• Title/Summary/Keyword: atrial septal defect

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Unbalanced Translocations of Chromosome 2 and Chromosome 20 in a Two-Generation Family (2대에 걸쳐 나타난 염색체 2번과 20번의 비균형적 전위 1례)

  • Min, Saeah;Lim, Seonwoong;Kim, Youngsook;Lee, Ohkyung
    • Clinical and Experimental Pediatrics
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    • v.45 no.7
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    • pp.917-922
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    • 2002
  • An unbalanced translocation is frequently the result of inheritance of an unbalanced haploid set from a parent with a balanced translocation. Families in which one parent is a balanced translocation carrier fall into the following classes : Those in which none of the possible abnormal offsprings is viable; Those in which one type of offspring, usually the one with the smaller deletion, is born alive; Those in which two types of abnormal offspring are viable. We report a neonate whose karyotype was 46,XX,der(2)t(2;7)(q21;p21.2),der(20)t(2;20)(q21;p13). She was small for her gestational age and had multiple anomalies such as exophthalmos, corneal opacity, short neck, tongue tie, clinodactyly, atrial septal defect, patent ductus arteriosus and ventriculomegaly. Moreover, her mother's karyotype was 46,XX,der(2)t(2;7)(q21;p21.2),del(16)(q22.1),der(20)t(2;20)(q21;p13) but her father had normal karyotype. The same derivative chrosomes were found between mother and her infant, except for del(16)(q22.1) in her mother and these same unbalanced translocations in a two-generation family are extremely rare.

A Rare Case of Scimitar Syndrome Accompanied with an Imperforate Anus in a Newborn (항문 막힘증을 동반한 Scimitar 증후군 1례)

  • Chun, Peter;Lee, Hyoung-Doo;Kim, Young-Don
    • Neonatal Medicine
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    • v.18 no.2
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    • pp.404-408
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    • 2011
  • Scimitar syndrome (SS) is a rare congenital anomaly characterized by hypoplasia of the right lung and partially anomalous pulmonary venous drainage to the inferior vena cava. The term scimitar derives from the shadow created by the anomalous pulmonary vein on chest X-ray that closely resembles that of a curved Turkish sword. It rarely presents as an isolated abnormality. Various cardiac and non-cardiac anomalies have been association with SS, such as right lung hypoplasia, dextroposition of the heart, hypoplasia of the right pulmonary artery, systemic arterial blood supply to the right lower lung from the infra-diaphragmatic aorta, and a secundum type of atrial septal defect. However, an imperforate anus has not been reported previously in association with SS. We describe the first case of infantile scimitar syndrome accompanied with an imperforate anus in a newborn who presented with tachypnea and right pulmonary atelectasis.

Open Heart Surgery Through other than Full Sternotomy in Adults (성인에서 최소절개를 이용한 개심술)

  • 이재원;송명근
    • Journal of Chest Surgery
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    • v.31 no.6
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    • pp.576-580
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    • 1998
  • This study is to clarify the results of atrial septal defect(ASD) repair and mitral valve surgery through right anterolateral thoracotomy since 1989, and those of more generalized application of minimal invasive cardiac surgery since August 1997. We retrospectively analyzed the results of open heart surgery(OHS) through other than full sternotomy carried out until October 1997. There were 28 cases of OHS done through right anterolateral thoracotomy(17 cases of ASD, 4 cases of mitral valve repair, 6 cases of mitral valve replacement, and 1 redo mitral and tricuspid valve repalcement) which has demonstrated no surgical mortality or morbidity except only 1 case of reoperation for bleeding. During the period between August and October 1997, we performed near routine application of upper sternotomy or transverse sternotomy in aortic valve cases and routine application of minimal incision in cases with ASD and there was no evidence of early and late complications associated with this approach. We conclude that OHS with the use of minimal incisions is very safe, cosmetically excellent, and superior in terms of the amount of bleeding. The indication for minimal incision, therefore, should be extended afterwards.

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Intervention with Balloon Valvuloplasty followed by Patent Ductus Arteriosus Stent in a Patient with Pulmonary Atresia with Intact Ventricular Septum (풍선판막성형술과 동맥관 스텐트를 이용하여 치료한 심실중격결손을 동반하지 않은 폐동맥 폐쇄 1례)

  • Lim, Han Hyuk;Kim, Young Deuk;Lee, Jae Hwan;Chang, Mea Young;Kil, Hong Ryang
    • Clinical and Experimental Pediatrics
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    • v.48 no.11
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    • pp.1256-1256
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    • 2005
  • Pulmonary atresia with intact ventricular septum (PAIVS) is rare, less than 1% of congenital heart disease. It needs a therapeutic approach according to its individual morphologic feature. Surgical treatment of valvotomy and modified Blalock-Taussig shunt or non-surgical interventional catheter balloon valvuloplasty can be used for mild to moderate hypoplasia of right ventricle. Fontan operation can be considered for less optimum morphological substrate of two ventricular repair. A 3-day-old male neonate was admitted with cyanosis and cardiac murmur. On echocardiogram, he had membranous pulmonary atresia with intact ventricular septum, normal sized tripartite right ventricle, large atrial septal defect with right-to-left shunt, small sized patent ductus arteriosus, and moderate tricuspid regurgitation. He was treated with intravenous continuous infusion of prostaglandin $E_1$ ($PGE_1$) at once. On the third day of hospitalization, Balloon valvuloplasty was performed. After insertion of patent ductus arteriosus stent on the tenth day, $PGE_1$ infusion was discontinued. On the fifteenth day, he was discharged. Now, he is 9 months old and has nearly normal cardiac structure and function with 97% of percutaneous oxygen saturation.

