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1.
Clinical Pediatric Hematology-Oncology ; : 121-125, 2013.
Artigo em Coreano | WPRIM | ID: wpr-788494

RESUMO

Pericardial effusion (PcE) is one of the uncommon complications after hematopoietic stem cell transplantation (HSCT). Although many causes are related with PcE after HSCT, PcE after HSCT is usually late-onset and can be presented as a sign of acute or chronic graft-versus-host disease in allogeneic transplantation. Previous reports of PcE after autologous HSCT are very uncommon. Transplantation-associated thrombotic microangiopathy (TA-TMA) is a kind of renal microvascular complications after HSCT, which is similar to thrombotic thrombocytopenic purpura. The authors report a case of early-onset PcE, which maybe resulted from TA-TMA, after high-dose chemotherapy and autologous peripheral blood HSCT in a 4-year-old child with neuroblastoma.


Assuntos
Criança , Humanos , Tratamento Farmacológico , Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Células-Tronco Hematopoéticas , Neuroblastoma , Derrame Pericárdico , Pré-Escolar , Púrpura Trombocitopênica Trombótica , Microangiopatias Trombóticas , Transplante Homólogo
2.
Clinical Pediatric Hematology-Oncology ; : 121-125, 2013.
Artigo em Coreano | WPRIM | ID: wpr-130759

RESUMO

Pericardial effusion (PcE) is one of the uncommon complications after hematopoietic stem cell transplantation (HSCT). Although many causes are related with PcE after HSCT, PcE after HSCT is usually late-onset and can be presented as a sign of acute or chronic graft-versus-host disease in allogeneic transplantation. Previous reports of PcE after autologous HSCT are very uncommon. Transplantation-associated thrombotic microangiopathy (TA-TMA) is a kind of renal microvascular complications after HSCT, which is similar to thrombotic thrombocytopenic purpura. The authors report a case of early-onset PcE, which maybe resulted from TA-TMA, after high-dose chemotherapy and autologous peripheral blood HSCT in a 4-year-old child with neuroblastoma.


Assuntos
Criança , Humanos , Tratamento Farmacológico , Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Células-Tronco Hematopoéticas , Neuroblastoma , Derrame Pericárdico , Pré-Escolar , Púrpura Trombocitopênica Trombótica , Microangiopatias Trombóticas , Transplante Homólogo
3.
Clinical Pediatric Hematology-Oncology ; : 121-125, 2013.
Artigo em Coreano | WPRIM | ID: wpr-130754

RESUMO

Pericardial effusion (PcE) is one of the uncommon complications after hematopoietic stem cell transplantation (HSCT). Although many causes are related with PcE after HSCT, PcE after HSCT is usually late-onset and can be presented as a sign of acute or chronic graft-versus-host disease in allogeneic transplantation. Previous reports of PcE after autologous HSCT are very uncommon. Transplantation-associated thrombotic microangiopathy (TA-TMA) is a kind of renal microvascular complications after HSCT, which is similar to thrombotic thrombocytopenic purpura. The authors report a case of early-onset PcE, which maybe resulted from TA-TMA, after high-dose chemotherapy and autologous peripheral blood HSCT in a 4-year-old child with neuroblastoma.


Assuntos
Criança , Humanos , Tratamento Farmacológico , Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Células-Tronco Hematopoéticas , Neuroblastoma , Derrame Pericárdico , Pré-Escolar , Púrpura Trombocitopênica Trombótica , Microangiopatias Trombóticas , Transplante Homólogo
4.
Journal of Korean Medical Science ; : 1336-1342, 2010.
Artigo em Inglês | WPRIM | ID: wpr-187908

RESUMO

This study attempted to assess the incidence and outcome of anthracycline cardiotoxicity and the role of dexrazoxane as a cardioprotectant in childhood solid tumors. The dexrazoxane group included 47 patients and the control group of historical cohort included 42. Dexrazoxane was given in the 10:1 ratio to doxorubicin. Fractional shortening and systolic and diastolic left ventricular diameters were used to assess the cardiac function. The median follow-ups were 54 months in the dexrazoxane group and 86 months in the control group. The mean cumulative doses of doxorubicin were 280.8+/-83.4 mg/m2 in the dexrazoxane group and 266.1+/-75.0 mg/m2 in the control group. The dexrazoxane group experienced significantly fewer cardiac events (27.7% vs. 52.4%) and less severe congestive heart failure (6.4% vs. 14.3%) than the control group. Thirteen cardiotoxicities including one cardiac death and 2 congestive heart failures occurred in the dexrazoxane group, and 22 cardiotoxicities including 2 cardiac deaths and 4 congestive heart failures, in the control group. Five year cardiac event free survival rates were 69.2% in the dexrazoxane group and 45.8% in the control group (P=0.04). Dexrazoxane reduces the incidence and severity of early and late anthracycline cardiotoxicity in childhood solid tumors.


