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.
Background: The Damus-Kaye-Stansel (DKS) procedure is a proximal MPA-ascending aorta anastomosis used to relieve systemic ventricular outflow tract obstructions (SVOTO) and pulmonary hypertension. The purpose of this study was to review the indications and outcomes of the DKS procedure, including the DKS pathway and semilunar valve function. Material and Method: A retrospective review of 28 patients who underwent a DKS procedure between May 1994 and April 2006 was performed. The median age at operation was 5.3 months ($13\;days{\sim}38.1\;months$) and body weight was 5.0 kg ($2.9{\sim}13.5\;kg$). Preoperative pressure gradients were $25.3{\pm}15.7\;mmHg$ ($10{\sim}60\;mmHg$). Eighteen patients underwent a preliminary pulmonary artery banding as an initial palliation. Preoperative main diagnoses were double outlet right ventricle in 9 patients, double inlet left ventricle with ventriculoarterial discordance in 6,. another functional univentricular heart in 5, Criss-cross heart in 4, complete atrioventricular septal defect in 3, and hypoplastic left heart variant in 1. DKS techniques included end-to-side anastomosis with patch augmentation in 14 patients, classical end-to-side anastomosis in 6, Lamberti method (double-barrel) in 3, and others in 5. The bidirectional cavopulmonary shunt and Fontan procedure were concomitantly performed in 6 and 2 patients, respectively. Result: There were 4 hospital deaths (14.3%), and 3 late deaths (12.5%) with a follow-up duration of $62.7{\pm}38.9$ months ($3.3{\sim}128.1$ months). Kaplan-Meier estimated actuarial survival was $71.9%{\pm}9.3%$ at 10 years. Multivariate analysis showed right ventricle type single ventricle (hazard ratio=13.960, p=0.004) and the DKS procedure as initial operation (hazard ratio=6.767, p=0.042) as significant mortality risk factors. Four patients underwent staged biventricular repair and 13 received Fontan completion. No SVOTO was detected after the procedure by either cardiac catheterization or echocardiography except in one patient. There was no semiulnar valve regurgitation (>Gr II) or semilunar valve-related reoperation, but one patient (3.6%) who underwent classical end-to-side anastomosis needed reoperation for pulmonary artery stenosis caused by compression of the enlarged DKS pathway. The freedom from reoperation for the DKS pathway and semilunar valve was 87.5% at 10 years after operation. Conclusion: The DKS procedure can improve the management of SVOTO, and facilitate the selected patients who are high risk for biventricular repair just after birth to undergo successful staged biventricular repair. Preliminary pulmonary artery banding is a safe and effective procedure that improves the likelihood of successful DKS by decreasing pulmonary vascular resistance. The long-term outcome of the DKS procedure for semilunar valve function, DKS pathway, and relief of SVOTO is satisfactory.
Kim, Sang-Yeol;Han, Sang-Ik;Oh, Seong-Hwan;Lee, Jong-Hee;Park, No-bong;Kwon, Oh-Deog;Kwon, Young-Up
KOREAN JOURNAL OF CROP SCIENCE
/
v.60
no.3
/
pp.282-292
/
2015
Seeds of colored rice generally have high anthocyanin and flavonoid content but some cultivars have low seed germination rate. This defect poses unstable seedling establishment in nursery bed. The seed gravity variations of 10 colored rice varieties and its effect on germination, seed soaking duration and their optimum seeding rates for seedling raising in machine transplanting were investigated in the experiment. Based on seed gravity distribution of the 7 black- and 3 red-colored rice varieties at three levels of seed gravity (1.0<, 1.0~1.06, 1.06>), the black colored seeds such as Josaengheugchal and Sinmyungheugchal had high amount of specific gravity of below 1.0 ranging from 86~ 96%, while they had only 3~13% of seeds in specific gravity above 1.06. Sintoheugmi, Heugjinju, Heugnam, Heugkwang had various mass of seeds, showing 29~44% for specific gravity of below 1.0, 24~39% for 1.0~1.06 and 25~45% for above 1.06. On the other hand, the red colored rice such as Hongjinju, Jeogjinju and Geonkanghongmi had high percentage of specific gravity of above 1.06 with 84~86% while they had only 9~12% of specific gravity below 1.0 similar to Ilmibyeo of noncolored rice. The black colored seed generally showed low germination percentage, slow germination speed and long mean germination time, and low water absorption rate as compared with seed of the red colored rice which was similar to those of Ilmibyeo used as a control cultivar. The black colored seeds took 2~4 days longer seed soaking duration than the red colored rice before germination. This was related to high seed amount of specific gravity below 1.0 in black colored rices. The high amount of seed gravity above 1.06 in the colored rice seeds was positively correlated with percentage of germination, germination speed and mean germination time, ripened grain ratio and water absorption. Seed gravity distribution effect were less pronounced between back- and red- colored seeds in seedling emergence. The black colored rice has slightly lower normal seedling emergence rate than the red colored rice and Ilmibyeo in the seedbed soil due to high percentage of ungerminated seed. Normal seedling emergence rate of the black colored rice in the seedbed was 75.2~82.2% for 10-day old seedling and 85.3~ 90.9% for 30-day old seedling which was lower by 4.5~ 8.0% and 0~3.3%, respectively, than the red colored rice. Based on the normal seedling number per tray of Ilmibyeo for seeding rate of 10-day and 30-day old seedlings, the recommended seeding rate of black colored rice is 200~ 220g seeds for the 10-day old seedling and 110~130g for the 30-day old seedling in transplanting rice while the seeding rate of the red colored rice was 220g seeds for the 10-day old seedling and 130g for the 30-day old seedling.
