• Journal of Chest Surgery
• /
• 제22권1호
• /
• pp.50-58
• /
• 1989

The emergence and expansion of cardiac surgery over the past decade has resulted in an increasing number of patients undergoing cardiac operations but many kinds of heart surgery was realized only palliative, resulting in increasing numbers of secondary cardiac procedures. From 1978 to 1988, 10 cases of various congenital heart diseases and 17 cases of acquired heart diseases were reoperated at Hanyang University Hospital. The leading indication of second operation was residual shunt or valvular malfunction due to technical failure in congenital heart disease and primary valve failure, endocarditis, paravalvular leakage were for acquired heart disease. The mortality of reoperation was 0% for congenital heart disease and 11.7%[2 death among the 17 patients] for acquired heart disease. The leading causes of death were myocardial failure, sepsis with endocarditis, acute renal failure and congestive heart failure.

• Korean Circulation Journal
• /
• 제52권8호
• /
• pp.623-631
• /
• 2022

Background and Objectives: Pulmonary valve replacement (PVR) is the most common operation in adults with congenital heart disease (CHD). There is controversy regarding the best bioprosthesis. We compare the performance of stented bioprosthetic valves (the Mosaic [Medtronic^TM] porcine pericardial against Carpentier Perimount Magna Ease [Edwards^TM] bovine) in pulmonary position in patients with CHD. Methods: Between January 1999 and December 2019, all the PVRs were identified from hospital databases in 2 congenital heart centres in Spain. Valve performance was evaluated using clinical and echocardiographic criteria. Propensity score matching was used to balance the 2 treatment groups. Results: Three hundred nineteen patients were retrospectively identified. After statistical adjustment, 79 propensity-matched pairs were available for comparison Freedom from reintervention for the porcine cohort was 98.3%, 96.1%, and 91.9% at 3, 5, and 10 years and 100%, 98%, and 90.8% for the bovine cohort (p=0.88). Freedom from structural valve degeneration (SVD) for the porcine cohort was 96.9%, 92.8% and 88.7% at 3, 5, and 10 years and 100%, 98%, and 79.1% for the bovine cohort (p=0.38). Bovine prosthesis was associated with a reintervention hazard ratio (HR), 1.12; 95% confidence intervals (CIs), 0.24-5.26; p=0.89 and SVD HR, 1.69 (0.52-5.58); p=0.38. In the first 5 years, there was no difference in outcomes. After 5 years, the recipients of the bovine bioprosthesis were at higher risk for SVD (reintervention HR, 2.08 [0.27-16.0]; p=0.49; SVD HR, 6.99 [1.23-39.8]; p=0.03). Conclusions: Both bioprosthesis have similar outcomes up to 5 years, afterwards, porcine bioprosthesis seem to have less SVD.

• Journal of Yeungnam Medical Science
• /
• 제16권1호
• /
• pp.125-130
• /
• 1999

저자들은 최근 25세 여자 환자로 뚜렷한 가족력이 없으면서 3-4세경부터 발생한 근육의 강직현상과 현저한 근비대소견이 있는 환자를 경험하였다. 임상적으로 타진성 근긴장증을 보이고 전기생리적 검사로 새끼손가락 외향근에서의 운동과 반복신경자극에서 복합근육활동전위의 감소를 보였으며, 이두박근의 근생검 소견상 용적이 증가된 근섬유와 중심핵화현상이 관찰되었다. 환자는 mexiletin 경구 투여후 근육의 강직현상이 다소 호전되었다. 이에 문헌고찰과 함께 Becker형 선천성 근긴장증 1례를 보고하는 바이다.