Assuntos
Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Antibióticos Antineoplásicos/efeitos adversos , Cardiomiopatias/induzido quimicamente , Fármacos Cardiovasculares/uso terapêutico , Estudos de Coortes , Intervalo Livre de Doença , Doxorrubicina/efeitos adversos , Ecocardiografia , Seguimentos , Insuficiência Cardíaca/induzido quimicamente , Neoplasias/tratamento farmacológico , Razoxano/uso terapêutico , Função Ventricular Esquerda/fisiologia
5.
Korean Circulation Journal ; : 23-30, 2010.
Artigo em Inglês | WPRIM | ID: wpr-161416

RESUMO

BACKGROUND AND OBJECTIVES: Although coronary artery obstruction, aortic insufficiency (AI), and pulmonary stenosis (PS) have been reported after arterial switch operation (ASO), limited long-term studies on ASO exist. Our study aimed to examine long-term outcomes after ASO for simple complete transposition of the great arteries (TGA). SUBJECTS AND METHODS: All 108 patients with simple complete TGA who underwent ASO at Seoul National University Children's Hospital between 1987 and 2004 were enrolled. We retrospectively reviewed the patients' medical records and the results of various functional and imaging studies. RESULTS: Among 108 cases of ASO for simple TGA, 96 have been followed-up through the present time (mean follow-up duration was 11.7+/-8.6 years: range= 4 to 23 years). The 20-year rates of freedom from significant AI, PS, and coronary obstruction were 78.6%, 67.8%, and 95.8%, respectively. AI showed a tendency to progress as follow-up time increased in 21.4% of the population studied (p=0.014); however, AS, PS, and PI showed no such progression. Late coronary artery occlusion was not associated with the initial coronary arterial pattern. Re-operations were done for 13 patients (13.5%) at an average of 8+/-4.3 years after ASO. The survival rate was 96%, while the re-operation-free was 90% at 10 years and 83% at 20 years. Most patients showed normal physical growth with good activity {98%; New York Heart Association (NYHA) class 1 activity} and normal development (96%). CONCLUSION: Although most patients showed normal physical growth and development after successful ASO, meticulous long-term follow-up is necessary because of progressive AI and coronary complications.


Assuntos
Humanos , Insuficiência da Valva Aórtica , Artérias , Vasos Coronários , Seguimentos , Liberdade , Crescimento e Desenvolvimento , Coração , Prontuários Médicos , New York , Avaliação de Resultados em Cuidados de Saúde , Estenose da Valva Pulmonar , Estudos Retrospectivos , Taxa de Sobrevida , Transposição dos Grandes Vasos
6.
Korean Circulation Journal ; : 36-41, 2010.
Artigo em Inglês | WPRIM | ID: wpr-161414

RESUMO

BACKGROUND AND OBJECTIVES: Only a few studies have specifically investigated the reasons for emergency room (ER) visits in patients with congenital heart disease (CHD). The aim of this study was to identify the major reasons for ER presentation among patients with CHD that were acutely and seriously ill at a tertiary medical center in Korea. SUBJECTS AND METHODS: All 368 admissions of patients with CHD via the ER from 2003 to 2008 were enrolled. We conducted a retrospective study with review of the medical records. RESULTS: Eighty two patients were newly diagnosed as having CHD. Their major presentations were: symptoms of heart failure (41.5%), murmur (31.7%), and cyanosis (18.3%). There were 286 visits that were cases with known CHD. Their major presentations were respiratory tract infection (24.1%, 2.7+/-4.1 years of age), dysrhythmia (16.4%, 16.7+/-9.5 years), symptoms of heart failure (14.3%, 7.6+/-9.4 years), aggravated cyanosis (5.6%, 0.8+/-1.4 years), protein-losing enteropathy (4.9%), hemoptysis (4.5%), drug side effects (4.1%), and infective endocarditis (3.0%). There were significant correlations between the age distributions and major modes of presentation. Surgical treatments were required within 1 month in 38%, and 2.7% of all patients died during hospitalization. The patient group with respiratory infections and CHD showed the highest mortality (5.8%). Atrial flutter was the most frequent arrhythmia (70.2%) and 70% of these patients were post-Fontan surgery condition. The causes of heart failure in the patients with previous surgical repair were: pulmonary hypertension, myocardial dysfunction, valve regurgitation, and uncorrected lesions. CONCLUSION: Improved understanding of the common problems in the ER can help prepare clinicians to manage patients that present with CHD.


