Background: We retrospectively evaluated the clinical results of surgically managing patients with Ebstein's anomaly. Material and Method: Between Feb. f 984 and June 2006, 50 patients who underwent surgical treatment for Ebstein's anomaly at Yonsei Cardiovascular Center were retrospectively reviewed. The mean age of the patients was 26.9 years and 19 patients were male, Associated anomalies included atrial septal defect (33), patent ductus arteriosus (2), ventricular septal defect (1), and pulmonary stenosis (4), and 90%, (45/50) of the patients had more than a moderate degree of tricuspid regurgitation. Carpentier type A was present in 6 patients, type B in 26, type C in 14 and type D in 4. Ten patients were associated with WPW syndrome. Conservative surgery was possible in 31 patients (tricuspid annuloplasty, plication of the atrialized RV), Fontan's operation was peformed in 4 patients, tricuspid valve replacement was done in 12 and palliative surgery was done in 2 patients. Thirteen patients were associated with hi-directional cavopulmonary shunt (BCPS: one and a half ventricular repairs): 10 patients with WPW syndrome and 4 patients with atrial fibrillation underwent concomitant ablation. Result: The postoperative median NYHA functional class $(3{\rightarrow}1)$ and the mean cardio-thoracic ratio $(0.65{\rightarrow}0.59)$ were decreased significantly (p<0.001, p=0.014). The mean oxygen saturation $(86.6{\rightarrow}94.1%)$, and median TR grade $(4{\rightarrow}1)$ were also significantly improved (p=0.004, p<0.001). For comparison of BCPS and conservative surgery, the preoperative right ventricular pressure (33.0 vs. 41.3 mmHg), the ICU stay (2.80 vs. 1.89 days), the hospital say (10.6 vs. 16.8 days), and the left ventricular ejection fraction (64.3 vs. 72.8%) were statistically different. Postoperative mortality occurred in 3 patients (6%) due to biventricular failure in 2 patients and sepsis in the other patient. The mean follow up duration was 101.5 months, and one patient died of Fontan failure and 6 patients required reoperation (bioprosthetic degenerative change (2) and Fontan conversion (4)). The overall survival rate at 10 years was 90.2%, the freedom from reoperation rate and rate of cardiac related events were 78.9% and 49.2%, respectively. Conclusion: Surgical management of Ebstein's anomaly can be performed safely, and the associated BCPS may be helpful for high-risk patients. Adequate application of surgical management may increase the long-term survival with a reduced rate of reoperation.
Lee Sak;Park Han-Ki;Lim Sang-Hyun;Hong You-Sun;Chang Byung-Chul;Kang Meyun-Shick;Cho Bum-Koo;Park Young-Hwan
Journal of Chest Surgery
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v.38
no.10
s.255
/
pp.699-704
/
2005
Background: Coronary artery fistula is rare congenital anomaly, which account for $0.27\~0.40\%$ of all congenital heart diseases. We report the clinical observations of 45 patients with coronary artery fistula. Material and Method: We reviewed all patients presented with or without symptoms of coronary artery fistula between 1987 and 2004. Age ranged from 1 to 83 years. Twenty-six patients were female. The patients were divided into 2 groups according to the presenting symptoms. Twelve patients were in group A (asymptomatic) and 33 patients in group B (symptomatic). The most common clinical presentation in group B was angina (18) followed by dyspnea (7), atypical chest pain (5), syncope (1), fatigue (1), and palpitation (1). Twenty-five patients were associated with other cardiac diseases, which were atrial septal defect (4), coronary artery occlusive disease (6), hypertension (12), and valvular heart disease (2). Result: Patients were followed-up for a mean period of $64.8\pm62.7$ months. There was no complication related to coronary artery fistula during the follow-up period in both group. There was no mortality related to coronary artery fistula. Conclusion: In symptomatic patients, early surgical treatment is recommended considering the low perioperative morbidity. In asymptomatic patients receiving medical treatment, close follow up may be necessary.