• Korean Journal of Radiology
• /
• 제19권6호
• /
• pp.1031-1041
• /
• 2018

Objective: To compare image quality and radiation dose of high-pitch dual-source spiral cardiothoracic computed tomography (CT) between non-electrocardiography (ECG)-synchronized and prospectively ECG-triggered data acquisitions in young children with congenital heart disease. Materials and Methods: Eighty-six children (${\leq}3$ years) with congenital heart disease who underwent high-pitch dual-source spiral cardiothoracic CT were included in this retrospective study. They were divided into two groups (n = 43 for each; group 1 with non-ECG-synchronization and group 2 with prospective ECG triggering). Patient-related parameters, radiation dose, and image quality were compared between the two groups. Results: There were no significant differences in patient-related parameters including age, cross-sectional area, body density, and water-equivalent area between the two groups (p > 0.05). Regarding radiation dose parameters, only volume CT dose index values were significantly different between group 1 ($1.13{\pm}0.09mGy$) and group 2 ($1.07{\pm}0.12mGy$, p < 0.02). Among image quality parameters, significantly higher image noise ($3.8{\pm}0.7$ Hounsfield units [HU] vs. $3.3{\pm}0.6HU$, p < 0.001), significantly lower signal-to-noise ratio ($105.0{\pm}28.9$ vs. $134.1{\pm}44.4$, p = 0.001) and contrast-to-noise ratio ($84.5{\pm}27.2$ vs. $110.1{\pm}43.2$, p = 0.002), and significantly less diaphragm motion artifacts ($3.8{\pm}0.5$ vs. $3.7{\pm}0.4$, p < 0.04) were found in group 1 compared with group 2. Image quality grades of cardiac structures, coronary arteries, ascending aorta, pulmonary trunk, lung markings, and chest wall showed no significant difference between groups (p > 0.05). Conclusion: In high-pitch dual-source spiral pediatric cardiothoracic CT, additional ECG triggering does not substantially reduce motion artifacts in young children with congenital heart disease.

• Korean Journal of Radiology
• /
• 제21권6호
• /
• pp.717-725
• /
• 2020

Objective: To assess left ventricular remodeling patterns using cardiac computed tomography (CT) in children with congenital heart disease and correlate these patterns with their clinical course. Materials and Methods: Left ventricular volume and myocardial mass were quantified in 17 children with congenital heart disease who underwent initial and follow-up end-systolic cardiac CT studies with a mean follow-up duration of 8.4 ± 9.7 months. Based on changes in the indexed left ventricular myocardial mass (LVMi) and left ventricular mass-volume ratio (LVMVR), left ventricular remodeling between the two serial cardiac CT examinations was categorized into one of four patterns: pattern 1, increased LVMi and increased LVMVR; pattern 2, decreased LVMi and decreased LVMVR; pattern 3, increased LVMi and decreased LVMVR; and pattern 4, decreased LVMi and increased LVMVR. Left ventricular remodeling patterns were correlated with unfavorable clinical courses. Results: Baseline LVMi and LVMVR were 65.1 ± 37.9 g/m² and 4.0 ± 3.2 g/mL, respectively. LVMi increased in 10 patients and decreased in seven patients. LVMVR increased in seven patients and decreased in 10 patients. Pattern 1 was observed in seven patients, pattern 2 in seven, and pattern 3 in three patients. Unfavorable events were observed in 29% (2/7) of patients with pattern 1 and 67% (2/3) of patients with pattern 3, but no such events occurred in pattern 2 during the follow-up period (4.4 ± 2.7 years). Conclusion: Left ventricular remodeling patterns can be characterized using cardiac CT in children with congenital heart disease and may be used to predict their clinical course.

• Journal of Chest Surgery
• /
• 제10권1호
• /
• pp.118-123
• /
• 1977

Congenital cystic disease of the lung is very rare and controversial disease. But in general is regarded as developmental anomaly. Occasionally failure of the primitive lung bud to develop combined with cardiac anomaly had been reported but it was very rare. Recently a case of cystic lung disease combined with pulmonic valvular stenosis was seen in this clinic with clinical pictures of nonspecific respiratory infection and X-ray finding very similar to that of far advanced pulmonary tuberculosis, destroyed left lung. This case was treated by closed pulmonic valvulotomy and left side pneumonectomy successfully.

• Journal of Chest Surgery
• /
• 제17권4호
• /
• pp.804-810
• /
• 1984

Two hundred twenty one cases of open heart surgery were done in the Department of Thoracic and Cardiovascular Surgery, Pusan National University Hospital from July, 1981 to October, 1984. 1.There were 154 cases [73%] of congenital anomalies and 57 cases [27%] of acquired valvular heart diseases. Among the congenital cases, 128 cases were acyanotic and 26 cases were cyanotic. Among the 57 cases of acquired valvular replacement surgery, 3 cases had open heart commissurotomy, one had Kay annuloplasty. 2.The age distribution of the congenital acyanotic anomalies ranged from 5 to 32 years with mean age of 12.8 years, the congenital cyanotic anomalies from 3 to 29 years with mean age of 14.2 years and the acquired valvular diseases from 9 to 51 years with mean age of 30 years. The difference of sex distribution was no significance. 3.Three methods for debubbling process were used in our institute, in 133 cases, the vent was inserted into the left ventricular apex, in 61 cases inserted into the left atrium through right superior pulmonary vein and in 17 cases used needle aspiration only. 4.For cardioplegia, the GIK solution was infused repeatedly from 30 to 40 minutes interval and brought excellent results for myocardial protection during open heart surgery. 5.Overall mortality was 7.6%. The mortality along with each disease is 1.56% in congenital acyanotic cases, 26.9% in congenital cyanotic cases and 12.3% in acquired valvular disease.