Assuntos
Humanos , Distribuição por Idade , Arritmias Cardíacas , Flutter Atrial , Cianose , Emergências , Endocardite , Coração , Cardiopatias , Insuficiência Cardíaca , Hemoptise , Hospitalização , Hipertensão Pulmonar , Coreia (Geográfico) , Prontuários Médicos , Enteropatias Perdedoras de Proteínas , Infecções Respiratórias , Estudos Retrospectivos
7.
Korean Circulation Journal ; : 131-136, 2010.
Artigo em Inglês | WPRIM | ID: wpr-78780

RESUMO

BACKGROUND AND OBJECTIVES: With the increasing survival of preterm infants, pulmonary hypertension (PH) related to bronchopulmonary dysplasia (BPD) has become an important complication. The aim of this study was to investigate the characteristics and outcome of PH in preterm infants with BPD and to identify the risk factors for PH. SUBJECTS AND METHODS: We reviewed the records of 116 preterm infants with BPD cared for at a single tertiary center between 2004 and 2008. RESULTS: Twenty-nine (25%) infants had PH >2 months after birth. PH occurred initially at a median age of 65 days (range, 7-232 days). Severe BPD, a birth weight <800 g, long-term ventilator care and oxygen supplementation, a high ventilator setting, infection, and a patent ductus arteriosus (PDA) were related to PH based on univariate analysis (p<0.05). The infants who had longer oxygen supplementation were significantly more likely to have PH (odds ratio, 18.5; 95% confidence interval, 4.1-84.6; p<0.001). PH was improved in 76% of infants after a median of 85 days (range, 20-765 days). Four infants (14%) died. The death of 3 infants was attributed to PH. CONCLUSION: BPD was frequently complicated by PH. Although PH resolved in the majority of infants, PH in preterm infants with BPD can be fatal. Regular screening for PH and adequate management are required.


Assuntos
Humanos , Lactente , Recém-Nascido , Peso ao Nascer , Displasia Broncopulmonar , Permeabilidade do Canal Arterial , Concentração de Íons de Hidrogênio , Hipertensão Pulmonar , Recém-Nascido Prematuro , Programas de Rastreamento , Oxigênio , Parto , Fatores de Risco , Ventiladores Mecânicos
8.
Korean Journal of Pediatrics ; : 1029-1034, 2009.
Artigo em Inglês | WPRIM | ID: wpr-135427

RESUMO

Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology that affects children. There are few reports that describe the Epstein-Barr virus (EBV) as the possible infectious agent of KD. Here, we describe a case of KD in a 15-year-old boy complicated with giant coronary artery aneurysms, pericardial effusion, and splenic infarction. The clinical course of KD was refractory to intravenous gamma globulin and aspirin. Our patient also showed typical findings of concomitant EBV-associated infectious mononucleosis, such as hepatosplenomegaly and generalized lymphadenopathy, with EBV-positive atypical lymphoid hyperplasia. He improved dramatically after receiving intravenous methylprednisolone followed by oral prednisolone. Ultimately, the coronary artery aneurysms remained as the only sequelae. We report a rare case of adolescent KD with EBV-associated infectious mononucleosis and splenic infarction.


