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

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

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*

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*

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

The congenital diverticulum of ventricle is a rare cardiac malformation arising from the left or right ventricle, the former being more common. Ventricular diverticulum is usually associated with other anomalies including intracardiac, midline thoracic, diaphragmatic and abdominal wall defect. The authors experienced a case of congenital diverticulum of left ventricle in nine month-old female infant. Left Blolock-Taussig shunt operation was done and total correction will be done later. Thereafter we presented a case with brief review of the related literatures.
Abdominal Wall ; Diverticulum* ; Female ; Heart Ventricles ; Humans ; Infant ; Pulmonary Atresia*

Bilateral coronary artery fistula is very uncommon congenital anomaly which occupy small percentage of all congenital coronary artery fistula. About 20% of the cases associated with additional congenital heart disease. And it may cause congestive heart failure, especially severely in neonate. We experienced a case of neonate who was 1 day-old-male with bilateral coronary artery-right ventricular fistula associated with atrial septal defect and congestive heart failure. The Patient was admitted because dyspnea, cyanosis since birth. 2D ecgicardiogram and cardiac catheterization revealed that a fistulous communicating ; forming a large aneurysm, was noted from bilateral coronary artery emptied into the right ventricle, and there was oxygen step-up in right ventricle.
Aneurysm ; Cardiac Catheterization ; Cardiac Catheters ; Coronary Vessels ; Cyanosis ; Dyspnea ; Fistula* ; Heart Defects, Congenital ; Heart Failure ; Heart Septal Defects, Atrial* ; Heart Ventricles ; Humans ; Infant, Newborn* ; Oxygen ; Parturition