From August 1986 until June 1995, single aortic valve replacement was performed in 65 patients at the Chonnam National University Hospital. worthy-eight were male and 17 were female patients, ranging from 19 to 68 years of age(median : 43 years). The causes of the valve lesions were rheumatic in 29 patients (44.6%), bicuspid aortic valve in 6 patients (6.2%), endocarditis in 6 patients(6.2%), unknown in others. Concomitant surgical procedures were performed in 10 patients : repair of congenital defect in 5, pericardiectomy in 1, coronary artery bypass grafting in 1, noncoronary sinus plication in 1, Valsalva sinus aneurysmectomy in 1, subaortic membrane resection in 1 Used valves were 51. Jude-Medical valve in 42, Duromedics valve in 22, Bjork-Shiley valve in 2, Carpentier-Edward valve in 1. There were 3 hospital deaths (4.6%), and 2 late deaths (3.2%). Follow-up was 95.2% complete. The 10-year acturlal survival rate was 85.3%. Postoperative complications were low cardiac utput in 8, arrythmia in 5, valve related hemolysis in 1, cerebral infarction in 1, and gastrointestinal bleeding in 2. Reoperation was performed in 4 for surgical bleeding, in 3 for paravalvular leak. The mean improvement in New York Heart Association functional class is from 2.79 $\pm$ 0.66 preoperatively to 1.25 $\pm$ 0.49 postoperatively(p < 0.001) The change of cardiothoracic ratio from preoperative to postoperative is 0.57 $\pm$ 0.06 to 0.54 $\pm$ 0.05 (p < 0.05). The left ventricular ejection fraction change is not significant perioperatively. There are no mechanical failures. This early and intermediate-term follow-up suggests that in adults in whom valve repair is not possible, the mechanical valve is a reliable and durable prosthesis with good hemodynamic function and a low rate of thromboembolic event.
Background: Homografts and bioprostheses are most commonly used for Rastelli operation in congenital heart disease, but the limited durability is responsible for multiple reoperations associated with increased morbidity This study evaluated long-term results after Rastelli operation with a mechanical valved conduit. Material and Method: A total of 20 patients underwent Rastelli operation with mechanical valved conduit from January 1990 to July 1992. Operative mortality was 1 of 20 patients, and a retrospective review of 19 patients(10 males, 9 females) was done. Initial diagnosis was congenitally corrected transposition of great arteries(cc-TGA, n=4), complete TGA (n=2), ventricular septal defect with pulmonary atresia(VSD with PA, n=9), truncus arteriosus(n=2), double outlet right ventricle with pulmonary stenosis(DORV with PS, n=2). The mean age at Rastelli operation was $4.6{\pm}3.4$ years, and mean follow-up period was $12.8{\pm}2.7$ years. Patients underwent Rastelli opearation using 16 CarboMedics mechanical valve, and 3 Bjork-Shiley mechanical valve($17{\pm}2$ mm). Result: There were 15 reoperations for failed mechanical valved conduit. The freedom from reoperation at 5 and 10 years was 53% and 37%. Most patients were received oral anticoagulation with warfarin, and maintained the international normalized ratio(INR) of 1.5 to 2.0. There was no anticoagulation or thromboembolism related complication. There was a significant difference in the causes of a conduit failure between early(within 3 years) and late(after 3 years) failure groups. The six patients reported early prosthetic valve failure, mainly due to valvular dysfunction by thrombosis or pannus formation. The other nine patients reported late prosthetic valve failure, mainly due to dacron conduit stenosis at anastomosis sites, whereas their valvar motion was normal except 1 patient. Conclusion: To avoid early prosthetic valve failure, strict anticoagulation therapy would be helpful. About the late development of obstructive intimal fibrocalcific peels within the Dacron conduit, an improvement of conduit material is necessary to reduce late prosthetic valve failure. In selected patients, the long term results were satisfactory.