• 대한유전성대사질환학회지
• /
• 제6권1호
• /
• pp.24-31
• /
• 2006

Purpose : The Ministry of Health and Social Affairs adopted newborn screening for the low-income families in 1991 and expanded in 1997 to cover all newborns. At the beginning of the program 6 diseases were selected for screening but the number of screening items had been reduced to two (congenital hypothyroidism and phenylketonuria) from the year 1995. Now, the government program has a fifteen year history. The purpose of this study was to analyze results of neonatal screening tests and prevalence at birth of phenylketonuria and congenital hypothyroidism in Korea. Methods : The results of neonatal screening tests were collected from public health centers during 15 years from 1991 to 2005. These data were analyzed for number of tested newborns and prevalence at birth of the inborn errors of metabolism. Results : Neonatal screening test for inborn error of metabolism was performed for 3,707,773 newborns for 15 years. Among newborns who were screened 718 congenital hypothyroidisms and 86 phenylketonurias were detected, and these presented an prevalence at bith of congenital hypothyroidism 1/5,164 and that of phenylketonuria 1/43,114. The total prevalence of two diseases was 1/4,612. Conclusion : National screening program should be expanded to include all items of screening tests for whole newborns and established correct prevalence of other inherited metabolic diseases in Korea.

• Journal of Chest Surgery
• /
• 제19권1호
• /
• pp.116-121
• /
• 1986

Patients over 15 years of age who have undergone a surgical correction of a congenital cardiac malformation during period of January 1958 through January 1986 have been reviewed. During this period there were 3957 congenital cardiac lesions consisting of 2712 acyanotic and 1245 cyanotic cases. Among them, a total of 725 adults [18.3%] with a variety of congenital heart lesions, 548 acyanotic group and 177 cyanotic group were operated on. 372 patients were male and 252 patients were female. There were 280 patients under 20 years of age, 206 between 20-24 years, 102 between 25-29 years, 48 between 30-34 and 89 over 35 years. The most common defects were atrial septal defect which accounted for 207 cases [28.6%] and other common anomalies were VSD [140 cases, 19.3%], TOF [136 cases, 18.6%], PDA [120 cases, 16.6%] and PS [33 cases, 4.6%] in order of incidence. Overall operative mortality for this series was 3.6% [1.8% of a cyanotic group and 9.0% of cyanotic group] compared with 2.8% of total cases of congenital heart disease [acyanotic group 1.1% and cyanotic group 6.5%]. This reviewed series reveals the incidence of operable congenital heart defects appearing in any adult life and demonstrates that surgical repair can be accomplished with a satisfactory low mortality rate.

• Journal of Yeungnam Medical Science
• /
• 제39권2호
• /
• pp.161-167
• /
• 2022

Congenital web formations are extremely rare anomalies of the extrahepatic biliary tree. We herein report a case of common bile duct septum combined with multiple intrahepatic bile duct strictures in a 74-year-old female patient who was successfully treated with radiological intervention. The patient initially visited the hospital because of upper abdominal pain. Imaging studies revealed multifocal strictures with dilatation in both intra- and extrahepatic ducts; the final clinical diagnosis was congenital common bile duct web combined with multiple intrahepatic duct strictures. Surgical treatment was not indicated because multiple biliary strictures were untreatable, and the disease was clinically diagnosed as benign. The multiple strictures were extensively dilated twice through bilateral percutaneous transhepatic biliary drainage (PTBD) for 2 months. After 1 month of observation, PTBD catheters were successfully removed. The patient is doing well at 6 months after completion of the radiological intervention, with the maintenance of normal liver function. Congenital web of the bile duct is very rare, and its treatment may vary depending on the patterns of biliary stenosis. In cases where surgical intervention is not indicated for congenital web and its associated disease, radiological intervention with balloon dilatation can be a viable therapeutic option.