A clincal study was made on 383 adult patients with congenital heart disease who visited the Seoul National university Hospital during the period of March, 1961-July, 1979. 1. The incidence of congenital heart disease for the consecutive years was increasing in 1970s, especially in recent two years. 2. The sex incidence of congental heart disease was female 47.0%, male 53.0%, with male preponderance in tetralogy of Fallot, ventricular septal defect, coarctation of aorta, aortopulmonary window and female preponderance in atrial septal defect, patent ductus arteriosus. 3. Age distribution of congenital heart disease showed that 79.1% of all were below 30 years of age but atrial septal defect showed wide distribution below and above 30 years of age. 4. The incidences of each congenital heart disease were atrial septal defect 20.9%, tetralogy of Fallot 20.6%, ventricular septal defect 19.%, patent ductus arteriosus 12.0%, pulmonary stenosis 7.6%, trilogy of Fallot 1.8%, Ebstein's anomaly 1.8%, ventricular septal defect with pulmonary stenosis 1.3%, coarctation of aorta 1.3%, ventricular septal defect with aortic insufficiency 1.0%, transposition of great vessels 1.0%, in order and rare and various combined anomalies. 5. Average duration of illness in congenital heart disease was less than 15 years in majority, but duration from 11 years to 20 years was most common in tetralogy of Fallot. 6. Functional class of congenital heart disease is class II and I in majority, but class III was most common in tetralogy of Fallot. 7. Comparision of precatheterization diagnosis with postcatheterization diagnosis showed concordence in 92.2% and comparison of postcatheterization diagnosis with postoperative diagnosis showed concordence in 95.5% of cases. 8. Electrocardiographic findings in 5 major congenital heart disease were observed as follows; right ventricular hypertrophy 86.3%, right atrial enlargement 16.4% in tetralogy of Fallot, right ventricular hypertrophy 30.9%, incomplete right bundle branch block 27.9% in atrial septal defect, left ventricular hypertrophy 28.8%, right ventricular hypertrophy 10.6%, biventricular hypertrophy 10.6% in ventricular septal defect, left ventricular hypertrophy 63.2% in patent ductus arteriosus and right ventricular hypertrophy 70% in pulmonary stenosis. 9. Postoperative changes in electrocardiographic findings were observed in 36.1% of operated patients, of whom teteralogy of Fallot 61.7%, ventricular septal defect 48.0% were most common. Pstoperative electrocardiographic changes were complete right bundle branch block 58.3%, incomplete right bundle branch block 11.7%, myocardial ischemia 10%, nonspecific ST-T changes 10%, etc. in order.
Adult* ; Age Distribution ; Aortic Coarctation ; Bundle-Branch Block ; Diagnosis ; Ductus Arteriosus, Patent ; Ebstein Anomaly ; Electrocardiography ; Female ; Heart Defects, Congenital ; Heart Diseases* ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Heart* ; Humans ; Hypertrophy ; Hypertrophy, Left Ventricular ; Hypertrophy, Right Ventricular ; Incidence ; Male ; Myocardial Ischemia ; Pulmonary Valve Stenosis ; Seoul ; Tetralogy of Fallot ; Transposition of Great Vessels ; Trilogy of Fallot