Failure of mitral valve repair sometimes may be ascribed to severe or progressive alteration of the subvalvar apparatus. The aim of this study was to evaluate the effects of new chordae formation on mitral repair. Material and Method: From March 1997 to february 1999, 26 patients underwent mitral valve repairs with new chordae formation, we compared the symptoms and echocardiographic findings checked at preoperative state, and intraoperative period, discharge, and their last OPD visit. There were 45 male, and 11 female patients, and their mean age was 51.2$\pm$43.4 years. Etiology of the lesions was degenerative (18), rheumatic (6), infective (1) and ischemic (1). Chordal lesions were caused by rupture (18), elongation (6), and a combination of two causes (2). Associated lesions included atrial septal defect (2), tricuspid insufficiency (7), aortic insufficiency(4), and a combination of previous two factors (2). The number of mean artificial chordae was 3.6$\pm$1.6. Annuloplasty was per-formed in all cases. The CPB time was 182,1$\pm$63.7 minutes and the ACC time was 133.1$\pm$45.6 minutes. Aver-age follow up period was 49.2$\pm$7.1 months. Result: There was no early death. Early reoperation was performed in bud patients, one patient received mitral valve replacement because of an abnormality of annuloplasty and ano-ther received pericardiostomy due to postoperative pericardial effusion. During the follow up of 49.2$\pm$7.1 moths, there was no late mortality. Postoperative NYHA functional class checked at last OPD visit was class I in 22 patients (88%), class II in 2 (8%), and class III in 1 (4%). Regarding the late echocardiogram MR was absent in 20 patients (78%), 1 in 4 (15%), and II in 1 (4%). The postrepair mitral valve area was 2.2$\pm$0.35 $\textrm{cm}^2$ Conclusion: This study suggests that mitral valve repair using new chordae formation provides good early and mid term survivals and functional improvement. We think that the artificial chorda formation with polytetrafluoroethylene suture might be safe and effective technique for mitral valve repair.
Purpose : Long-term administration of anticonvulsants in children with epilepsy may cause short stature, hypocalcemia and low bone mineral density. This study was performed for the early detection of abnormal bone metabolism in children with epilepsy on taking anticonvulsants. Methods : Thirty children aged 5 to 16 years who were diagnosed with epilepsy were enrolled in this study. All had taken anticonvulsants for more than one year. Bone mineral density of lumbar vertebra was measured by dual-energy X-ray absorptiometry. Serum calcium, phosphorous, alkaline phosphatase, 25-hydroxycholecalciferol[$25(OH)D_3$], parathyroid hormone, and urine deoxypyridinoline were measured as biochemical bone markers. Bone age and body mass index were also calculated. Results : Bone minreal density, body mass index, bone age, and height were significantly decreased in two female patients who had taken two antiepileptic drugs for more than four years and they also had chronic diseases such as cerebral palsy with microcephaly, encephalomalacia, and microcephaly with atrial septal defect. Bone mineral density had significant positive correlations with body mass index(P<0.01) and bone age(P<0.01). Conclusion : This study showed chronic medication of anticonvulsants in children may cause low bone mineral density and short stature. Bone age and body mass index could be the important surrogate markers to find the population at risk. More studies, including a large study population and long term cohort study, will be required.
