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*

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*

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

A total of 120 infants, children, and teenagers, who were admitted to the Department of Pediatrics, Severance Hospital from April 1989 to August 1989, were examined by cross-sectional echocardiography. Right and left atrial and ventricular dimensions and areas were measured in the parasternal, apical, and subcostal views. Dimensions of the inferior caval vein, the pulmonary and the aorta were obtained in the parasternal, suprasternal, and subcostal views. The results were as follows : 1) Statistical analysis showed no difference between boys and girls. 2) For all parameters, a positive linear correlation was found with age, height, weight, and body surface area. The best correlation was with body surface area. 3) The correlation was further improved if the logarithmic values of the measurements and body surface area were considered. 4) Measurements by cross-sectional echocardiography are clinically useful, especially in the study of the right-sided cardiac structures that are difficult to evaluate with M-mode echocardiography, but the problems of reproducibility have to be taken into account.
Adolescent ; Aorta ; Body Surface Area ; Child* ; Echocardiography* ; Female ; Humans ; Infant* ; Pediatrics ; Reference Values ; Veins

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

Splenic anomalies are commonly associated with anomalies of cardiovascular system and of other organ systems. "Polysplenia" refers to the state in which the bulk of splenic tissue is divided into two and others more equal-sized splenic masses. We experienced a case of Polysplenia associated with complicated cardiovascular anomalies(Situs ambiguus, D-loop, normally related great arteries, interrupted inferior vena cave, bilateral superior vena cava, single atrium, common A-V canal, infundibular stenosis). We have presented this rare case and reviewed brief literatures.
Arteries ; Cardiovascular System ; Vena Cava, Superior

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*

Aneurysms of the coronary arteries are rare. They may be due to atheroselerosis, mucocutaneous lymph node syndrome, mycotic emboli, syphilis or trauma and occasionally they are congenital. The prognosis appears to be poor and death can occur suddenly from rupture of the aneurysm, peripheral coronary embolism or bacterial endocarditis. Recently surgical treatment has been successful. In a 5 year old patient with ventricular septal defect, two aneurysmal sacs located at the cardiac crux and just posterior to it was proved by coronary angiography and MRI, and successfully excised at operation.
Aneurysm* ; Child, Preschool ; Coronary Angiography ; Coronary Vessels* ; Embolism ; Endocarditis, Bacterial ; Heart Septal Defects, Ventricular ; Humans ; Magnetic Resonance Imaging ; Mucocutaneous Lymph Node Syndrome ; Prognosis ; Rupture ; Syphilis

BACKGROUND: The prostaglandin E1(PGE1) is a well known protent dilator of arteriosus. Maintaining of the patency of ductus arteriosus is crucial for the survival of patients suffering from ductus-dependent cyanotic congenital heart disease. We aimed to analyse the efficacy and the influencing factors upon PGE1 in patients suffering from this disease. METHODS: Between May 1991 and April 1993, 26 neonates and infants with ductus- dependent cyanotic congenital heart disease received on intravenous infusion of PGE1 in the Division of Pediatric Cardiology. Yonsei Cardiovascular Center. The result was a dramatic improvement in systemic arterial oxygen tension and oxygen saturation during infusion of PGE1with a dependency on the infusion of PGE1. We evaluated the arterial blood gas analysis both at the immediate pre-infusion stage and 2 hours after infusion. We aimed to analyse the factors which may influence the intravenous of PGE1to infant suffers of ducts-dependent cyanotic congenital heart disease, such as pulmonary atresia(n=14), severe pulmonary stenosis(n=7) or complete transposition of the great arteries(n=5). RESULTS: 1) There was a significant increase in PaO2 and Oxygen saturation 2 hours after the infusion of PGE1. This appeared to be unrelated to the different forms of the disease when compared with the pre-infusion values. 2) The infants' responsiveness of the ductus arteriosus appeared to be age related with significant differences emerging between the 2 group(p<.05). In infants younger than 9 hours old, the differences in PaO2 changes between pre-infusion and post-infusion of PGE1 were 16.3+/-3.7mmHg compared to just 10.4+/-0.4mmHg in infants older than 96 hours. 3) No significant difference emerged between an increase in PaO2or oxygen saturation relating to the shape of ductus arteriosus ; or the level of PaO2prior to the infusion. 4) The side effects of PGE1were as follows ; fever(84.6%),loose stool(61.5%), apnea(30.8%) and hypotension(15.4%), etc.. CONCLUSION: PGE1provides excellent medical palliation for infants suffering from ductus-dependent cyanotic congenital heart disease until the pulmonary arteries are large enough for a modified Blalock-Taussig shunt ; or until corrective surgery is possible.
Alprostadil ; Blalock-Taussig Procedure ; Blood Gas Analysis ; Cardiology ; Ductus Arteriosus ; Heart Defects, Congenital* ; Humans ; Infant* ; Infant, Newborn ; Infusions, Intravenous ; Oxygen ; Pulmonary Artery