Assuntos
Adolescente , Criança , Humanos , Corticosteroides , Aneurisma , Aspirina , Doença da Artéria Coronariana , Vasos Coronários , gama-Globulinas , Herpesvirus Humano 4 , Hiperplasia , Mononucleose Infecciosa , Doenças Linfáticas , Metilprednisolona , Síndrome de Linfonodos Mucocutâneos , Derrame Pericárdico , Prednisolona , Infarto do Baço , Vasculite Sistêmica
9.
Korean Journal of Pediatrics ; : 1029-1034, 2009.
Artigo em Inglês | WPRIM | ID: wpr-135425

RESUMO

Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology that affects children. There are few reports that describe the Epstein-Barr virus (EBV) as the possible infectious agent of KD. Here, we describe a case of KD in a 15-year-old boy complicated with giant coronary artery aneurysms, pericardial effusion, and splenic infarction. The clinical course of KD was refractory to intravenous gamma globulin and aspirin. Our patient also showed typical findings of concomitant EBV-associated infectious mononucleosis, such as hepatosplenomegaly and generalized lymphadenopathy, with EBV-positive atypical lymphoid hyperplasia. He improved dramatically after receiving intravenous methylprednisolone followed by oral prednisolone. Ultimately, the coronary artery aneurysms remained as the only sequelae. We report a rare case of adolescent KD with EBV-associated infectious mononucleosis and splenic infarction.


Assuntos
Adolescente , Criança , Humanos , Corticosteroides , Aneurisma , Aspirina , Doença da Artéria Coronariana , Vasos Coronários , gama-Globulinas , Herpesvirus Humano 4 , Hiperplasia , Mononucleose Infecciosa , Doenças Linfáticas , Metilprednisolona , Síndrome de Linfonodos Mucocutâneos , Derrame Pericárdico , Prednisolona , Infarto do Baço , Vasculite Sistêmica
10.
Korean Circulation Journal ; : 382-385, 2009.
Artigo em Inglês | WPRIM | ID: wpr-151432

RESUMO

Pulmonary thromboembolism is a very rare event in children, but the mortality rate is reported to be approximately 10%. The majority of children with thromboemboli have multiple risk factors, such as a catheter-related thrombosis, an infection, and a congenital prothrombotic disorder. Hypereosinophilia is very rarely associated with pulmonary emboli in adults; however, this condition has not been reported in children. We present a 12-year-old boy who had a pulmonary thromboembolism and deep vein thrombosis associated with hypereosinophilia and thrombocytopenia. The thromboembolism was managed with anticoagulant therapy and the hypereosinophilia resolved spontaneously.


Assuntos
Criança , Humanos , Eosinofilia , Embolia Pulmonar , Fatores de Risco , Trombocitopenia , Tromboembolia , Trombose , Trombose Venosa
11.
Journal of Korean Medical Science ; : 77-82, 2008.
Artigo em Inglês | WPRIM | ID: wpr-157441

RESUMO

We examined whether alterations in vascular endothelial function and early structural changes in atherosclerosis are associated with microvascular complications in patients with type 1 diabetes mellitus (DM). Flow-mediated dilation (FMD) of the brachial artery and carotid intima-media thickness (IMT) measurement were performed in 70 young adults (aged 19 to 35 yr), 48 with type 1 DM, and 22 normal controls. Patients with diabetes had a lower peak FMD response (7.8+/-3.9 vs. 11.1 +/-1.9%, p<0.001) and increased IMT (0.51+/-0.10 vs. 0.42+/-0.07 mm, p<0.001) compared with controls. Twenty (41.7%) of the patients had microvascular complications including neuropathy, nephropathy, or retinopathy. In these complicated diabetic patients, we found a lower FMD response (6.1+/-2.5 vs. 9.9+/-3.5%, p=0.001) compared with diabetics without microvascular complications. The presence of microvascular complications was also associated with older age and longer duration of the disease. However, no differences were observed in IMT, body size, blood pressure, HbA1c, C-reactive protein, low-density lipoprotein or high-density lipoprotein cholesterol levels between complicated and non-complicated patients. Endothelial dysfunction and early structural atherosclerotic changes are common manifestations in type 1 DM, and endothelial dysfunction is thought to be an early event in the atherosclerotic process and important in the pathogenesis of microvascular complications.