Pulmonary atresia with intact ventricular septum has continued to have a high surgical mortality and morbidity. This mAy attribute to the non-uniformity of the anomaly. We reviewed a total of 34 infants with pulmonary atresla and intact ventricular septum managed in this hospital between 1987 and 1995. Mean age and body weight were 57.2 (range, 3-208) days and 4.1 (range, B.3∼6.8) kg. The preoperative Z-value of the diameter of the tricuspid valve was less than -2 in 85.2% of patients and less than -4 in 33. 3% . It is well correlated w th right ventricular cavity size (n=27. r10.68, p< 0.05). Coronary artery-right ventricular fistulas were identified in 3 patients, and right ventricular dependency was suspected in 1 Over All hospital mortality was 23.5%(8/34), although it decreased to 16.6%(4124) in 1990s. Subsequent procedures were performed in 6 patients between 3 days and 58 months after Initial palliation : one bidirectional cavopulmonary shunt and 1 Fontan operation after systemic-pulmonary shunt, 3 transannular patch + atrial septal defect closure and 1 additional systemic-pulmonary shunt after polmonary valvectomy or valvotomy. Changes of Z-values of the diameter of tricuspid valve have been followed up in 11 patients between 1 and 66 months postoperatively. Z-values were increased In 5 out of 8 transannular right ventriculAr outflow tract enlargement group and in 1 out of 3 pulmonary valvectomy or valvotomy group. Our data suggest that tailoring a treatment to right ventricular cavity size and coronary anom lies may improve the surgical outcome. A Z-value of the tricuspid valve diameter could be used.
Purpose : Isolated noncompaction of the ventricular myocardium(INVM) is one of the unclassified cardiomyopathies that is characterized by numerous, excessively prominent trabeculations, and deep intertrabecular recesses. We performed this study to evaluate the clinical features of INVM in children. Methods : The medical records of 10 patients with INVM were reviewed. We analyzed the clinical manifestations, hemodynamics, pattern of inheritance, and long-term prognosis of INVM in children. Results : Age at diagnosis was $45{\pm}53months$(1 day-14 years) with follow-up lasting as long as 78 months. Most INVM was asymptomatic on diagnosis. Associated cardiac anomalies were noted in six patients(ventricualr or atrial septal defect, patent ductus arteriosus with mitral valve prolapse, or mitral valve cleft). Depressed or flat changes of T wave in lead II, III and aVF were observed on electrocardiography. Various arrhythmia including WPW syndrome with paroxysmal supraventricular tachycardia, third-degree atrioventricular block, and familial sick sinus node dysfuction were observed. The degree of trabeculation in INVM was significantly prominent from level of mitral valve to apex compared to age-matched control. Familial recurrences were noted in two patients. The systolic function of the left ventricle was decreased in 20% of patients during the follow-up period, but systemic embolism or ventricular tachycardia was not observed. Conclusion : INVM is not a rare disorder. The cardiac function may be deteriorated in children as well as adults during long-term follow up. Thus early diagnosis and long-term follow-up must be done. So, the nation-wide multicenter clinical study would be mandatory to evaluate the incidence, long-term prognosis, and establishment of objective diagnostic criteria of INVM.
Kim, So Youn;Chung, Hae Yul;Back, Hee Jo;Choi, Ic Sun;Cho, Chang Yee;Choi, Young Youn
Clinical and Experimental Pediatrics
/
v.45
no.12
/
pp.1512-1518
/
2002
Purpose : Twins have a higher mortality and morbidity than singletons. Co-twin with one fetal death is particularly at risk. We investigated the neonatal outcome of live co-twins when one fetus had died after the 20th gestational week, and associated risk factors. Methods : A retrospective study was performed in fifteen cases of twin pregnancy with single intrauterine fetal deaths after the 20th gestational week during the period from January 1996 to December 2000 at Chonnam University Hospital. Results : Gestational age was $33.7{\pm}3.2weeks$, birth weight was $1,992{\pm}592g$. Interval between one fetal death being detected and the delivery of a live co-twin was $32.4{\pm}29.5days$. There were 11 cases(73.3%) of premature babies less than 37 gestational weeks. Main causes of preterm delivery were preterm labor and premature rupture of membranes. Hematologic findings suggesting disseminated intravascular coagulopathy(DIC) were not found in all mothers before delivery, and was not associated with DIC and encephalomalacia of the live co-twin. Perinatal outcome of fifteen live co-twins was as follows : six were normal(40%), three were DIC(20.0%), three were encephalomalacia(20.0%), one suffered intrauterine growth retardation, there was one case of twin to twin transfusion syndrome, and one of congenital heart disease(atrial septal defect with pulmonary stenosis). The occurrence of DIC and encephalomalacia in live co-twins was not related to placental chorionicity, birth weight, gestational week, and the interval between the detection one fetal death and the delivery of a live co-twin. Conclusion : We could not find any maternal hematologic problems in twin pregnancies complicated by one fetal death. Twenty percent of live co-twins showed DIC and encephalomalacia. However, its associated risk factors were not found. We need to investigate more closely the cases of live co-twins with one intrauterine fetal death.
