Pulmonary atresia with major aorto-pulmonart collateral arteries(abbreviated as MAPCA in the following)as a route for pulmonary blood flow presents many problem in diagnosis and management and recently new approach to surgical management (unifocalization)was suggested and few reported it's application. Nineteen cases who had pulmonary with MAPCA, diagnosed at Seoul National University Children's Hospital from October 1987 to May 1989, were studied to lool at the relationship between MAPCA, central pulmonary artery and segmental pulmonary arteries. Also was observed the clinical course and operative management. The average number of MACPA in each patient was 4, two toward right lung and the other two toward left lung. MACPA arose most frequently from descending aorta and innominate artery contralateral to the side of aortic arch. Central pulmonary artery was identified in 86.7%. The number of bronchopulmonary segment connected to central pulmonary artery directly or indrectly was very variable so was the number of bronchopulmonary segment connected to MAPCA only. Three had a total correction and 14 had a various kinds of palliative operation once or twice. Seven had a called "unifocalization"(connection of MAPCA to central pulmonary artery) to correct arborization abnormality but the result was not satisfactory for technical reason.
Aorta, Thoracic ; Arteries* ; Brachiocephalic Trunk ; Diagnosis* ; Humans ; Lung ; Pulmonary Artery ; Pulmonary Atresia* ; Seoul

No abstract available.
Heterotaxy Syndrome*

BACKGROUND: To get the information about the clinical characteristics of the paroxysmal junctional tachycardia in children and to get the general principle in managing these children. METHOD: Analysis of the medical records of the 43 patients(male 30, female 13) with paroxysmal junctional tachycardia(JT) who had been followed-up in this hospital for a mean of 4.6 years(range 1 month up to 12 years) was done. RESULT: In 19 patients, JT started before 1 years of age : in 2, during gestational period, in 15, within 4 months of age, in 2, after 4 months of age. The next peak was 7 in the age of 5 years. The associated cardiac abnormalities were complex congenital defects in 2, tumor in 1, dilated and hypertrophic cardiomyopathy in 1 each. The significant hemodynamic disturbances during JT were noticed in 25. Among those whose surface electrocardiogram during JT were available, mean heart rate during JT was 232rpm(range 160-310) ; narrow QRS complex in 33 and wide in 1 ; P` wave in ST segment or T wave in 22. The delta waves were noticed after stopping JT and during followe up in 20. The types of delta waves were A in 7, B in 9, and indeterminate in 4. The different forms of delta waves unrelated to the degree of fusion were noticed in 6 ; disappearance or intermittent form of delta wave in 4. The efficacy of stopping JT was as follows : ATP 84.4%(38/45), diving reflex 50%(7/14), other vagal stimulation 71.4%(5/7), digoxin 72.7%(8/11), verapamil 54.5%(12/22), D/C cardioversion 62.5%(5/8), neosynephrine 100%(2/2). There were 2 deaths due to associated cardiac defects and 2 elective catheter ablations during the followe up period. The preventive medication with digoxin, beta blocker, and/or verapamil was succesful in 14, partially succesful in 11, failed in 14. The 7 persistent JT were treated with amiodarone in 3, with amiodarone and beta blocker in 1, with flecainide and digoxin in 1. In 1, surgical ablation of accessory pathway was done due to persistent JT. At present, JT do not recur or occur transiently without drugs in 29 ; with drugs, JT become controlled without recurrence in 4, with transient episodes in 4 and with intermittent episodes in 1. CONCLUSION: Althouh the JT in children is benign in most cases spite of the severity during the early period, JT is persistent in cases and needs potent drugs to control JT. Ablation of the foci may be necessary in these cases. Even in patients whose long-term results are benign, it is necessary to choose the optimal drugs to terminate and prevent the JT during the intervening period.
Adenosine Triphosphate ; Amiodarone ; Cardiomyopathy, Hypertrophic ; Catheters ; Child* ; Congenital Abnormalities ; Digoxin ; Diving ; Electric Countershock ; Electrocardiography ; Female ; Flecainide ; Heart Rate ; Hemodynamics ; Humans ; Medical Records ; Phenylephrine ; Recurrence ; Reflex ; Tachycardia* ; Verapamil

No abstract available.
Angiography* ; Heart Septal Defects, Ventricular* ; Pulmonary Artery* ; Pulmonary Atresia*

BACKGROUND AND OBJECTIVES: Idiopathic restrictive cardiomyopathy is a very rare and poorly recognized disease in children. This study is performed to describe the clinical course and to define potential predictors of outcome. MATERIAL AND METHOD: We reviewed the medical records and diagnostic studies of 11 consecutive patients during the period from Jan.1991 to Aug. 2000. RESULTS: The age at diagnosis was 1.2-13 years (median 7 years) and the duration of follow up was 3-90 months (median 3.6 years). All except one were symptomatic (dyspnea in ten, chest pain in four). The chest pain was associated with significant ST depression on both resting and exercise ECG, suggesting myocardial ischemia. Two had complete heart block as either initial or terminal event. Cardiac catheterization was done in nine ( mean pulmonary arterial wedge pressure 23+/-6mmHg, systolic pulmonary arterial pressure 47+/-14mmHg, mean right atrial pressure 11+/-9mmHg). Echocardiographic dimensional ratio of left atrium and aorta (LA/Ao) was 2.41+/-0.58. Mitral E/A inflow ratio was 2.72+/-1.42, E wave deceleration time was 93.6+/-44.2ms. During follow up, six died. The 2 year and 5 year cumulative survival rates were 54.5% and 18.8% respectively. The predictor for nonsurvivor were pulmonary venous congestion and LA/Ao >2.5(p<0.05). Verapamil was tried in 6 cases without favorable effect in all. CONCLUSION: Considerable numbers of restrictive cardiomyopathy have myocardial ischemia associated with ST depression and chest pain. The patients with pulmonary venous congestion and severe left atrial enlargement (LA/Ao>2.5) were at risk for death, requiring prompt definitive treatment such as cardiac transplantation.
Aorta ; Arterial Pressure ; Atrial Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Cardiomyopathy, Restrictive* ; Chest Pain ; Child* ; Deceleration ; Depression ; Diagnosis ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Heart Atria ; Heart Block ; Heart Transplantation ; Humans ; Hyperemia ; Medical Records ; Myocardial Ischemia ; Pulmonary Wedge Pressure ; Survival Rate ; Verapamil

No abstract available.
Aneurysm* ; Coronary Vessels* ; Mucocutaneous Lymph Node Syndrome*

No abstract available.
Angiography ; Coronary Angiography* ; Coronary Vessels ; Echocardiography* ; Mucocutaneous Lymph Node Syndrome*

The double-chambered right ventricle is congenital or acquired cardiac anomaly, which is characterized by aberrent hypertrophied muscular bands that divide the right ventriclar cavity into two different pressure chamber. This anomaly can complicate the natural history of patient with isolated ventricular septal defect. We experienced two cases of cases of acquired DCRV, which confirmed by two separate cardiac catherterization and angiography. The purpose of this report is to show that the aberrant muscular bands may be nonobstructive in early infancy and that the obstructive effect is developed with time as the bands become progressively more hypertrophied.
Angiography ; Heart Septal Defects, Ventricular ; Heart Ventricles* ; Humans ; Natural History

No abstract available.
Heart Septal Defects, Ventricular* ; Pulmonary Valve Stenosis*

No abstract available.
Humans ; Infant, Newborn ; Meningitis, Bacterial*