• Title/Summary/Keyword: Congenital anomaly

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Total Repair through Arterial Switch Operation in a Patient with Taussig-Bing Anomaly Undergoing the Modified Damus-Kaye-Stansel Procedure -1 case- (Damus-Kaye-Stansel 술식을 받은 Taussig-Bing 기형의 환자에서 관상동맥 이식을 통한 완전 교정술 - 1예 보고-)

  • 황여주;한미영;전양빈;박철현;박국양;이창하
    • Journal of Chest Surgery
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    • v.37 no.9
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    • pp.796-799
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    • 2004
  • A 52 day-old male infant who had Taussig-Bing anomaly with coarctation of the aorta underwent initial palliative Damus-Kaye-Stansel (DKS) procedure including arch reconstruction because of suspected intramural coronary artery, size discrepancy of great arteries, potential subaortic stenosis, refractory pneumonia, and severe congestive heart failure. Total repair was done 44 months later, which was composed of VSD patch closure, DKS take-down, and arterial switch procedure, We report a successful case of DKS take-down and arterial switch operation for the reuse of native aortic and pulmonary valves rather than Rastelli-type procedure in a patient with Taussig-Bing anomaly having palliative DKS procedure.

Clinic Analysis of Heart Surgery -110 cases- (심장수술 110례 임상고찰)

  • Chang, Hoon;Shin, Sung-Aia;No, Joong-Kee;Rho, Jun-Ryang
    • Journal of Chest Surgery
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    • v.34 no.8
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    • pp.597-603
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    • 2001
  • Background: The purpose of writing this article is to get better clinical results and further clinical improvement based on subject to 110 cases of cardiac surgery which were performed and clinically analyzed. Material and Method: Since January 1995, the patent ductus arteriosus surgery had started in our hospital. In February 1999, an open heart surgery had started and up to September 2000, total of 110 cases were performed as of double ligation of patent ductus arteriosus(10 cases) and open heart surgery(100 cases). Result: Among the patients, Korean-Chines was 74(67.3%) and Han-Chinese was 35(31.8%). Congenital heart disease was 95 cases and acquired valvular heart disease was 15 cases. 83 cases of acyanotic congenital heart disease consisted of ventricular septal defect(VSD) with associated anomaly(45 cases), atrial septal defect(ASD) with associated anomaly(20 cases), patent ductus arteriosus(PDA) with associated anomaly(11 cases), cogenital aortic stenosis(5 cases), double chamber right ventrical(1 case) and Ebstein's anomaly(1 case). Among the 12 cases of cyanotic congenital heart disease, 11 cases of tetralogy of Fallot underwent total correction. Among the 15 cases of acquired valvular heart disease, valvular replacement(7 cases), double valve replacement (3 cases), mitral valve replacement(3 cases) and aortic valve replacement(1 case) were performed. And 8 cases of valvuloplasty were performed by using of commissurotomy, chordal plasty, plasty of papillary muscle, ring type annuloplasty, repair of leaflet. Conclusion: on cogenital heart disease, short term results of surgery for acyanotic cogenital heart disease was good. Among the cyanotic congenital heart disease, tetralogy of Fallot showed a little difference of recovery according to the surgery method so that further follow up observation was needed for long term result. On acquired valvular heart disease, especially, in terms of short term result of valvuloplasty, was relatively good, but further follow up observation was also needed for long term result. There wasn's any operative mortality.

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Multiple congenital anomalies in a Korean native calf (Bos taurus coreanae)

  • Islam, Mohammad Rafiqul;Roh, Yoon-Seok;Cho, Ara;Park, Heejin;Heo, Soo Young;Lee, Kichang;Lee, Han Kyung;Lim, Chae Woong;Kim, Bumseok
    • Korean Journal of Veterinary Research
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    • v.51 no.1
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    • pp.63-67
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    • 2011
  • Supernumerary ectopic limb(s) (SEL) is a congenital anomaly defined as the presence of accessory limb(s) attached to various body regions. This paper describes a case of SEL with ectopic lung and ectopia cordis in a newborn calf, based on macroscopic, microscopic and radiographic findings. External features of multiple congenital anomalies included an ectopic lung growing over the middle of the backbone and covered with normal haired skin. Ectopia cordis was found in the abdominal cavity and attached to the liver. Two extra abnormal limbs originated separately from within the ectopic lung. Most of the abdominal organs were exposed to the outside through the opened abdominal cavity. Microscopically the ectopic lung tissue had edema in the connective tissue around the bronchus and artery. Changes in other organs included congestion of the renal medulla, infiltration of inflammatory cells (lymphocytes and eosinophils) around the hepatic portal tract, and edema surrounding blood vessels and neurons in the brain. The rudimentary humerus of the forelimb was attached to the thoracic spine, as viewed radiographically. The hindlimb was consisted of an irregularly shaped femur, short tibia and fibula, two tarsal bones, one metatarsal bone, and three phalanges. This is the first description of congenital anomalies involving the SEL, ectopic lung and ectopia cordis in a calf.