Current Trend of Robotic Thoracic and Cardiovascular Surgeries in Korea: Analysis of Seven-Year National Data

  • Kang, Chang Hyun;Bok, Jin San;Lee, Na Rae;Kim, Young Tae;Lee, Seon Heui;Lim, Cheong
    • Journal of Chest Surgery
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    • v.48 no.5
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    • pp.311-317
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    • 2015
  • Background: Robotic surgery is an alternative to minimally invasive surgery. The aim of this study was to report on current trends in robotic thoracic and cardiovascular surgical techniques in Korea. Methods: Data from the National Evidence-based Healthcare Collaborating Agency (NECA) between January 2006 and June 2012 were used in this study, including a total of 932 cases of robotic surgeries reported to NECA. The annual trends in the case volume, indications for robotic surgery, and distribution by hospitals and surgeons were analyzed in this study. Results: Of the 932 cases, 591 (63%) were thoracic operations and 340 (37%) were cardiac operations. The case number increased explosively in 2007 and 2008. However, the rate of increase regained a steady state after 2011. The main indications for robotic thoracic surgery were pulmonary disease (n=271, 46%), esophageal disease (n=199, 34%), and mediastinal disease (n=117, 20%). The main indications for robotic cardiac surgery were valvular heart disease (n=228, 67%), atrial septal defect (n=79, 23%), and cardiac myxoma (n=27, 8%). Robotic thoracic and cardiovascular surgeries were performed in 19 hospitals. Three large volume hospitals performed 94% of the case volume of robotic cardiac surgery and 74% of robotic thoracic surgery. Centralization of robotic operation was significantly (p<0.0001) more common in cardiac surgery than in thoracic surgery. A total of 39 surgeons performed robotic surgeries. However, only 27% of cardiac surgeons and 23% of thoracic surgeons performed more than 10 cases of robotic surgery. Conclusion: Trend analysis of robotic and cardiovascular operations demonstrated a gradual increase in the surgical volume in Korea. Meanwhile, centralization of surgical cases toward specific surgeons in specific hospitals was observed.

Characteristics of allergic pollens and the recent increase of sensitization rate to weed pollen in childhood in Korea (알레르기 화분의 특성과 최근 소아에서 잡초류 화분의 감작률 증가)

  • Oh, Jae-Won
    • Clinical and Experimental Pediatrics
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    • v.51 no.4
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    • pp.355-361
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    • 2008
  • Pollen is very important causing factor for allergy such as allergic rhinitis, allergic conjunctivitis, and asthma, and pollen allergy has a remarkable clinical impact all over Korea. The main pollination period covers about half the year, from spring to autumn, and the distribution of airborne pollen taxa of allergological interest is related to pollen season dynamics. Korean academy of pediatric allergy and respiratory diseases (KAPARD) has evaluated the pollen characteristics and nationwide pollen count for over 10 years since 1997. Airborne particles carrying allergens were collected daily from nationwide 8 stations (Seoul, Guri, Cheongju, Daegu, Kwangju, Busan, Kangneung, and Jeju) by using 7 days-Burkard sampler (Burkard Manufacturing Co Ltd, Hertfordshire, UK) in South Korea (July 1, 1997-June 30, 2007). They were counted and recorded along with the meteorological factors daily. Tree pollen is a major airborne allergen in spring, grass is most common in summer, and weed pollen is major pollen in autumn in Korea. There has two peak seasons for pollen allergy, as summer and autumn. There is some evidence suggesting that the prevalence of allergic diseases in Korea has been on the increase in the past decade. However, recent findings of the phase I and II studies of the international Study of Asthma and Allergies in Childhood (ISAAC) study showed the absence of increases or little changes in prevalence of asthma symptoms and diagnosis rates in Korea, whereas the prevalence of allergic rhinitis and atopic dermatitis were increased. We reported the evidence that sensitization rate to weed pollen has been increased yearly since 1997 in childhood. Climate change and air pollution must be the major causing factors for the increase of pollen counts and sensitization rate to pollen. Climate change makes the plants earlier pollination and persisting pollination longer. In conclusion, data on pollen count and structure in the last few years, the pathogenetic role of pollen and the interaction between pollen and air pollutants with climate change gave new insights into the mechanism of respiratory allergic diseases in Korea.

