OBJECTIVETo analyze symptoms, associated anomalies, diagnostic approach, and surgical procedures in patients with unroofed coronary sinus syndrome and atrioventricular canal defect.

METHODSThe clinical data of 20 patients with unroofed coronary sinus syndrome from September 1999 to October 2007 were reviewed retrospectively. There were 10 male and 10 female patients. The age ranged from 6 months to 38 years old, with a mean of (11.4 +/- 11.0) years old. The body weight ranged from 6.7 to 73.0 kg, with a mean of (28.4 +/- 21.3) kg. There were 18 cases of partial atrioventricular canal defect, 2 cases of complete atrioventricular canal defect, and 12 cases of common atrium. The initial diagnosis of unroofed coronary sinus syndrome was made by the surgeon at repair of a partial or complete atrioventricular canal defect in 20 patients. Complex unroofed coronary sinus with left superior vena cava (LSVC) directly draining into the left atrium was found in 11 cases, 1 case of LSVC was ligated, 10 cases were reconstructed the intraatrial baffle or the intracardiac tunnel to drain LSVC to right atrium. The other 9 patients with simple unroofed coronary sinus were repaired with other procedures. The associated cardiac lesions were corrected concomitantly.

RESULTSDeath occurred in 1 patient with complex congenital cardiac disease due to pulmonary infection. In the 14 early survivors, who had been followed up from 4 months to 3 years, there was no death and severe complications.

CONCLUSIONSWhen associated with a partial or complete atrioventricular canal defect, LSVC and a common atrium, unroofed coronary sinus syndrome should be considered as a possible additional finding. Repair according to the type of unroofed coronary sinus syndrome is effective.

Adolescent ; Adult ; Child ; Child, Preschool ; Coronary Sinus ; abnormalities ; Coronary Vessel Anomalies ; complications ; surgery ; Endocardial Cushion Defects ; complications ; surgery ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Retrospective Studies ; Treatment Outcome ; Young Adult

Endocardial Cushion Defects ; complications ; Female ; Humans ; Infant, Newborn ; Lung ; abnormalities ; Lung Diseases ; complications ; congenital

The partial endocardial cushion defect including ostium primum atrial septal defect and anterior mitral leaflet cleft, presents less significant clinical symptoms than complete endocardial cushion defect. But, as mitral insufficiency develops, cardiomegaly, congestive heart failure, pulmonary arterial hypertension appear. So, partial endocardial cushion defect has poor prognosis and is rarely seen in elderly patients. A 67 years old woman admitted at our hospital for operative treatment with partial endocardial cushion defect. She had increased pulmonary pressure of 45/22 mmHg, mean 32 mmHg. She had repair of ostium primum defect with patch, and the mitral valve was treated with valve replacement. Because advanced atrioventricular block developed postoperatively, she received permanent pacemaker.
Adult ; Aged ; Atrioventricular Block ; Cardiomegaly ; Endocardial Cushion Defects* ; Endocardial Cushions* ; Female ; Heart Defects, Congenital ; Heart Failure ; Heart Septal Defects, Atrial ; Humans ; Hypertension ; Hypertension, Pulmonary* ; Mitral Valve ; Mitral Valve Insufficiency ; Prognosis

The partial endocardial cushion defect including ostium primum atrial septal defect and anterior mitral leaflet cleft, presents less significant clinical symptoms than complete endocardial cushion defect. But, as mitral insufficiency develops, cardiomegaly, congestive heart failure, pulmonary arterial hypertension appear. So, partial endocardial cushion defect has poor prognosis and is rarely seen in elderly patients. A 67 years old woman admitted at our hospital for operative treatment with partial endocardial cushion defect. She had increased pulmonary pressure of 45/22 mmHg, mean 32 mmHg. She had repair of ostium primum defect with patch, and the mitral valve was treated with valve replacement. Because advanced atrioventricular block developed postoperatively, she received permanent pacemaker.
Adult ; Aged ; Atrioventricular Block ; Cardiomegaly ; Endocardial Cushion Defects* ; Endocardial Cushions* ; Female ; Heart Defects, Congenital ; Heart Failure ; Heart Septal Defects, Atrial ; Humans ; Hypertension ; Hypertension, Pulmonary* ; Mitral Valve ; Mitral Valve Insufficiency ; Prognosis