A clincal study was made on 383 adult patients with congenital heart disease who visited the Seoul National university Hospital during the period of March, 1961-July, 1979. 1. The incidence of congenital heart disease for the consecutive years was increasing in 1970s, especially in recent two years. 2. The sex incidence of congental heart disease was female 47.0%, male 53.0%, with male preponderance in tetralogy of Fallot, ventricular septal defect, coarctation of aorta, aortopulmonary window and female preponderance in atrial septal defect, patent ductus arteriosus. 3. Age distribution of congenital heart disease showed that 79.1% of all were below 30 years of age but atrial septal defect showed wide distribution below and above 30 years of age. 4. The incidences of each congenital heart disease were atrial septal defect 20.9%, tetralogy of Fallot 20.6%, ventricular septal defect 19.%, patent ductus arteriosus 12.0%, pulmonary stenosis 7.6%, trilogy of Fallot 1.8%, Ebstein's anomaly 1.8%, ventricular septal defect with pulmonary stenosis 1.3%, coarctation of aorta 1.3%, ventricular septal defect with aortic insufficiency 1.0%, transposition of great vessels 1.0%, in order and rare and various combined anomalies. 5. Average duration of illness in congenital heart disease was less than 15 years in majority, but duration from 11 years to 20 years was most common in tetralogy of Fallot. 6. Functional class of congenital heart disease is class II and I in majority, but class III was most common in tetralogy of Fallot. 7. Comparision of precatheterization diagnosis with postcatheterization diagnosis showed concordence in 92.2% and comparison of postcatheterization diagnosis with postoperative diagnosis showed concordence in 95.5% of cases. 8. Electrocardiographic findings in 5 major congenital heart disease were observed as follows; right ventricular hypertrophy 86.3%, right atrial enlargement 16.4% in tetralogy of Fallot, right ventricular hypertrophy 30.9%, incomplete right bundle branch block 27.9% in atrial septal defect, left ventricular hypertrophy 28.8%, right ventricular hypertrophy 10.6%, biventricular hypertrophy 10.6% in ventricular septal defect, left ventricular hypertrophy 63.2% in patent ductus arteriosus and right ventricular hypertrophy 70% in pulmonary stenosis. 9. Postoperative changes in electrocardiographic findings were observed in 36.1% of operated patients, of whom teteralogy of Fallot 61.7%, ventricular septal defect 48.0% were most common. Pstoperative electrocardiographic changes were complete right bundle branch block 58.3%, incomplete right bundle branch block 11.7%, myocardial ischemia 10%, nonspecific ST-T changes 10%, etc. in order.
Adult* ; Age Distribution ; Aortic Coarctation ; Bundle-Branch Block ; Diagnosis ; Ductus Arteriosus, Patent ; Ebstein Anomaly ; Electrocardiography ; Female ; Heart Defects, Congenital ; Heart Diseases* ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Heart* ; Humans ; Hypertrophy ; Hypertrophy, Left Ventricular ; Hypertrophy, Right Ventricular ; Incidence ; Male ; Myocardial Ischemia ; Pulmonary Valve Stenosis ; Seoul ; Tetralogy of Fallot ; Transposition of Great Vessels ; Trilogy of Fallot

BACKGROUND: Aortopulmonary window (APW) is a very rare congenital heart anomaly, often associated with other cardiac anomalies. It causes a significant systemic to pulmonary artery shunt, which requires early surgical correction. Accurate diagnosis and surgical correction will bring good outcomes. The purpose of this study was to describe our 20-year experience of aortopulmonary window. MATERIAL AND METHOD: Between March 1985 and January 2005, 16 patients with APW underwent surgical repair. Mean age at operation was 157.8+/-245.3 (15.0~994.0) days and mean weight was 4.8+/-2.5 (1.7~10.7) kg. Patent ductus arteriosus (8), atrial septal defect (7), interrupted aortic arch (5), ventricular septal defect (4), patent foramen ovale (3), tricuspid valve regurgitation (3), mitral valve regurgitation (2), aortic valve regurgitation (1), coarctation of aorta (1), left superior vena cavae (1), and dextrocardia (1) were associated. Repair methods included 1) division of the APW with primary closure or patch closure of aorta and pulmonary artery primary closure or patch closure (11) and 2) intra-arterial patch closure (3). 3) Division of the window and descending aorta to APW anastomosis (2) in the patients with interrupted aortic arch or coarctation. RESULT: There was one death. The patient had 2.5 cm long severe tracheal stenosis from carina with tracheal bronchus supplying right upper lobe. The patient died at 5th post operative day due to massive tracheal bleeding. Patients with complex aortopulmonary window had longer intensive care unit and hospital stay and showed more morbidities and higher reoperation rates. 5 patients had reoperations due to left pulmonary artery stenosis (4), right pulmonary artery stenosis (2), and main pulmonary artery stenosis (1). The mean follow-up period was 6.8+/-5.6 (57.0 days~16.7 years)years and all patients belonged to NYHA class I. CONCLUSION: With early and prompt correction of APW, excellent surgical outcome can be expected. However, optimal surgical method needs to be established to decrease the rate of stenosis of pulmonary arteries.
Aorta ; Aorta, Thoracic ; Aortic Coarctation ; Aortic Valve ; Bronchi ; Constriction, Pathologic ; Dextrocardia ; Diagnosis ; Ductus Arteriosus, Patent ; Follow-Up Studies ; Foramen Ovale, Patent ; Heart ; Heart Defects, Congenital ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Hemorrhage ; Humans ; Intensive Care Units ; Length of Stay ; Mitral Valve Insufficiency ; Pulmonary Artery ; Reoperation ; Tracheal Stenosis ; Tricuspid Valve Insufficiency ; Vena Cava, Superior