Kim, June Bum;Suh, Jin-Soon;Kim, Young-Hoon;Chung, Seung-Yun;Lee, In-Goo;Whang, Kyung-Tai;Lee, Byung-Churl
Clinical and Experimental Pediatrics
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v.48
no.9
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pp.924-928
/
2005
Purpose : Treatment efficacy for children with speech and language delay has been the subject of considerable debate in recent years. We evaluated the clinical features of children with delayed speech and language and their prognoses according to their etiologies after 6 months of speech and language therapy. Methods : From January, 2000 to March, 2004, we retrospectively reviewed 56 children with speech and language delay who were administered speech and language therapy for 6 months in Uijongbu St. Mary's Hospital. Results : Of 56 cases, the proportion of developmental language disorder was 66.1 percent, structural malformation 19.6 percent, mental retardation 12.5 percent, hearing defect 1.8 percent. The ratio of male to female was 4.6 : 1 and the most frequent age group was over 47 months. The mean age of first spontaneous words with useful meaning was 15.9 months. The mean gestational age of the subjects was 39.8 weeks. The proportion of full-term infants was 96.4 percent and of premature infants was 3.6 percent. As for the birth order, the proportion of the first baby was 51.8 percent, the one of second babies it was 42.9 percent, and percent of third babies it was 7.1 percent. After 6 months of language intervention, 32.4 percent of patients with developmental language disorder showed normal linguistic development. All the patients with mental retardation showed sustained language and speech delay. As for the patients with structural malformations, five out of 11 patients showed normal linguistic development. Conclusion : The relatively advanced old age of majority of participants in this study suggests the necessity of screening test for language delay in this local community.
Purpose : The mucopolysaccharidoses (MPSs) are a heterogeneous group of lysosomal storage disorders. They are caused by a deficiency of the enzymes involved in the degradation of glycosaminoglycans. Early recognition is important because recombinant enzyme replacement therapy is now available for MPS. We studied the clinical characteristics of 80 MPS children with the object of determining the epidemiological, clinical and radiological features in Korean MPS children. Methods : Diagnosis of MPS was confirmed by skin fibroblast enzyme analysis in 80 patients between February 1995 and December 2004. Charts were retrospectively reviewed for clinical and radiological findings, as well as for intelligence and speech evaluations. Results : Hunter syndrome (MPS type II) was the most prevalent type, appearing in 51/80 cases (64 %), followed by Sanfilippo syndrome (MPS III-18%), Hurler syndrome (MPS I-15%), and Morquio syndrome (MPS IV-4%). The average age at diagnosis was 5.5 years (range 1 to 20), and the male-to-female ratio was 4.7 : 1. Typical radiographic changes were observed in 45/54 cases (83%). Mitral regurgitation was the most common cardiac defect. Moderate to profound mental retardation and hearing loss were present in 14/35 cases (56%) and 33/38 cases (82%), respectively. Four MPS II patients had bone marrow transplantation, with mixed outcomes. Five MPS I patients are currently on enzyme replacement therapy. Conclusion : Our study showed a high proportion of MPS II cases (64%), which may represent population variability. By studying the clinical features of these patients, we hope to alert pediatricians of the warning signs of MPS.