Assuntos
Adulto , Feminino , Humanos , Masculino , Diabetes Mellitus Tipo 1/complicações , Angiopatias Diabéticas/etiologia , Endotélio Vascular/fisiologia , Microcirculação , Túnica Íntima/patologia , Túnica Média/patologia , Vasodilatação
12.
Korean Journal of Pediatrics ; : 1320-1323, 2008.
Artigo em Coreano | WPRIM | ID: wpr-103108

RESUMO

PURPOSE: The aim of this study was to investigate the epidemiologic status of Kawasaki disease (KD) in infants 6 months of age. RESULTS: A total of 1,739 patients 6 months old with data from 1,739 KD patients < or =6 months old showed significantly higher incidences of CA abnormalities and CA aneurysms in the younger patients.


Assuntos
Idoso , Feminino , Humanos , Lactente , Masculino , Idade de Início , Aneurisma , Aneurisma Coronário , Ecocardiografia , Estudos Epidemiológicos , Incidência , Coreia (Geográfico) , Síndrome de Linfonodos Mucocutâneos , Estudos Retrospectivos
13.
Korean Circulation Journal ; : 644-650, 2008.
Artigo em Inglês | WPRIM | ID: wpr-146100

RESUMO

BACKGROUND AND OBJECTIVES: Pulmonary arterial hypertension (PAH) is a rare disease with a poor prognosis. The aim of this study was to characterize PAH in pediatric patients by evaluating the patients demographics, clinical and hemodynamic variables, treatments, and outcomes. SUBJECTS AND METHODS: Sixty-five patients who were diagnosed with PAH at Seoul National University Children's Hospital between January 1985 and August 2007 were retrospectively reviewed. RESULTS: There was no difference in gender distribution (males, 33; females, 32). The mean age at the time of diagnosis was 5.7+/-5.2 years and the mean follow-up period was 6.3+/-5.5 years. The major causes of PAH were congenital heart disease (CHD) in 32 patients (49.2%) and idiopathic PAH in 11 patients (16.9%). The most common presenting symptom was dyspnea in 44 patients (67.7%). The mean cardiothoracic ratio was 58.9+/-8.3%, which decreased to 55.9+/-8.3% after vasodilator therapy (p=0.011). The mean pulmonary arterial pressure at the time of cardiac catheterization was 59.7+/-18.7 mmHg and the mean pulmonary vascular resistance was 14.9+/-9.7 wood units . m2. Forty-three of 65 patients (66.2%) had vasodilator therapy (prostacycline, sildenafil, and bosentan). A statistically significant decrease in tricuspid valve regurgitation velocity (4.8+/-0.8 m/sec vs. 3.6+/-1.0 m/sec, p=0.001), and an increase in diastolic dimension of the left ventricle (28.3+/-12.1 mm vs. 33.2+/-10.2 mm, p=0.021) on echocardiography before and after vasodilator therapy was demonstrated. The 5-, 10-, and 15-year survival rates were 96%, 92% and 65%, respectively. Six of 65 patients (9.2%) with PAH died. There was no significant correlation between outcome and the immediate response to the vasodilators. CONCLUSION: PAH is a devastating disease which is rare in children. PAH in children has a variable pattern cause and progression, the confirmation of which requires analysis of detailed registries from nationwide hospitals.


Assuntos
Criança , Feminino , Humanos , Pressão Arterial , Cateterismo Cardíaco , Cateteres Cardíacos , Demografia , Dispneia , Ecocardiografia , Seguimentos , Cardiopatias Congênitas , Cardiopatias , Ventrículos do Coração , Hemodinâmica , Hipertensão , Hipertensão Pulmonar , Piperazinas , Prognóstico , Purinas , Doenças Raras , Sistema de Registros , Estudos Retrospectivos , Sulfonas , Taxa de Sobrevida , Insuficiência da Valva Tricúspide , Resistência Vascular , Vasodilatadores , Madeira , Citrato de Sildenafila
14.
Korean Circulation Journal ; : 348-352, 2007.
Artigo em Inglês | WPRIM | ID: wpr-219478