The DiGeorge syndrome is a rare congenital abnormality consisting of aplasia or hypoplasia of the thymus and parathyroid glands resulting from malformation of the third and fourth pharyngeal pouches. This syndrome usually includes congenital cardiac anomalies and abnormal facial features. We experienced a case of congenital cardiac anomaly associated with DiGeorge syndrome. The patient was 1 month old boy weighing 3.5 kg. The congenital cardiac anomalies included ventricular septal defect, atrial septal defect, coactation of aorta, and patent ductus arteriosus. We performed one-stage operation with two separate incisions for these cardiac anomalies. Postoperative course was uneventful and the patient at 6 months of follow up is doing well.
Aorta ; Congenital Abnormalities ; DiGeorge Syndrome* ; Ductus Arteriosus, Patent ; Follow-Up Studies ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Infant, Newborn ; Male ; Parathyroid Glands ; Thymus Gland

A coronary arteriovenous fistula consists of a communication between a coronary artery and a cardiac chamber, a great artery or the vena cava. It is the most common anomaly that can affect coronary perfusion. Bilateral involvement of coronary fistula, however, constitutes an uncommon subgroup of coronary arteriovenous fistulas. We report a case which shows a rare occurrence of bilateral coronary arteriovenous fistula coexistent with atrial septal defect and pulmonic stenosis.
Arteries/abnormalities ; Arteriovenous Fistula/complications* ; Case Report ; Coronary Vessel Anomalies/complications* ; Heart Septal Defects, Atrial/complications* ; Human ; Male ; Middle Age ; Pulmonary Valve Stenosis/complications* ; Veins/abnormalities

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*

Congenital hypoplasia of the right ventricular myocardium, also known as parchment heart or Uhl's anomaly, is a rare congenital heart defect. It was first described in Oslers principles and Practice of Medicine in 1905 and reviewed by Segall We found 29 reported cases, but there was no reported case in Korea. Recently, we experienced a case of Uhls anomaly associated with patent ductus arteriosus, atrial septal defect, pulmonary atresia and tricuspid abnormality in a 3 month-old infant, which was confirmed by autopsy. We present a case with a brief review of litteratures.
Autopsy ; Ductus Arteriosus, Patent ; Heart ; Heart Defects, Congenital ; Heart Septal Defects, Atrial ; Humans ; Infant ; Korea ; Myocardium ; Pulmonary Atresia

A giant intracranial venous aneurysm(varix) is rare and has been associated mostly with vein of Galen fistulae. A 32-year-old man presents with intracranial hemorrhage which was caused by the rupture of supratentorial arteriovenous malformation with giant venous aneurysm. Surgical resection was performed without the occurrence of additional neurological deficits. We review the literatures on this clinical entity.
Adult ; Aneurysm* ; Arteriovenous Malformations ; Cerebral Veins ; Fistula ; Humans ; Intracranial Arteriovenous Malformations* ; Intracranial Hemorrhages ; Rupture

No abstract available.
Coronary Sinus* ; Heart Defects, Congenital* ; Heart Septal Defects, Atrial

Recently, the radionuclide angiocardiographic study had been enhanced by rapid development of gamma scintillation camera and computer system, and utilized as noninvasive diagnostic measurement of heart disease. We obtained the following results by the radionuclide angiocardiography with human serum albumin tagged with 99m-Technetium from two groups. One of them was congenital heart disease group(n=50, ventricular septal defect 20, atrial septal defect 6, pulmonary stenosis 2, tetralogy of Fallot 10, truncus arteriousus 1, transposition of great artery 1, patent ductus arteriosus 10) operated from Oct. 1984 to Jun. 1985, and the other was the normal human group(n=10) as control. 1) In the cases of left to right shunt, Qp/Qs values were calculated by th gamma variet fit curve. The preoperative values ranged from 1.441-3.00, the postoperative values were normal among all the patients except one(atrial septal defect, postop. Qp/Qs 2.335). 2) In the cases of right to left shunt, Qp/Qs values showed no apparent change between pre. andpostoperative period, possibly due to still remained collateral circulation and hardly obtained lower value(less than QP/QS 1.0) by computer system, But the double peak curve was noticed in the left ventricular time activity curve, which indicate right to left shunt, disappeared after the surgery, suggesting shunt closed. 3) The ejection fraction of left ventricle was obtained from the congential cardiac patients, ranged 0.38-0.65 before the surgery and 0.41-0.67 after the surgery. 4) The circulation time calculated from the first-pass radionuclide angiocardiography, ranged normal in the most congenital cardiac patients, but prolonged in the severe pulmonary hypertension.
Angiocardiography ; Arteries ; Collateral Circulation ; Computer Systems ; Ductus Arteriosus, Patent ; Gamma Cameras ; Heart Defects, Congenital* ; Heart Diseases ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Heart Ventricles ; Hemodynamics* ; Humans ; Hypertension, Pulmonary ; Pulmonary Valve Stenosis ; Serum Albumin ; Tetralogy of Fallot