Infrahepatic interruption of the inferior vena cava with azygos continuation is relatively infrequent cardiovascular developmental anomaly occurring both in association with congenital heart disease and as an isolated anomaly of no hemodynamic importance. During the past 5 years, we observed 38 cases of infrahepatic interruption of IVC with azygos continuation out of 2,397 cases of congenital heart disease catheterized at Yonsei Cardiovascular Center. We conducted the study with a view point of position of the heart and abdominal organs and segmental analysis of the underlying congenital heart disease. We also analysed the associated exracardiac vascular anomalies The following results wer obtained: 1) The incidence of this anomaly among congenital heart disease was 1.7% and the sex ratio 1.4:1. Twenty four cases(63.1%) was below 5 years of age and 30 cases (79.0%) had cyanosis. 2) We observed 21 cases (55.2%) with the malposition of the heart and 17 cases (44.8%) with malposition of the abdominal organs. The ventricular loops revealed D-loop in 25 cases, L-loop in 5 cases and in the remaining 7 cases, it was uncertain. Eighteen showed normally related great arteries and malposition was present in another 18 cases. 3) Associatcd cardiovascular anomalics wcre as follows: right-sidcd aortic arch: 12 cases, patentductus arteriosus: 10 cases, pulmonic stenosis: 19 cases and bilateral superior vena cava: 9 cases. In conclusion, the presence of this anomaly should alert one to seek the severeintracardiac anomalies which are usually associated with it. Also, it is essential that, if any patient with this anomaly should come to thoracotomy, the azygos vein be not sacrificed.
Aorta, Thoracic ; Arteries ; Azygos Vein ; Catheters ; Cyanosis ; Heart ; Heart Defects, Congenital* ; Hemodynamics ; Humans ; Incidence ; Pulmonary Valve Stenosis ; Sex Ratio ; Thoracotomy ; Vena Cava, Inferior ; Vena Cava, Superior

No abstract available.
Diatomaceous Earth* ; Pneumoconiosis*

No abstract available.
Aneurysm* ; Child* ; Diagnosis* ; Echocardiography* ; Humans

The pressure and resistance of the pulmonary artery are the most important factors when evaluating the congenital heart disease. They are easily measured by the cardiac catheterization, but this procedure is a invasive technique, and difficult to perform repeatedly. There were some reports to estimate the pulmory artery pressure by using non-invasive techniques, such as the echocardiography. The accuracy and the application of the estimate derived from this technique is still a matter of controversy. We estimate the right ventricular pressure by the real time two dimensional echocardiography. The way to measure accuracy for this technique is by of camparing the estimate the right ventricular pressure derived by the echocardiograpy against the right ventricular pressure measured by the cardiac catheterization. The diameter of the left ventricle in the plane of the papillary muscles in measured by short axis view from parasternal positions at the end-systolic phase. The diameter between the interventricular sulcus[B] and the longest vertical from to the interventricular septum[C] are measured. The relationship between the ration, C/B by the two-dimensional echocardiography and the RVP/LVP by the cardiac catheterization is relatively well correlated(r=-0.88). Thus we may conclude that measuring the right ventricular pressure by using the two-dimensional echocardiography is relatively accurate and should be used for evaluating the congenital heart disease.
Arteries ; Axis, Cervical Vertebra ; Cardiac Catheterization ; Cardiac Catheters ; Echocardiography* ; Heart Defects, Congenital* ; Heart Ventricles ; Papillary Muscles ; Pulmonary Artery ; Ventricular Pressure*