Extracorporeal Membrane Oxygenation in the Patient with Cardiopulmonary Resuscitation Failure after Open Heart Surgery. - A case report - (개심술 후 심폐소생술 실패환아에서의 체외막산소화 치험 1례)

  • 전희재;성시찬;우종수;이혜경
    • Journal of Chest Surgery
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    • v.32 no.1
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    • pp.53-57
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    • 1999
  • We describe a case of successful extracorporeal membrane oxygenation(ECMO) in a small infant with cardiopulmonary resuscitation(CPR) failure after an open heart surgery. A 35-day-old male infant weighing 4.4 kg who had congestive heart failure and pulmonary hypertension underwent patch closure of ventricular septal defect without any intraoperative event. Postoperative course was unremarkable in the intensive care uint for about 5 hours before the junctional ectopic tachycardia developed. Sudden cardiac decompensation with bradycardia occurred about 50 minutes after the development of junctional ectopic tachycardia. He was put on ECMO by arterial cannulation at the ascending aorta and by venous cannulation at the right atrial appendage after 4 hours' CPR. The hemodynamics were stable with enough urine output during ECMO. He was weaned from ECMO 38.5 hours after initiation. Delayed sternal closure was attempted. He was extubated on postoperative day 7 and discharged home on postoperative day 21 without any neurologic sequelae.

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Endovascular Aortic Balloon Clamping for Various Heart Disease (대동맥 내 풍선 차단법을 이용한 여러 가지 심장수술)

  • Choi, Jin-Ho;Park, Pyo-Won
    • Journal of Chest Surgery
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    • v.41 no.1
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    • pp.61-67
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    • 2008
  • Background: Conventional ascending aortic cross clamping is often limited. This study was carried out to evaluate the safety and efficacy of an endovascular aortic clamping technique with an intraaortic balloon catheter. Material and Method: From April 2004 to January 2007, surgery with endoaortic clamping was performed in seven patients. A retrograde access perfusion (RAP) catheter was used in six patients and a Pruitt's balloon catheter in two patients. The indication for the operation was a retrosternal pseudoaneurysm of the aortic root in six patients, diffuse calcification of the ascending aorta with aortic regurgitation in 2 patients and an atrial septal defect in one patient. Five patients had at least two prior cardiac surgeries. Result: Successful insertion of the catheter and endoaortic clamping was achieved in all patients. Conclusion: The endovascular aortic clamping technique with a balloon catheter was a useful alternative method for patients in whom conventional cardiac surgery was limited.

Clinical features and surgical outcomes of complete transposition of the great arteries

  • Hong, Suk Jin;Choi, Hee Joung;Kim, Yeo Hyang;Hyun, Myung Chul;Lee, Sang Bum;Cho, Joon Yong
    • Clinical and Experimental Pediatrics
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    • v.55 no.10
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    • pp.377-382
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    • 2012
  • Purpose: This single-center study aimed to assess the clinical features and surgical approaches and outcomes of complete transposition of the great arteries (TGA). Methods: TGA patients who had undergone surgical correction at the Kyungpook National University Hospital from January 2000 to December 2010, were retrospectively evaluated for patient characteristics, clinical manifestation, preoperative management, intraoperative findings, postoperative progress, and follow-up status. Results: Twenty-eight patients (17 boys and 11 girls, mean age=$10.6{\pm}21.5$ days) were included and were categorized as follows: group I, TGA with intact ventricular septum (n=13); group II, TGA with ventricular septal defect (VSD, n=12); and group III, TGA/VSD with pulmonary stenosis (n=3). Group I underwent the most intensive preoperative management (balloon atrial septostomy and prostaglandin E1 medication). Group II showed the highest incidence of heart failure (P<0.05). Usual and unusual coronary anatomy patterns were observed in 20 (71%) and 8 patients, respectively. Arterial and half-turned truncal switch operations were performed in 25 and 3 patients (Group III), respectively. Postoperative complications included cardiac arrhythmias (8 patients), central nervous system complications (3 patients), acute renal failure (1 patient), infections (3 patients), and cardiac tamponade (1 patient), and no statistically significant difference was observed between the groups. Group II showed the mildest aortic regurgitation on follow-up echocardiograms (P<0.05). One patient underwent reoperation, and 1 died. The overall mortality rate was 4%. Conclusion: Our study showed favorable results in all the groups and no significant difference in postoperative complication, reoperation, and mortality among the groups. However, our results were inadequate to evaluate the risk factors for reoperation and mortality owing to the small number of patients and short follow-up duration.

First Experience of Cardiac Surgery using da $Vinci^{TM}$ Surgical System in Korea (da $Vinci^{TM}$ 수술로봇을 이용한 심장수술 - 첫 번째 치험 보고)

  • Park, Seong-Yong;Lee, Sak;Joo, Hyun-Chul;Yang, Hong-Suk;Park, Young-Hwan;Park, Har-Ki
    • Journal of Chest Surgery
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    • v.40 no.2 s.271
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    • pp.128-131
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    • 2007
  • da $Vinci^{TM}$ Surgical System is an advanced mode of minimal invasive surgery, using 3-dimensional imaging system and robotic arms which can mimic the dexterity of the human hand. Thus, various operations can be performed with minimal incision and limited surgical field and the merits of minimal invasive surgery can be maximized by using it. We report our first experience of robotic open heart surgery using the da $Vinci^{TM}$ Surgical System for repairing atrial septal defect.