BACKGROUND: The surgical results of the patients with single ventricle(SV) associated with total anomalous pulmonary venous connection(TAPVC) has been reported with high mortality and morbidity due to their morphologic and hemodynamic complexity. A retrospective review was undertaken to report the outcome of the first-stage palliative surgery in our institution and to determine the factors influencing early death. MATERIAL AND METHOD: Between January 1987 and June 2002, 39 patients with SV and TAPVC underwent surgical intervention with or without TAPVC repair. Age at operation ranged from 1day to 10.7months (median age, 2.4month), and 29 patients were male. Preoperative diagnosis included 20 right-dominant SV, 15 SV with endocardial cushion defect, 3 left-dominant SV, and 1 tricuspid atresia. The pulmonary venous connection was supracardiac in 22, cardiac in 5, infracardiac in 11, and mixed in 1. Obstructed TAPVC was present in 11. First-stage palliative surgery was performed in 37. Repair of TAPVC, either alone or in association with other procedures, was performed during the initial operation in 31. Univariate and multivariate analyses were performed to analyze the risk factors influencing the operative death. RESULT: A mean follow-up period of survivors was 34.3+/-43.0(0.53~146.2)months. Overall early operative mortality was 43.6%(17/39). The causes were low cardiac output in 8, failure of weaning from cardiopulmonary bypass in 3, sepsis in 2, pulmonary hypertensive crisis in 1, pulmonary edema in 1, pneumonia in 1, and postoperative arrhythmia in 1. Risk factors influencing early death in univariate analysis were body weight, surgical intervention in neonate, obstructive TAPVC, preoperative conditions including metabolic acidosis, and need for inotropic support, TAPVC repair in initial operation, operative time, and cardiopulmonary bypass(CPB) time. In multivariable analysis, body weight, age at initial operation, surgical intervention in neonate, preoperative conditions including metabolic acidosis, need for inotropic support and CPB time were the risk factors. CONCLUSION: In this study, we demonstrated that the patients with SV and TAPVC had high perioperative mortality. Preoperative poor condition, young age, the length of operative and CPB time, the presence of obstructive TAPVC had been proven to be the risk factors. This fact suggests that the avoidance of unnecessarily additional procedures may improve the surgical outcomes of the first-stage palliative surgery. However further observation and collection of the data is mandatory to determine the ideal surgical strategy.
Acidosis ; Arrhythmias, Cardiac ; Body Weight ; Cardiac Output, Low ; Cardiopulmonary Bypass ; Diagnosis ; Endocardial Cushion Defects ; Follow-Up Studies ; Hemodynamics ; Humans ; Infant, Newborn ; Male ; Mortality ; Multivariate Analysis ; Operative Time ; Palliative Care ; Pneumonia ; Pulmonary Edema ; Retrospective Studies ; Risk Factors ; Sepsis ; Survivors ; Tricuspid Atresia ; Weaning

Annular pancreas is a rarely found gastrointestinal tract malformation, and is frequently associated with Down syndrome. Endocardial cushion defect is a type of congenital heart disease, commonly also related to Down syndrome. However, a combination of endocardial cushion defect with annular pancreas has not been reported previously. We recently experienced such a case in 2 month old boy. Moreover Down syndrome was excluded through physical examination and a chromosomal study. We think it difficult to obtain a complete explanation for this condition through the currently known pathogenesis of annular pancreas and endocardial cushion defect.
Down Syndrome* ; Endocardial Cushion Defects ; Endocardial Cushions* ; Gastrointestinal Tract ; Heart Defects, Congenital ; Humans ; Infant ; Male ; Pancreas* ; Physical Examination

BACKGROUND: Trisomy 16 in mice is considered to be an animal model for Down's syndrome in human. We studied the morphologic characteristics of the heart and the edema, and their significance to the teratogenesis in this animal model. METHODS: A total of 30 dams were sacrificed to bear 125 (61.9%) normal fetuses 35 (17.3%) abnormal fetuses and 42 (20.8%) resorptions. Cytogenetic study and morphological examination were performed using microdissection agar-mount sectioning histologic examination and scanning electron microscope. RESULTS: The crown-rump length was significantly shorter in abnormal (trisomic fetuses) than the normal (eusomic) fetuses. Trisomic fetuses showed massive edema at the back from the vertex to the lumbar area. Four-chamber view section of the agar-mount and histologic section showed a common atrioventricular valve bridging the left and the right atrioventricular junction. Scanning electron microscopic examination on atrioventricular valves showed three types of atrioventricular valves:five cases with common atrioventricular orifice, three cases with partitioned atrioventricular orifice and two cases with atresia of the right atrioventricular orifice. CONCLUSION: This study confirmed the presence of massive edema and cardiac anomalies in the mouse with trisomy 16. But there was morphologic diversity of cardiac anomaly in this model.
Animals ; Crown-Rump Length ; Cytogenetics ; Down Syndrome ; Edema* ; Endocardial Cushion Defects ; Fetus* ; Heart ; Humans ; Hydrops Fetalis ; Mice ; Microdissection ; Models, Animal ; Pathology* ; Teratogenesis ; Trisomy*