Double outlet right ventricle, a complex of congenital cardiac anomalies in which both great arteries arise wholly or in large part from the morphologic right ventricle presents diverse clinical manifestations according to the morphological characteristics. Eighty cases with DORV were diagnosed and operated at Yonsei Cardiovascular Center from 1988 to 1992. The analysis of the morphological characteristics of this anomaly and comparative study of operative methods and mortality according to the morphological classification led to the following results: 1) The location of VSD varied to be subaortic in 40 cases, subpulmonic in 15, doubly committed in 6 and noncommitted in 19 cases. The great arteries were interrelated in D-malposition in 43 cases and L-malposition in 18 and 26 cases among above mentioned 61 cases revealed the side-by-side relationship. Ten of the cases showed normal position and the rest 9, A-malposition. 2) Pulmonary stenosis was found in 60 cases, and when associated with subpulmonic VSD, occurring less frequently. PDA and ASD secundum were associated in successional order and only 3 cases were verified to carry aortic arch anomalies such as coarctation and interruption, all with subpulmonic VSD. 3) Intraventricular tunnel repair was possible in every case associated with subaortic VSD and the postoperative prognosis was excellent. In comparison, most of the cases associated with other types of VSD in which only palliative surgery or various types of intraventricular repair were performed, the general outcome was poor. But total cavopulmonary connection, one of the physiological corrective methods, showing a better postoperative prognosis is being carefully considered for its broad adoptation in future. In conclusion, double outlet right ventricle, a pathophysiological complex of various anomalies, should be thoroughly evaluated for the morphological characteristics to decide the most appropriate types of operation and for consequent improvement of prognosis.
Aorta, Thoracic ; Arteries ; Classification ; Double Outlet Right Ventricle* ; Heart Ventricles ; Mortality ; Palliative Care ; Prognosis ; Pulmonary Valve Stenosis

No abstract available.
Echocardiography* ; Heart Ventricles* ; Tendons*

To evaluate the diagnostic accuracy of two-dimensional echocardiogrphy(2-D echo) in ventricular septal defect, location and size of the defects, estimated right ventricular systolic pressure and associated cardiac anomaly were compared to the operative findings in 139 children operated for correction of ventricular septal defect at Severance Hospital from Jan. 1983 to June 1987. In addition, postoperative complications and mortality cases were anlysed. The following results were obtained; 1) Perimembranous defects were 82 cases(66.1%), subarterial infundibular defects 33 cases(26.6%), and muscular defect was found in only 1 case(0.8%). The accuracy of 2-D echo in localizing the defects was 84.2%. 2) In 79.2% of the patients, the defects were moderate to large in size, and actual size measured at operation was larger than that obtained by echocardography in general. 3) The estimated right ventricular systolic pressure was correlated(r=0.650) with that measured at the operating field. 4) Combined cardiac anomalies were patent ductus arteriosus(22 cases), atrial septal defect(3 cases), valvular pulmonic stenosis(2 cases) and interventricular septal aneurysm(2 cases). The sensitivity of 2-D echo in detecting these anomalies was 65.5% and the specificity was 96.4%. 5) Among 32 patients who had postoperative complications, in two thirds, there were pulmonary complication including lung atelectasis(16 cases), pleural effusion(5 cases)and pneumonia(5 cases). 6) Operative mortality was 2.9%(4 cases). The causes of death were low cardiac output state due to left ventricular myocardial failure in 3 patients and respiratory failure from asphyxia in one case. In conclusion, with close cooperation with cardiac surgeons, there will be few problems in diagnosing and operating patients with ventricular septal defect on the basis of two-dimensional echocardiographic findings without invasive procedures, such as cardiac catheterization, even with pulmonary hypertension, unless Eisenmenger syndrome is complicated.
Asphyxia ; Blood Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Cardiac Output, Low ; Catheterization* ; Catheters* ; Cause of Death ; Child* ; Echocardiography* ; Eisenmenger Complex ; Heart Failure ; Heart Septal Defects, Ventricular* ; Humans ; Hypertension, Pulmonary ; Lung ; Mortality ; Postoperative Complications ; Respiratory Insufficiency ; Sensitivity and Specificity