Understanding of Fetal Surgery and Application to the Cleft Lip and Palate Patient (태수술에 대한 이해와 구순구개열 환자에서의 적용)

  • Kim, Soung-Min;Park, Jung-Min;Myoung, Hoon;Choi, Jin-Young;Lee, Jong-Ho;Choung, Pill-Hoon;Kim, Myung-Jin
    • Korean Journal of Cleft Lip And Palate
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    • v.11 no.2
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    • pp.49-58
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    • 2008
  • The development of fetal surgery has led to promising options for many congenital malformations, such as congenital diaphragmatic hernia (CDH), obstructive uropathy, twin-to-twin transfusion syndrome (TTTS), and sacrococcygeal teratoma. However, preterm labor (PTL) and premature rupture of membranes continue to be uniquitous risks for both mother and fetus. To reduce maternal morbidity and the risk of prematurity, minimal access techniques were developed and are increasingly employed recently. Lift-threatening diseases as well as severely disabling but not life-threatening conditions are potentially amenable to treatment. Recently, improvement of video-endoscopic technology has boosted the development of operative techniques for feto-endoscopic surgery, which has been demonstrated to be less invasive than the open approach. Fetal surgery for repair of cleft lip and palate, a congenital anomaly which is not life threatening, is inappropriate until such time that the benefits are shown to outweigh the risks of both the procedure itself and preterm delivery. Further animal studies will be needed before intrauterine surgery for humans should be considered. For the better understanding of recent techniques and complications associated with fetal intervention of congenital facial defect patients, we reviewed recent related articles about the current knowledge and new perspectives of experimental fetal fetal surgery in the cleft lip and palate defects.

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Radiographic evaluation of congenital vertebral anomalies in Korean raccoon dogs (Nyctereutes procyonoides koreensis)

  • Lee, Eun Gee;Park, Sool Yi;Lee, Kija;Jang, Min;Kim, Jong Taek;Choi, Sooyoung;Park, Inchul
    • Journal of Veterinary Science
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    • v.22 no.4
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    • pp.52.1-52.8
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    • 2021
  • Background: The normal vertebral anatomy of Korean raccoon dogs and their variants require research attention as a prerequisite for identifying pathologies and anomalies. Objectives: This retrospective study aimed at describing the vertebral formula and congenital vertebral anomalies in Korean raccoon dogs (Nyctereutes procyonoides koreensis). Methods: Radiographs of 82 raccoon dogs (42 males, 40 females) acquired from May 2013 to June 2020 in the Gangwon Wildlife Medical Rescue Center were reviewed to evaluate the cervical, thoracic, and lumbar vertebrae of the spine. Results: Normal morphology of all vertebrae was observed in 50 of the 82 raccoon dogs, and the vertebral formula was cervical 7, thoracic 13, and lumbar 7. Congenital vertebral anomalies were found in 32 raccoon dogs: transitional vertebrae (TV) in 31 and block vertebrae in 2. Two raccoon dogs had 2 types of vertebral anomalies: one had TV and block vertebra, and the other had 2 types of TV. Twenty-nine raccoon dogs had thoracolumbar TV (TTV) and 3 had lumbosacral TV. TTV was morphologically classified into 4 different types: unilateral extra-rib in 5 raccoon dogs, bilateral extra-ribs in 14, bilateral elongated transverse processes in 4, and an asymmetric mixed formation of extra-rib with elongated transverse process in 6. Conclusions: This study showed that TTV is common in Korean raccoon dogs, and that the vertebral formula is relatively diverse. The bilateral extra-ribs type TTV is the most common variant, which is almost similar to normal rib to be confused the radiographic evaluation.

Open Heart Surgery of Congenital Heart Diseases -Report of Four Cases- (선천성심질환(先天性心疾患)의 심폐기(心肺器) 개심수술(開心手術) - 4례(例) 보고(報告) -)

