No abstract available.
Endocardial Cushion Defects* ; Endocardial Cushions* ; Hernia, Diaphragmatic*

No abstract available.
Endocardial Cushion Defects* ; Endocardial Cushions* ; Ultrasonics*

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

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: 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*

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

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

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