Chlorinated solvents are recognized as well-known drinking water contaminants and potent teratogens for developing embryos. Among theses compounds, tetrachloroethylene was studied to evaluate the teratogenic effects in detail. This study was also undertaken to understand the teratogenetic mechanisms by assessing the relationship between the physiological impairments and cellular degeneration including apoptosis induced by tetrachloroethylene, and the cardiovascular malformations. Fertilized white leghorn eggs (n=530) were incubated for 90~96 hours up to Hamburger-Hamilton stage 27~28. To the experimental group, tetrachloroethylene was dissolved in mineral oil at concentrations of 40 micrometer and 50 micrometer and was injected into the chorioallantoic membrane. One half of the control was injected with saline and the other half with mineral oil. The experimental animals are studied for ECG changes, and light and electron microscopic observation. Two silver thread electrodes were used to record the ECG. ECG changes were recorded for 6~30 minutes after tetrachloroethylene injections. In parallel studies, blood velocity through the dorsal aorta was measured by a pulse Doppler velocity meter with 20 MHz probe. The specimens were stained with hematoxylin and eosin for light microscopic study and the Hitachi H-800 EM was used for transmission electron microscopic evaluation. The embryos underwent formalin fixation on day 14, and the presence of malformations was observed by a microscopy. The results obtained are as follows: Repeated injections of 40~50 micrometer tetrachloroethylene appeared to have a strong influence on the formation of cardiovascular malformations. Various types of cardiac malformations were observed in the experimental group compared to control group, that were statistically significant (p<0.01) and many embryos also died. The malformations observed were large ventricular septal defect, endocardial cushion defect, double outlet right ventricle and truncus arteriosus associated with single ventricle. Large ventricular septal defect was most common type of cardiovascular abnormalities. Arrythymias including ventricular fibrillations were evident on ECG 6~30 minutes after the injections. Blood flow through the dorsal aorta was markedly decreased in concordance with the ECG changes. A large amount of individual cell death suggesting apoptosis was seen on light microscopic evaluation and the electron microscope revealed cellular degeneration of the embryonic myocardium. We concluded that tetrachloroethylene, like other chloride compounds has potent teratogenic effects. The cardiac malformations were probably caused by decreased blood flow due to physiofunctional changes and cellular necrosis associated with apoptosis. By including birds and mammals in further experiments, we hope to elucidate an embry-ological clue into how early cardiovascular malformations are caused.
Animals ; Aorta ; Apoptosis ; Birds ; Cardiovascular Abnormalities ; Cell Death ; Chick Embryo* ; Chorioallantoic Membrane ; Double Outlet Right Ventricle ; Drinking Water ; Eggs ; Electrocardiography ; Electrodes ; Embryonic Structures ; Endocardial Cushion Defects ; Eosine Yellowish-(YS) ; Formaldehyde ; Heart Septal Defects, Ventricular ; Hematoxylin ; Hope ; Mammals ; Microscopy ; Mineral Oil ; Myocardium ; Necrosis ; Ovum ; Silver ; Solvents ; Teratogens ; Tetrachloroethylene* ; Truncus Arteriosus ; Ventricular Fibrillation