RESUMO

BACKGROUND AND OBJECTIVES: Based on our previous studies on pulmonary atresia with ventricular septal defect (VSD) and major aorto-pulmonary collateral arteries (MAPCAs), a management strategy for this disease was formulated at our hospital in 1999. To evaluate this strategy, it was applied to the new patients and their outcomes were measured. SUBJECTS AND METHODS: The subjects were a cohort of newly diagnosed patients who were without any prior interventions and these patients were treated at our hospital from January 2000 to December 2003. The management strategy focused on promoting the growth of the pulmonary arterial confluence (PAC), if present, by performing a right ventricle-pulmonary artery conduit operation (RV-PA conduit). If the PAC was large or absent, then the management plan was discussed among the surgeons after performing an exhaustive work-up. RESULTS: Seventeen patients were enrolled and their age at the time of diagnosis ranged from 8 days to 34 months with a median age of 3 months. The initial surgical method varied from one-stage total correction (4 cases) to a conduit operation with or without additional MAPCAs procedures (13 cases). All but three of the patients survived their initial surgery. There were two late deaths. In addition to the 3 survivors from one-stage total correction, 4 patients underwent a staged total corrective operation. The 5 remaining patients are still a subtotal correction state or a palliated state. CONCLUSION: When a large PAC is present, one-stage total correction is the best surgical option. However, when the PAC is small, then RV-PA conduit without any vascular procedures may be a good alternative. This staged approach makes a larger PAC, which enables surgeons to create a much easier vascular anastomosis later on.


Assuntos
Humanos , Artérias , Estudos de Coortes , Diagnóstico , Comunicação Interventricular , Atresia Pulmonar , Sobreviventes
15.
Korean Circulation Journal ; : 489-496, 2007.
Artigo em Inglês | WPRIM | ID: wpr-212718

RESUMO

BACKGROUND AND OBJECTIVES: This study aimed to evaluate the usefulness of cardiopulmonary exercise test (CPET) in children with various congenital heart diseases (CHDs). SUBJECTS AND METHODS: Forty-eight children and adolescents (18 girls and 30 boys; mean+/-SD age, 12.6+/-0.5 years) with CHD who had undergone corrective surgery performed CPET using a programmable treadmill. The participants were divided into 4 groups based on the surgery performed: Fontan operation (12 patients), total correction for tetralogy of Fallot (13 patients), repair for coarctation of aorta (11 patients), and corrective surgery for simple CHDs (12 patients). Patients with simple CHDs who had undergone an early surgery were selected as controls. RESULTS: Progressive exercise time was the longest in the simple CHD group than in the other groups (10.6+/-2 versus 8.2-8.7 min, p=0.122). Absolute and predicted values of mean peak oxygen consumption were the lowest in the Fontan operation group (p=0.220 and 0.091, respectively). The coarctation of aorta group exhibited a prominent blood pressure response, that is, an elevation in the systolic blood pressure relative to that in the other groups at rest (131.7+/-18.8 versus 114.4-121.3 mmHg) and at peak exercise (196.5+/-41.5 versus 146.8-184.3 mmHg). CONCLUSION: CPET can be a useful tool to clarify the exercise capacity and reflect the hemodynamic status of patients who have undergone surgery for various CHDs and other heart diseases.


Assuntos
Adolescente , Criança , Feminino , Humanos , Coartação Aórtica , Pressão Sanguínea , Teste de Esforço , Técnica de Fontan , Cardiopatias Congênitas , Cardiopatias , Hemodinâmica , Consumo de Oxigênio , Tetralogia de Fallot
16.
Journal of Korean Medical Science ; : 633-640, 2007.
Artigo em Inglês | WPRIM | ID: wpr-48771

RESUMO

The purpose of this study was to analyze left ventricular (LV) torsion and untwisting, and to evaluate the correlation between torsion and other components of LV contraction in children with dilated cardiomyopathy (DCM). Segmental and global rotation, rotational rate (Vrot) were measured at three levels of LV using the twodimensional (2D) speckle tracking imaging (STI) method in 10 DCM patients (range 0.6-15 yr, median 6.5 yr, 3 females) and 17 age- and sex-matched normal controls. Global torsion was decreased in DCM (peak global torsion; 10.9+/-4.6degrees vs. 0.3+/-2.1degrees, p<0.001). Loss of LV torsion occurred mainly by the diminution of counterclockwise apical rotation and was augmented by somewhat less reduction in clockwise basal rotation. In DCM, the normal counterclockwise apical rotation was not observed, and the apical rotation about the central axis was clockwise or slightly counterclockwise (peak apical rotation; 5.9+/-4.1degrees vs. -0.9+/-3.1degrees, p<0.001). Systolic counterclockwise Vrot and early diastolic clockwise Vrot at the apical level were decreased or abolished. In DCM, decreased systolic torsion and loss of early diastolic recoil contribute to LV systolic and diastolic dysfunction. The STI method may facilitate the serial evaluation of the LV torsional behavior in clinical settings and give new biomechanical concepts for better management of patients with DCM.