  • Kim, Kun Ho;Park, Young Kwan;Jee, Heng Ok;Kim, Young Tae;Rhee, Chong Bae;Chung, Yun Chae;Oh, Chull Soo
    • Journal of Chest Surgery
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    • v.9 no.1
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    • pp.1-9
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    • 1976
  • The present. study reports four cases of congenital heart diseases, who received open heart surgery by the Sarn's Heart-Lung-Machine in the department of Thoracic Surgery, Hanyang University Hospital during the period between July 1975 and May 1976. The Heart-Lung-Machine consisted of the Sarn's five head roller pump motor system (model 5000), heat exchanger, bubble trap, the Rygg-Kyvsgaard oxygenator, and monitors. The priming of pump oxygenator was carried out by the hemodilution method using Hartman's solution and whole blood. Of the four cases of the heart diseases, three whose body weight were below 30kg, received the partial hemodilution priming and the remaining one whose body weight was 52kg received the total hemodilution priming with Hartman's solution alone. The rate of hemodilution was in the average of 60.5ml/kg. Extracorporeal circulation was performed at the perfusion flow rate of the average 94.0ml/kg/min, and at the moderate hypothermia between 35'5"C and 30'5"C of the rectal temperature. In the total cardiopulmonary bypass, arterial blood pressure was anged between 30 mmHg and 85 mmHg, generally maintaining over 60 mmHg and venous pressure was measured between 4 and $23cmH_2O$, generally maintaining below $10cmH_2O$. The first case: The patient, a nine year old girl having the symptoms and physical signs typical to cardiac anomaly was definitely diagnosed as isolated pulmonary stenosis through the cardiac catheterization. There was, however, no cyanosis, no pathological finding by X-ray and E.C.G. tracings. The valvulotomy was performed through the arteriotomy of pulmouary artery under the total cardiopulmonary bypass. Postoperative course of the patient was uneventful, and murmur and the clinical symptoms disappeared. The second case: A 12 year old boy with congenital heart anomaly was positively identified as having ventricular septal defect through the cardiac catheterization. As in the case with the first case, the patient exhibited the symptoms and physical signs typical to cardiac anomaly, but no pathological abnormality by X-ray and E.C.G. tracings. The septal defect was localized on atrioventricular canal and was 2 by 10 mm in size. The septal defect was closed by direct simple sutures under the cardiopulmonary bypass. Postoperative hemodynamic study revealed that the pressure of the right ventricle and pulmonary artery were decreased satisfactory. Postoperative course of the patient was uneventful, and murmur and the clinical symptoms disappeared. The third case: The patient, a 19 year old girl had been experienced the clinical symptoms typical to cardiac anomaly for 16 years. The pink tetralogy of Fallot was definitey diagnosed through the cardiac catheterization. The patient was placed on an ablolute bed rest prior to the operation because of severe exertional dyspnea, fatigability, and frequent syncopal attacks. However, she exhibited very slight cyanosis. Positive findings were noted on E.C.G. tracings and blood picture, but no evidence of pathological abnormality on X-ray was observed. All of the four surgical approaches such as Teflon patch closure (3 by 4cm in size) of ventricular septal defect, myocardial resection of right ventricular outflow tract, valvulotomy of pulmonary valvular stenosis, and pericardial patch closing of ventriculotomy wound were performed in 95 minutes under the cardiopulmonary bypass. Postoperative hemodynamic study revealed that the pressure of the right ventricle was decreased and pulmonary artery was increased satisfactorily. Postoperative course of the patient was uneventful, and murmur and the clinical symptoms disappeared. The fourth case: The patient, a 7 1/4 year old girl had the symptoms of cardiac anomaly for only three years prior to the operation. She was positively identified as having acyanotic tetralogy of Fallot by open heart surgery. The patient showed positive findings by X-ray and E.C.G. tracings, but exhibited no cyanosis and normal blood picture. All of the three surgical approaches, such a myocardial resection of hypertrophic sight ventricular outflow tract, direct suture closing of ventricular septal defect and pericardial patch closing of ventriculotomy wound were carried out in 110 minutes under the cardiopulmonary bypass. Postoperative hemodynamic study revealed that the pressure of the right ventricle was decreased and pulmonary artery was increased satisfactorily. Postoperative course of the patient was uneventful, and the symptoms disappeared.

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Three Cases of Thyroid Hemiagenesis (갑상선 일측 무형성증 3예)

  • Kim, Ki-Hyun;Chung, Hyun-Pil;Kim, Jae-Wook;Koh, Yoon-Woo
    • Korean Journal of Head & Neck Oncology
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    • v.22 no.2
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    • pp.183-187
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    • 2006
  • Thyroid hemiagenesis is a very rare congenital anomaly in which one thyroid lobe fails to develop. The cause of thyroid hemiagenesis is still unknown. The true prevalence of thyroid hemiagenesis is not known, but it is estimated to be from 0.05% to 0.2% in normal children. Thyroid hemiagenesis is common in female with the left lobe being absent. The common disease in the remaining lobe is hyperthyroidism, benign adenoma, a toxic multinodular goiter, chronic thyroiditis, primary myxedema, and rarely carcinoma. Tc-99m pertechnate scintigraphy, ultrasonography and computerized tomography can be used to confirm this anomaly. Here we report three cases with left lobe agenesis. Two of them were euthyroid state while the other hypothyroid patient had a ectopic lingual thyroid.