BACKGROUND: From Sept. 1985 to Sept. 1997, 2,000 cases of open heart surgery (OHS) were performed in the Department of Thoracic & Cardiovascular Surgery, Pusan Paik Hospital, College of Medicine, Inje University. MATERIAL AND METHOD: Among the total of 2,000 cases of OHS, 1532 cases were congenital heart disease (CHD) and 468 cases were acquired heart disease (AHD). The age distribution was 9 days (4.0kg) to 68 years in CHD and 11 to 66 years in AHD. In 1532 cases of CHD, there were 1403 acyanotic cases and 129 cyanotic cases. RESULT: The CHD cases consisted of 940 ventricular septal defects (61.4%), 324 atrial septal defects (21.1%), 112 tetralogy of Fallot (7.3%), 46 pulmonary stenosis (3%), 38 endocardial cushion defects (2.5%), 15 valsalva sinus ruptures (1%), 4 transposition of great arteries (0.3%), 4 double outlet right ventricles (0.3%), and etc. Corrective operations were applied for congenital heart disease with a result of 3.1% hospital mortality. Of 468 AHD, 381 cases were valvular heart diseases, 48 ischemic heart diseases, 12 cardiac tumors, 8 annuloaortic ectasias, 16 dissecting aortic aneurysms and etc. In the 381 valvular heart diseases, there were 226 single valve replacements (36 aortic valve replacements (AVR), 188 mitral valve replacements (MVR), and 2 tricuspid valve replacements (TVR), among these were 71 cases of double valve replacements (AVR & MVR), 54 cases of MVR with tricuspid valve annuloplasty (TVA), and 18 cases of AVR, MVR with TVA. The total implanted prosthetic valves were 466. In MVR, 123 St. Jude Medical valves, 90 Carpentier-Edwards valves, 65 CarboMedics valves, 42 Sorin valves and 16 other valves were used. In AVR, 68 St. Jude Medical valves, 36 CarboMedics valves, 14 Carpentier-Edwards valves and 9 other valves were used. Coronary Artery Bypass Surgery (CABG) were performed in 48 cases. The patterns of bypass graft were 14 patients of single vessel graft, 21 patients of two vessels graft, 10 patients of three vessels graft and 3 patients of four vessels graft. CONCLUSION: The hospital operation mortality rate of congenital acyanotic, cyanotic and acquired heart diseases were 2.0%, 15.5%, and 5.1% respectively. The overall mortality rate was 3.6% (72/2,000).
Age Distribution ; Aortic Aneurysm ; Aortic Valve ; Busan ; Coronary Artery Bypass ; Dilatation, Pathologic ; Endocardial Cushion Defects ; Heart Defects, Congenital ; Heart Diseases ; Heart Neoplasms ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Heart Valve Diseases ; Heart Ventricles ; Heart* ; Hospital Mortality ; Humans ; Mitral Valve ; Mortality ; Myocardial Ischemia ; Pulmonary Valve Stenosis ; Rupture ; Sinus of Valsalva ; Tetralogy of Fallot ; Thoracic Surgery* ; Transplants ; Transposition of Great Vessels ; Tricuspid Valve

PURPOSE: The association of the tetralogy of Fallot(TOF) with the complete form of atrioventricular septal defect(AVSD) is rare cardiac malformation.The predominant clinical findings in both groups of cases were those associated with tetralogy of Fallot, but certain features need to be emphasized, however. METHODS: Forteen(2%) out of 659 patients with tetralogy of Fallot seen at the department of pediatrics, Seoul National University Hospital from January 1986 to June 1994, were associated with the complete form of AVSD. We reviewed clinical records, echocardiographic findings, angiographic findings and operative findings of patients. Our diagnostic criteria for TOF with complete AVSD are as follows. 1) Anterosuperior displacement of infundibular septum and RVOT obstruction. 2) Large (nonrestrictive) VSD and an overriding aorta. 3) Primum ASD with common AV valve.Goose-neck deformity of LVOT. RESULTS: 1) Only 14 cases(2%) of the 659 TOF patients were associated with complete AVSD. 2) The male to female ratio was 1:2.5. 3) The predominant features were those associated with TOF, but 9 patients(64.4%) had Down's syndrome. 4) The electrocardiogram showed left axis deviation except 2 patients. 5) Most common type among 14 patients with complete AVSD defect was Rastelli type C(78.5%). 6) Associated cardiovascular anomaly included PDA, ASD of secundum type, right aortic arch, left superior vena cava. 7) Among 12 patients who underwent operations, 9 patients are still alive and followed up. CONCLUSIONS: TOF associated with complete AV septal defect was a rare disease. There was a clinical difference between TOF and AVSD combined groups of case. The presence of Down's syndrome or left-axis deviation with TOF increases the likelihood of associated complete AVSD.Careful preoperative evaluation of these patient is essential to their management and successful operative treatment.
Aorta ; Aorta, Thoracic ; Axis, Cervical Vertebra ; Congenital Abnormalities ; Down Syndrome ; Echocardiography ; Electrocardiography ; Endocardial Cushion Defects ; Female ; Humans ; Male ; Pediatrics ; Rare Diseases ; Seoul ; Tetralogy of Fallot* ; Vena Cava, Superior