Assuntos
Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Cardiomiopatia Dilatada/patologia , Ecocardiografia Doppler/métodos , Reprodutibilidade dos Testes , Disfunção Ventricular Esquerda/patologia
17.
Korean Journal of Pediatrics ; : 163-169, 2007.
Artigo em Coreano | WPRIM | ID: wpr-71852

RESUMO

PURPOSE: Late hypertension (HT) after coarctoplasty occurs commonly. This study was performed to evaluate the relationships between late HT and associated factors to it. METHODS: Retrospective analysis of the medical records of 22 apparently healthy patients who had coarctoplasty during infancy and were followed up for more than 10 years was done. Casual blood pressure (BP) was obtained in all and 24 hour BP monitoring was obtained in 16. Echocardiogrphic Doppler flow velocity at coarctation of aorta (COA) repair site and left ventricular (LV) indexes were also obtained. The ratio of the narrowest diameter of aortic arch and the diameter of the descending aorta at the diaphragmatic level was obtained through 3-dimentional computed tomography. RESULTS: HT by casual BP was detected in 7 of 22 and HT by 24 hour BP monitoring in 10 of 16. Three were normotensive by casual BP, but hypertensive by 24 hour BP monitoring. Males predominated in patients with HT by casual BP (P=0.022) and by 24 hour BP monitoring (P=0.036). Doppler velocity at COA repair site was higher in HT group by casual BP (P=0.006) and also in HT group by 24 hour BP monitoring (P=0.041). Especially, patients with a velocity of over 3 m/sec were more in HT group by casual BP (P=0.026). CONCLUSION: Even if casual BP is normal, there can be hypertension by 24 hour BP monitoring. So every postoperative patient has to get 24 hour BP monitoring. Especially in patients with Doppler velocity at COA site over 3 m/sec, 24 hour BP monitoring should be done to define HT.


Assuntos
Humanos , Masculino , Aorta Torácica , Coartação Aórtica , Pressão Sanguínea , Hipertensão , Prontuários Médicos , Estudos Retrospectivos
18.
Journal of the Korean Pediatric Cardiology Society ; : 171-182, 2006.
Artigo em Coreano | WPRIM | ID: wpr-181730

RESUMO

PURPOSE: Based on our previous studies on ventricular septal defect (VSD), pulmonary atresia (PA) and major aortopulmonary collateral arteries (MAPCA), a management strategy formulated. To evaluate this strategy, it applied to a selected group of patients and outcome measured. METHODS: A management strategy was to promote growth of pulmonary arterial confluence (PAC), initially by right ventricle-pulmonary artery (RV-PA) conduit operation, later by balloon and or surgical angioplasty. Unifocalization performed only after PAC became big enough. If PAC absent, management plan discussed with surgeons after complete work-up. This strategy applied to four consecutive patients, initially diagnosed to have VSD+PA with MAPCA from January 2000 to December 2000. RESULTS: The age at diagnosis ranged from 8 days-2.5 months. PAC present in 3 children, all of whom underwent initial RV-PA conduit operation at the age range of 15 days-5 months. The remaining one patient not have PAC, underwent bilateral unifocalization at the age of 4 months. All had multiple operations (2.5 operations in average) and multiple interventions (4.5 interventional catheterizations in average) before reached to corrective or semi-corrective operation. All of them currently alive and have had either corrective operation (2 patients) or semi-corrective operation (fenestration in VSD patch in 2 patients). All but one asymptomatic and on no medication. CONCLUSION: Our management strategy for VSD+PA with MAPCA seems to work well. The cases with increased pulmonary blood flow needs meticulous plan to prevent both pulmonary vascular obstructive disease and naturally occurring pulmonary arterial stenosis and obstruction. However further study with more patients necessary.


