No abstract available.
Tricuspid Atresia*

No abstract available.
Coronary Sinus* ; Mitral Valve* ; Tricuspid Atresia*

tricuspid valve annulus diameter could not be measured in cases showing muscular tricuspid atresia and double-inlet LV. The initial RV dimensions quantified by CT were significantly lower for the hypoplastic RV group than for the control group (p < 0.001). The types of final surgery performed in the hypoplastic RV group were univentricular repair in 46 patients, biventricular repair in 4 patients, or an indeterminate surgery in 7 patients.CONCLUSION: Initial RV dimensions in infants with CHD and a hypoplastic RV can be quantified by CT and are substantially smaller than those in infants with tetralogy of Fallot.]]>
Follow-Up Studies ; Heart Defects, Congenital ; Heart Ventricles ; Humans ; Infant ; Tetralogy of Fallot ; Tricuspid Atresia ; Tricuspid Valve

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*

Isolated right ventricular hypoplasia, unassociated with severe pulmonary or tricuspid valvar malformations, is a rare primary congenital cardiac anomaly in which of the trabecular portion of right ventricle fails to develop. An atrial septal defect or a patent foramen ovale serves as an escape valve. We observed a 2-day-old neonate with this disorder who suffered from cyanosis. The diagnosis was made by Doppler-echocardiography which revealed marked reduction in right ventricular size and right-to-left shunt through the atrial septal defect. There was no other cardiac malformation such as pulmonary atresia or tricuspid atresia.
Cyanosis ; Diagnosis ; Foramen Ovale, Patent ; Heart Defects, Congenital ; Heart Septal Defects, Atrial ; Heart Ventricles ; Humans ; Infant, Newborn ; Pulmonary Atresia* ; Tricuspid Atresia* ; United Nations

The status of pulmonary circulation is regarded with utmost importance for the successful Fontan operation. The absence of unilateral pulmonary artery leads to decreased pulmonary vascular bed and elevated pulmonary vascular resistance which are the risk factors for Fontan operation. A 9-year-8-month-old female patient diagnosed as tricuspid atresia, pulmonary atresia with absent left pulmonary artery flow, received extracardiac conduit Fontan operation using 18 mm Gore-Tex graft. She was discharged on twenty sixth postoperative day with arterial O2 saturation of 70% on room air. On last follow up at 14 months after the operation, she was clinically well with O2 saturation of 91%.
Female ; Follow-Up Studies ; Fontan Procedure* ; Humans ; Polytetrafluoroethylene ; Pulmonary Artery* ; Pulmonary Atresia ; Pulmonary Circulation ; Risk Factors ; Transplants ; Tricuspid Atresia ; Vascular Resistance

A procedure descried by Fontan and Baudet in 1971 successfully bypassed the right ventricle in Tricuspid Atresia patients, after then many modification of the Fontan operation had been described and applied to many cyanotic complex heart patients. Forty patients with a variety of cardiac malformation underwent the Fontan operation at Seoul National University hospital (September 1978 to June 1986). The age at operation ranged 2 months to 18 years. Each number of cases according to basic cardiac anomaly was as follows; 17 in Tricuspid Atresia, 17 in Univertricular Heart, 2 in Double outlet of Right Ventricle, 2 in Transposition of the Great Arteries and 2 in Criss-cross heart. Total mortality rate after the Fontan operation was 50%. There was only one late death (>30 days). Mortality rate under 4 years of age (67%) was higher than that between 4 and 18 years of age (40%). we observed a significantly higher mortality for patients who, in the immediate postoperative period, had central venous pressure greater than 25cm H2O. 45% among survivals did not require further medication. Although mortality rate after the Fontan operation is much higher than that in the foreign literature, operative mortality will decline with the increased expirence of surgeon and the effective patients selection.
Arteries ; Central Venous Pressure ; Crisscross Heart ; Fontan Procedure* ; Heart ; Heart Ventricles ; Humans ; Mortality ; Postoperative Period ; Seoul ; Tricuspid Atresia

The conversion of failing Fontan circuit to total cavopulmonary connection(TCPC) is recommended as a therapeutic option in patients with late Fontan complications such as atrial arrhythmia, atrial enlargement, pulmonary venous obstruction, and ventricular dysfunction. Combined TCPC with extracardiac conduit and cryoablation of arrhythmia circuit is preferred for treatment of failing Fontan connection with atrial tachyarrhythmia. We report a case of conversion of atriopulmonary connection to extracardiac conduit Fontan and cryoablation of atrial arrhythmia circuit in a patient with tricuspid atresia, who also had ectopic atrial tachycardia, right atrial thrombi, pulmonary venous obstruction, and ventricular dysfunction. This patient and the parents were Jehovah's Witnesses; therefore, the patient underwent the procedure without blood transfusion.
Arrhythmias, Cardiac* ; Blood Transfusion ; Cryosurgery ; Humans ; Jehovah's Witnesses* ; Parents ; Tachycardia ; Tachycardia, Ectopic Atrial ; Tricuspid Atresia ; Ventricular Dysfunction

PURPOSE: To investigate the teratogenic effect of gamma-ray on the heart of chick embryo. METHODS: 50 rad, 100 rad, 150 rad, 200 rad, 250 rad, and 300 rad of gamma-ray were used to irradiate three days old chick embryos. The control group was not irradiated. After three weeks, the embryos were sacrificed and examined for cardiovascular malformation. RESULTS: The survival rate of the gamma-ray irradiated group was significantly lower than that of the control group(33.3-63.3% vs 76.4%, P=0.001). The cardiac malformation rate of the experimental group was 11.0%. In the control group, no congenital cardiac malformations were observed. The experimental groups had a significantly higher malformation rate(P=0.001). The types of malformation were ventricular septal defect, tricuspid atresia, Ebstein anomaly and aortic arch anomaly. In the gamma-ray irradiated group, the cardiac malformations were : 14 small ventricular septal defects (VSDs), five large VSDs, two tricuspid atresias, and one Ebstein anomaly. The higher the dose of radiation applied, the higher the incidence of cardiac malformation was noted. CONCLUSION: Gamma-ray irradiation of 3 days old chick embryos increased the rate of death and the rate of cardiac malformation significantly.
Animals ; Aorta, Thoracic ; Chick Embryo* ; Ebstein Anomaly ; Embryonic Structures ; Heart Septal Defects, Ventricular ; Heart* ; Incidence ; Survival Rate ; Tricuspid Atresia

Total 11 cases of tricuspid atresia were diagnosed radiographically at Seoul National University Hospital in recent two years since 1979. Some characteristic radiological findings were analyzed in chest P-A view and cineangiographies of right atrium and left ventricle. The results are as follows; 1. Among the 11 cases, 4 cases were male and 7 cases were female. Age distribution was from 2 months to 19 years and mean age was 3 1/2 years. 2. Anatomaic types of those cases according to Keith's classification were as follows; type Ia in 2 cases, type Ib in7 cases, type Ic 1 case and type IIb in 1 case. 3. The cineangiographic features of tricuspid atresia are sequential filling of contrast media in the order of right atrium, left atrium, and left ventricle, and triangular filling defect at the base of the heart in all cases, and right atrial dimple in two cases. 4. It is essential for the diagnosis of tricuspid atresia to undertake cineangiographies of right atrium and left ventricle, and the interrelationship of great arteries.
Age Distribution ; Arteries ; Cineangiography ; Classification ; Contrast Media ; Diagnosis ; Female ; Heart ; Heart Atria ; Heart Ventricles ; Humans ; Male ; Seoul ; Thorax ; Tricuspid Atresia