clinical analyusis of ventricular septal defect (심실중격결손증의 임상적 고찰)

  • 이승구
    • Journal of Chest Surgery
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    • v.19 no.2
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    • pp.265-272
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    • 1986
  • We operated on 199 patients of VSD from 1976 to April l986. Among them, patients of VSD whose medical records were available were analyzed clinically. Operation on patients of VSD occupied 23.9% of total open heart surgery [832 cases] during those days. Of the 164 patients, 93 patients were male [56.7%]. 71 patients were female [43.3%]. Their age ranged from 6 months to 28 years and the mean age was 9.5 year and 82.2% of the patients were between 2 and 15 year of age. Of the patients, body weight below 10Kg were 19 cases. The most common complaints were frequent URI and DOE. On Kirklin`s anatomical classification, type II defect was most common [60.1%], type I [38.4%], combined type I+II, type III, combined type II+Ill and combined type II+IV in orders. Associated anomaly was found in 66 patients [42.5%>]. Pulmonary stenosis was most commonly associated cardiac anomaly [8.4%] and aortic insufficiency [7.1%], ASD, Lt. SVC and PDA in orders. There were extracardiac anomalies such as polydactyly, cleft palate, hypospadia and congenital aniridia, etc. Relationship between ventricular hypertrophy and defect size and cardiac cath. data was analyzed. The overall mortality was 7.0% [14 cases] and complication rate was 22.5% [35 cases].

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Cor triatriatum associated with partial anomalous pulmonary venous drainage: one case report (폐정맥 환류이상을 동반한 삼중방심 치험 1)

  • Kim, Yeong-Ho;Kim, Gong-Su
    • Journal of Chest Surgery
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    • v.17 no.3
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    • pp.381-388
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    • 1984
  • Cor triatriatum is rare congenital cardiac anomaly first described by Church in 1868. The anomaly consists of an abnormal partitioning of the left atrium by a fibromuscular membrane that divides the atrium into an upper chamber, which receives the pulmonary veins, and a lower chamber, which contains the atrial appendage and the mitral valve. The upper and lower chambers communicate through a stenotic fenestration in the membrane, which has the hemodynamic consequence of pulmonary venous obstruction. Recently we experienced cot triatriatum associated with partial anomalous pulmonary drainage to right atrium. The upper chamber was connected to right atrium through a sinus venous type of ASD and received left superior and both inferior pulmonary vein, whereas the lower chamber so called true left atrium communicated with right atrium through foramen ovale type of ASD, left atrial appendage and mitral orifice. And the anomalous membrane has no fenestrations which permit blood flow. The operation was made right atrial approach under the CPB. We excised completely the anomalous septum and reconstructed atrial septal defect with pericardial patch to drain the right upper pulmonary vein to the left atrium. The postoperative course has been good during follow up.

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Hypoplastic Left Heart Syndrome - Experience in one Patient - (좌심저형성 증후군 경험 1)

  • 장봉현
    • Journal of Chest Surgery
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    • v.20 no.2
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    • pp.404-410
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    • 1987
  • An 18-day-old male neonate with hypoplastic left heart syndrome underwent surgical intervention by modification of the Norwood procedure on September 23, 1986. Hypoplastic left heart syndrome is a serious congenital cardiac anomaly that has a fatal outlook if left untreated. Included in this anomaly are [1] aortic valve atresia, and hypoplasia of the ascending aorta and aortic arch, [1] mitral valve atresia or hypoplasia, and [3] diminutive or absent left ventricle. Patent ductus arteriosus is essential for any survival, and there is usually a patent foramen ovale. Coarctation of the aorta is frequently associated with the lesion.z With a limited period of cardiopulmonary bypass, deep hypothermia, and circulatory arrest, the ductus arteriosus was excised. The main pulmonary artery was divided immediately below its branches, and the distal stump of the divided pulmonary artery was closed with a pericardial patch. The aortic arch was incised, and a 1 5mm tubular Dacron prosthesis was inserted between the main pulmonary artery and the aortic arch. A 4mm shunt of polytetrafluoroethylene graft was established between the new ascending aorta and the right pulmonary artery to provide controlled pulmonary blood flow. Following rewarming, the heart started to beat regularly, but the patient could not be weaned from cardiopulmonary bypass. At autopsy, the patient was found to have hypoplasia of the aortic tract complex with mitral atresia and aortic atresia. A secundum atrial septal defect was noted. Right atrial and ventricular hypertrophy was present, and the left ventricle was entirely absent. Although unsuccessful in this case report, continuing experience with hypoplastic left heart syndrome will lead to an improvement in result.

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