Assuntos
Criança , Humanos , Angioplastia , Artérias , Cateterismo , Catéteres , Constrição Patológica , Diagnóstico , Comunicação Interventricular , Atresia Pulmonar
19.
Korean Circulation Journal ; : 723-731, 2006.
Artigo em Coreano | WPRIM | ID: wpr-197993

RESUMO

BACKGROUND AND OBJECTIVES : Anti-endothelial cell antibodies (AECA) are found in the sera of many patients with Kawasaki disease (KD). In this study, the pathogenic role of AECA in the development of coronary arterial lesions of KD was investigated. SUBJECTS AND METHODS : Serum IgM-AECA concentrations were measured in 22 KD patients. Cultured human coronary artery endothelial cells (HCAEC) were incubated with either acute or convalescent phase sera, and their expressions of intercellular adhesion molecule-1 (ICAM-1) assessed. IgM fractions of the sera were purified, and their ability to induce ICAM-1 mRNA and protein expressions evaluated. To address the signal transduction pathways involved in IgM-AECA-induced ICAM-1 expression, the blocking effect of four protein kinase inhibitors, PD98059, SB203580, dimethylaminopurine (DMAP) and parthenolide were measured. RESULTS : IgM-AECA was present in 14 out of 22 (64%) acute KD sera. ICAM-1 expression of HCAEC incubated with acute KD sera (117.1+/-46.7) and AECA-positive acute KD sera (143.3+/-37.5) were significantly higher than those of the convalescent KD sera (88.9+/-14.4, p<0.05) or AECA-negative acute KD sera (71.2+/-11.8, p<0.05), respectively. IgM-AECA from KD patients significantly induced ICAM-1 protein and mRNA expression. The upregulation of ICAM-1 expression was significantly inhibited by SB203580, DMAP and parthenolide, but not by PD98059. CONCLUSION : IgM-AECA was detected in the sera of about 2/3 of acute KD patients, which activated endothelial cells by upregulation of ICAM-1 expression, possibly via p38, JNK MAPK and NF-kappaB signal transduction pathways. Thus, IgM-AECA may play a pathogenic role in the development of coronary arterial lesions in KD patients.


Assuntos
Humanos , Anticorpos , Anomalias dos Vasos Coronários , Vasos Coronários , Células Endoteliais , Imunoglobulina M , Molécula 1 de Adesão Intercelular , Síndrome de Linfonodos Mucocutâneos , NF-kappa B , Inibidores de Proteínas Quinases , RNA Mensageiro , Transdução de Sinais , Regulação para Cima
20.
Journal of the Korean Pediatric Cardiology Society ; : 284-291, 2006.
Artigo em Coreano | WPRIM | ID: wpr-80242

RESUMO

PURPOSE: To understand the pathophysiology of nonimmune hydrops fetalis, retrospective study was achieved. We evaluated cardiac anomalies and heart functions of newborns with nonimmune hydrops fetalis admitted to our neonatal intensive care unit. METHODS: A retrospective study was conducted on the newborn diagnosed as nonimmune hydrops fetalis (NIHF) between January 1995 and December 2005. To analyze cardiac structures and heart functions of the study population, echocardiographic data were used that carried out within 2 days after birth. RESULTS: During the study period, 29 newborns (18 males and 11 females; mean birth weight 2,877 g; mean gestational age 34.4 weeks) were identified as NIHF. There were 15 cases of structural cardiac anomaly, 5 cases of cardiomegaly, 2 cases of arrhythmia and one case of pericardial effusion. Among those patent ductus arteriosus were observed in 12 cases and there were 10 cases of patent foramen ovale (PFO) or atrial septal defect. Ebstein's anomaly with PFO and atrioventricular septal defect was one case respectively. Most cases appeared hypoalbuminemia and anemia. Difference of heart functions between neonatal survival group and neonatal death group had no statistical significance. CONCLUSION: Because there was no significant difference in cardiac function between neonatal survival group and neonatal death group, simple decline of the heart function is not sufficient for the explanation of pathophysiologic mechanisms. Nevertheless, NIHF remains a challenging entity to improve perinatal outcome.


Assuntos
Feminino , Humanos , Recém-Nascido , Masculino , Anemia , Arritmias Cardíacas , Peso ao Nascer , Cardiomegalia , Permeabilidade do Canal Arterial , Anomalia de Ebstein , Ecocardiografia , Forame Oval Patente , Idade Gestacional , Comunicação Interatrial , Coração , Hidropisia Fetal , Hipoalbuminemia , Terapia Intensiva Neonatal , Parto , Derrame Pericárdico , Estudos Retrospectivos
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