Purpose : The neonatal cardiac catheterization and angiogram for transcatheter therapy are still essential methods in congenital heart disease, so we reviewed our experience with neonatal cardiac catheterization over 10 years at a single institution. Methods : A retrospective review of all 139 neonatal catheterizations from January 1991 to December 2000 at Sejong Heart Institution was performed. The purpose of the catheterizations, ages, body weights and the complications by the exam was surveyed. Results : The mean age of our 139 patients was 14.9 days and the mean body weight was 3.3 kg. As for the diagnosis : TGA in 49 patients, PAIVS in 26 patients, DORV in 14 patients, PS in 14 patients, PAVSD in 11 patients. For the cardiac catheterization, general anesthesia was performed in 65% of total and fluoroscopy time was $20.1{\pm}14.5$ minutes during cardiac catheterization. In the interventional cardiac catheterization which was 75% of the total, the age and body weight were statistically the same but the irradiation time was longer than the diagnostic catheterization( P=0.001). There were 48 cases of atrial septostomy, 16 cases of balloon pulmonary valvuloplasty and 25 cases of transcatheter pulmonary valvotomy. Complications of cardiac catheterization were found in 16.3%, but there was no difference between interventional catheterization and diagnostic catheterization. Conclusion : Therapeutic cardiac catheterization in neonates is a relatively safe and effective method in congenital heart disease.
Background: Infective endocarditis shows high surgical mortality and morbidity rates, especially for aortic endocarditis. This study attempts to investigate the clinical characteristics and operative results of isolated aortic endocarditis. Material and Method: From July 1990 to May 2005, 25 patients with isolated aortic endocarditis (Group I, male female=18 : 7, mean age $43.2{\pm}18.6$ years) and 23 patients with isolated mitral endocarditis (Group II, male female=10 : 13, mean age $43.2{\pm}17.1$ years) underwent surgical treatment in our hospital. All the patients had native endocarditis and 7 patients showed a bicuspid aortic valve in Group I. Two patients had prosthetic valve endocarditis and one patients developed mitral endocarditis after a mitral valvuloplasty in Group II. Positive blood cultures were obtained from 11 (44.0%) patients in Group I, and 10 (43.3%) patients in Group II, The pre-operative left ventricular ejection fraction for each group was $60.8{\pm}8.7%$ and $62.1{\pm}8.1%$ (p=0.945), respectively. There was moderate to severe aortic regurgitation in 18 patients and vegetations were detected in 17 patients in Group I. There was moderate to severe mitral regurgitation in 19 patients and vegetations were found in 18 patients in Group II. One patient had a ventricular septal defect and another patient underwent a Maze operation with microwaves due to atrial fibrillation. We performed echocardiography before discharge and each year during follow-up. The mean follow-up period was $37.2{\pm}23.5$ (range $9{\sim}123$) months. Result: Postoperative complications included three cases of low cardiac output in Group I and one case each of re-surgery because of bleeding and low cardiac output in Group II. One patient died from an intra-cranial hemorrhage on the first day after surgery in Group I, but there were no early deaths in Group II. The 1, 3-, and 5-year valve related event free rates were 92.0%, 88.0%, and 88.0% for Group I patients, and 91.3%, 76.0%, and 76.0% for Group II patients, respectively. The 1, 3-, and 5-year survival rates were 96.0%, 96.0%, and 96.0% for Group I patients, and foo%, 84.9%, and 84.9% for Group II patients, respectively. Conclusion: Acceptable surgical results and mid-term clinical results for aortic endocarditis were seen.
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