Troponin I

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Comparison of Intervals to Peak Values of Laboratory Findings in Patients with Coronary Complication to Those without Complication in Kawasaki Disease.

Bo Yeon CHOI ; Jin Hee OH ; Soon Ju LEE ; Ji Whan HAN ; Dae Kyun KOH ; Chang Kyu OH

Journal of the Korean Pediatric Cardiology Society.2005;9(2):357-364.

PURPOSE: Intravenous immune globulin(IVIG) as a treatment for the Kawasaki disease (KD) has reduced the coronary complications. But, some patients suffer from coronary complication despite early IVIG infusion, and it is difficult to discriminate the susceptible patients in the acute phase. It is also challenging to decide additional therapy in cases showing fever after IVIG therapy. We investigated the relationship between intervals from the onset of fever to the day of peak laboratory values and coronary complications. METHODS: We reviewed the charts of KD patients with coronary aneurysm(group A, n=13) and without aneurysm(group B, n=35). All patients got IVIG therapy early in the acute phase and additional therapy in cases fever recurred. We counted the days from onset of fever to the peak level of acute phase reactants and analyzed the differences between two groups with t-test. RESULTS: In the comparison of two groups, the mean intervals from the onset of fever to peak CRP level was 9.23+/-4.71 days in group A, 6.63+/-2.47 days in group B. The mean intervals to peak ESR was 13.31+/-7.06 days in group A, 8.37+/-3.01 days in group B. The mean intervals to highest platelets counts was 14.62+/-4.96 days in group A, 11.14+/-3.59 days in group B. All of these results showed statistically significant differences. CONCLUSION: Our results show that the KD patients with coronary aneurysm have longer intervals between the onset of fever to day of peak acute reactants in spite of the aggressive treatment than those without aneurysm. So, in cases of KD with relapsing fever in spite of IVIG and the acute reactants are in the course of increment, additional immune modulation therapy and short term follow ups with echocardiography would be needed.
Acute-Phase Proteins ; Aneurysm ; Coronary Aneurysm ; Echocardiography ; Fever ; Follow-Up Studies ; Humans ; Immunoglobulins, Intravenous ; Mucocutaneous Lymph Node Syndrome* ; Relapsing Fever

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Lipid Profile Changes During and after Kawasaki Disease.

Sung Hee CHO ; Kyung Yil LEE ; Ja Hyun HONG ; Jin Hee OH ; Ji Whan HAN ; Joon Sung LEE ; Byung Churl LEE

Journal of the Korean Pediatric Cardiology Society.2005;9(2):350-356.

PURPOSE: We wanted to evaluate the serial lipid profiles in children with Kawasaki disease(KD) for a period of 1 year and the association of lipid profiles with the development of coronary artery lesions(CAL). METHODS: The subjects were 43 children with KD admitted at The Catholic University of Korea, Daejeon St. Mary's Hospital from January 2000 to December 2002. Serial sera were collected 4 times for 1 year from all patients; at admission(acute stage), 1-2 months, 6 months and 1 year after disease onset. The values of total cholesterol(TC), high-density lipoprotein cholesterol(HDL-C), low-density lipoprotein cholesterol(LDL-C) and triglyceride(TG) were measured by an automatic analyzer. The sera from 59 age- matched healthy children were used as controls. RESULTS: The values of TC, HDL-C, and LDL-C in KD patients in the acute stage were significantly lower than those at 1-2 months, at 6 months and at 1 year, and also lower than those in controls. HDL-C levels have a tendency to increase with a time elapsed. There was no significant difference in lipid profiles after the acute stage between the KD patients and control groups. In comparison between the group with CAL and the group without CAL, although the TC value in the acute stage was lower and TG value at 1 year was higher in the group with CAL than that in the group without CAL, there was no lipid profile with statistical significance between the groups. CONCLUSION: Our findings suggest that the altered lipid profile in the acute stage of KD may reflect the severity of inflammation in KD, and it recover within 2 months of disease onset. Alterations in lipid profile after the acute stage do not seem to be associated with the risk factor for premature atherosclerosis.
Atherosclerosis ; Child ; Cholesterol ; Coronary Vessels ; Humans ; Inflammation ; Korea ; Lipoproteins ; Mucocutaneous Lymph Node Syndrome* ; Risk Factors

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Assessment of Ventricular Function Using Tissue Doppler Imaging in Kawasaki Disease.

Myung Wan JANG ; Jae Ryun PARK ; L Youngmin EUN

Journal of the Korean Pediatric Cardiology Society.2005;9(2):342-349.

PURPOSE: The purpose of this study is to evaluate ventricular functional changes in Kawasaki disease using tissue Doppler echocardiographic measures with conventional echocardiographic measures. METHODS: Tissue Doppler imaging and conventional Doppler echocardiography were performed in 69 patients with Kawasaki disease and 39 healthy children. Peak velocities of systolic(Sa) and early(Ea), late(Aa) diastolic motion of mitral annulus were obtained at the lateral and septal side from apical 4 chamber view. RESULTS: Sa(7.7+/-1.8 cm/s vs 8.8+/-2.1 cm/s, P=0.01) and Ea(15.7+/-3.2 cm/s vs 17.2+/-3.3 cm/s, P=0.03) measured at lateral mitral annulus were significantly lower in patients with Kawasaki disease. Aa(7.0+/-2.0cm/s vs 6.2+/-1.3 cm/s, P=0.01) measured at septal mitral annulus were statistically higher in patients with Kawasaki disease. There was no significant difference in coronary arteriopathy patients compared with non-coronary arteriopathy patients in Kawasaki disease. In conventional mitral and pulmonary venous flow velocities, there was significant difference of E in Kawasaki disease patients. The ratio of E/Ea and flow propagation velocity(Vp) demonstrated difference in Kawasaki disease patients. CONCLUSION: Peak mitral annular velocities obtained by tissue Doppler imaging were significantly altered in acute phase of Kawasaki disease. Tissue Doppler imaging with conventional Doppler echocardiography can be a promising method for evaluation of ventricular function in patients with Kawasaki disease.
Child ; Echocardiography ; Echocardiography, Doppler ; Humans ; Mucocutaneous Lymph Node Syndrome* ; Ventricular Function*

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