Jea, Seo Jin;Kwon, Hyo Jin;Jang, Gi Young;Lee, Jae Young;Kim, Soo Jin;Son, Chang Sung;Lee, Joo Won
Clinical and Experimental Pediatrics
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v.51
no.4
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pp.401-408
/
2008
Purpose : Transcatheter closure of atrial septal defects (ASD) is currently established therapy as an alternative to surgery. But rarely, complications are reported in some studies. We report early and intermediate term complications associated with transcatheter closure of atrial septal defects using the Amplatzer septal occluder (ASO). Methods : From June 2003 to May 2006, 64 patients underwent transcatheter closure of secundum ASD or patent foramen ovale using the ASO. The ratio of male to female was 1:2.4, the median age was 17 years (range: 2.6-64 years) and their median weight was 47.5 kg (range: 2.6-64 kg). Results : The median diameter of ASD measured with transthoracic or transesophageal echocardiography was 15 mm (range: 6-28 mm), the median balloon stretched diameter was 18 mm (range: 6.5-34 mm), and the median size of device was 19.5 mm (range: 6-36 mm), was little difference with balloon stretched diameter. There were 10 cases of complications: arrhythmia (2), device malformation (2), aorta to right atrial fistula (1), hemolytic anemia (1), mitral valve encroachment (1), malposition (1), residual shunt (1), and inferior vena cava perforation (1). Conclusion : Transcatheter closure of ASD using ASO is effective and safe therapy. However, significant complications such as aorta to atrial fistula, atrial erosion, or device embolization can happen, so an appropriate selection of patient and device in relevance to size and anatomy of ASD is important for successful closure.
Ahn, Young Mee;Koh, Won-Jung;Kim, Cheol Hong;Lim, Seong Yong;An, Chang Hyeok;Suh, Gee Young;Chung, Man Pyo;Kim, Hojoong;Kwon, O Jung
Tuberculosis and Respiratory Diseases
/
v.54
no.3
/
pp.330-337
/
2003
Background : Low spirometric forced vital capacity(FVC) in conjunction with a normal or high ratio of the forced expiratory volume at 1 second to the forced vital capacity($FEV_1$/FVC%) has traditionally been classified as a restrictive abnormality. However, the gold-standard diagnosis of a restrictive pulmonary impairment requires a measurement of the total lung capacity (TLC). This study was performed to determine the predictive value of spirometric measurements of the FVC for diagnosing a restrictive pulmonary abnormality. Methods : Test results from 1,371 adult patients who undertook both spirometry and lung volume measurements on the same visit from January 1999 to December 2000 were enrolled in this study. The test values for the FVC, the TLC that was below 80% of predicted value, and a $FEV_1$/FVC% that was below 70%, were classified as being abnormal. Results : Of the 1,371 patients, 353 patients had a reduced a FVC. Of these patients, 186 patients had a reduced TLC. Therefore, the positive predictive value was 52.7%. Of the 196 patients with a normal $FEV_1$/FVC% and a reduced FVC, 148(75.5%) patients had a lower TLC. Thirty eight (24.2%) patients out of 157 patients with a low $FEV_1$/FVC% and a low FVC showed a restrictive defect. Conclusion : Spirometry is useful to rule out a restrictive pulmonary abnormality, but a restrictive pattern on the spirometry dose not mean there is a true restrictive disease. For the patients with a low FVC, TLC measurements are essential for diagnosing a restrictive pulmonary impairment.
Background: Thyrotoxic periodic paralysis is an uncommon illness characterized by intermittent flaccid paralysis of skeletal muscle, usually accompanied by hypokalemia, in patient with hyperthyroidism. However, the pathophysiology of thyrotoxic periodic paralysis remains largely unexplained and controversial. This report describes the clinical and biochemical findings in 19 patients with thyrotoxic periodic paralysis who were examined at the Yeungnam University Medical Center(YUMC) during the past decade. Methods: The medical records of 997 YUMC patients, seen between 1986 and 1996, with diagnosis of hyperthyroidism were reviewed. Nineteen patients out of 997 hyperthyroidism patients were diagnosed, and examined by history, physical examination, serum electrolyte value, and thyroid function test during paralysis. On the basis of these results, comparisons were made on age, sex, precipitating factors, timing, affected limbs, prognosis, serum potassium and serum phosphate and thyroid hormone levels. Results: The prevalence of periodic paralysis in hyperthyroidism was 1.9 percent and the male to female prevalence ratio was 30:1 and in all patients, the development of perodic paralysis was correlated with hyperfunctional state of the thyroid gland. Eleven cases of periodic paralysis were associated with hypokalemia and their thyroid hormone levels were significantly more increased than those of the patients without hypokalemia. Interestingly, our study shows the recurrence of paralysis after treatment. Conclusion: Although the precise pathophysiology of the disease is as yet undefined and controversial, it occurs primarily in Asians with an overwhelming male preponderance and prevalence of 2 percent in hyperthyroidism. The interactive roles of thyroid hormone, Na-K pump, and genetically inherited defect in the cellular membrane potential of the skeletal muscle can be speculated. Further investigation will be needed to firmly establish the mechanism of thyrotoxic periodic paralysis.
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