PURPOSE: The authors reviewed clinical features, response to treatment and recurrence rate of Tolosa-Hunt syndrome. METHODS: A retrospective chart review was performed on 6 patients, who fulfilled the diagnosis for Tolosa-Hunt syndrome according to the International Headache Society (IHS) classification of 2004. RESULTS: Every patient had orbital pain as a first symptom, followed by cranial nerve paresis. The third cranial nerve was most commonly involved (83.3%), followed by the sixth nerve (50%), the forth nerve (16.7%), and the first branch of the fifth cranial nerve (16.7%). Two of the patients showed multiple cranial nerve paresis (33.3%, 2 out of 6). All patients received high-dose steroid therapy for more than 5 days, and all patients had resolution of orbital pain within 72 hours of treatment. Full recovery of cranial nerve paresis occurred on average in 2.3 months (3 days to 12 months). During the 29 months of follow-up, 2 patients (33.3%) had a recurrence episode. CONCLUSIONS: Tolosa-Hunt syndrome responds well to steroid therapy, and full recovery is possible with proper treatment. The exact diagnosis and treatment of Tolosa-Hunt syndrome is important. Because Tolosa-Hunt syndrome often recurs after full recovery, the authors suggest a minimum follow-up period of 2 years.
Cranial Nerves ; Follow-Up Studies ; Headache ; Humans ; Oculomotor Nerve ; Orbit ; Paresis ; Recurrence ; Retrospective Studies ; Tolosa-Hunt Syndrome ; Trigeminal Nerve

The persistent left superior vena cava (LSVC) is not rare cardiovascular developmental anomaly occurring both in association with congenital heart disease and as an isolated anomaly of no hemodynamic importance. We have studied 73 cases of the LSCV out of 1,060 cases of congenital heart disease catheterized at Yonsei Cardiovascular Center. We conducted the study with a view point of position of the heart and abdominal organs and segmental analysis of the underlying congenital heart disease. We also analysed the associated extracardiac vascular anomalies. The following results were obtained: 1) The incidence of this anomaly among congenital heart disease was 6.9% and 41 cases(56.2%) had cyanosis. 2) We observed 20 cases(27.3%) with the malposition of the heart and 17 cases(23.3%) with malposition of the abdominal organs. The ventricular loops revealed D-loop in 60 cases, L-loop in 7 cases and in the remaining 6 cases, it was uncertain. 3) With a view point of type of LSVC by Lucas & Krabill, type A was in 50 cases(68.5%), type D in 14 cases(19.2%), type B in 5 cases(6.8%) and type C in 4 cases(5.5%). 4) Associated cardiovascular anomalies were as follows: ventricular septal defect; 42 cases(57.5%), atrial septal defect; 33 cases(45.2%), patent ductus arteriosus; 27 cases(36.9%), and tetralogy of Fallot; 18 cases(24.7%). In conclusion, LSVC usually has no hemodynamic importance, but this cardiac anomaly is frequently combined with complex intracardiac anomalies. Therefore, it is important to making accurate diagnosis and successful management for preventing the risk of it.
Catheters ; Classification* ; Cyanosis ; Diagnosis ; Ductus Arteriosus, Patent ; Heart ; Heart Defects, Congenital ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Hemodynamics ; Incidence ; Tetralogy of Fallot ; Vena Cava, Superior*

Morphological evaluations accounting the associated anomalies were performed in the 60 cases of tricuspid atresia, diagnosed at Division of Pediatric Cardiology. The following results were obtained. 1) Twenty one out of the 6 cases had transposition of the great vessels, among which 8 cases were in complete A-transposition. 2) Seven cases were associated with pulmonany atresia, in 1 case, aorta arise from morphological right ventricle and in 6 case, aorta from morphological left ventricle. Pulmonary stenosis or pulmonary outflow obstruction was found in 52 cases except the rest 8 cases. 3) Four cases were associated with double outlet right ventricle and double outlet left ventricle in 1 case. In conclusion, for the classification of tricuspid atresia on the clinical basis, every possible interrelation of great vessels should be put into full consideration and pulmonary atresia, with very few exceptions, be separated as and isolated item due to the impracticability to verify the origin of pulmonary arteries.
Aorta ; Cardiology ; Classification* ; Double Outlet Right Ventricle ; Heart Ventricles ; Pulmonary Artery ; Pulmonary Atresia ; Pulmonary Valve Stenosis ; Tricuspid Atresia*