• Title/Summary/Keyword: congenital defects

검색결과 302건 처리시간 0.027초

형제에서 발생한 선천성 심장질환 (Congenital Heart Disease in Siblings)

  • 박표원;홍장수;서경필
    • Journal of Chest Surgery
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    • 제14권1호
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    • pp.87-90
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    • 1981
  • Genetic and environmental factors are the two areas which have received attention in the etiology of congenital cardiac malformation. Genetic factor in many types of congenital heart disease have not been clearly delineated. Congenital heart diseases are a heterogenous category of developmental anomalies, representing in most cases the multifactorial inheritance of threshold characters, the expression of which is the product of a genetic - environmental interaction. Recently we experienced three pairs of congenital heart disease in siblings including ventricular septal defects in twin.

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성인에서 선천성 심장기형의 수술성적 -725 치험례 보고- (Operative result congenital cardiac disease in adult - A reort of 725 surgical cases-)

  • 이정렬;서경필
    • Journal of Chest Surgery
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    • 제19권1호
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    • pp.116-121
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    • 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.

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선천 매독성 치아기형 1예 보고 (A Case Report of Dental Defects in Congenital Syphilis)

  • 김종열;정순민
    • Journal of Oral Medicine and Pain
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    • 제7권1호
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    • pp.41-46
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    • 1982
  • The patient, 11 yeats old male was examined for routine oral health care. He had been hospitalized for treatment of nephritis. Hos physical condition os mental retarded & undergrowth state. In oral examination, notch on cutting edge and screw-driver shaped crown of maxillary central incisors, narrow crown and dwarfed & pinched occlusal surface of lower first molars and scars(rhagades) on the angle of the lip were shown. We diagnosed the above symptoms as dental defects of congenital syphilis; Huchinson's inscisors and mulberry molar.

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The use of the buccal fat pad flap for oral reconstruction

  • Kim, Min-Keun;Han, Wonil;Kim, Seong-Gon
    • Maxillofacial Plastic and Reconstructive Surgery
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    • 제39권
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    • pp.5.1-5.9
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    • 2017
  • Many congenital and acquired defects occur in the maxillofacial area. The buccal fat pad flap (BFP) is a simple and reliable flap for the treatment of many of these defects because of its rich blood supply and location, which is close to the location of various intraoral defects. In this article, we have reviewed BFP and the associated anatomical background, surgical techniques, and clinical applications. The surgical procedure is simple and has shown a high success rate in various clinical applications (approximately 90%), including the closure of oroantral fistula, correction of congenital defect, treatment of jaw bone necrosis, and reconstruction of tumor defects. The control of etiologic factors, size of defect, anatomical location of defect, and general condition of patient could influence the prognosis after grafting. In conclusion, BFP is a reliable flap that can be applied to various clinical situations.

Congenital Absence of a Cervical Spine Pedicle : Report of Two Cases and Review of the Literature

  • Oh, Young-Min;Eun, Jong-Pil
    • Journal of Korean Neurosurgical Society
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    • 제44권6호
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    • pp.389-391
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    • 2008
  • Congenital absence of a cervical spine pedicle is a rare clinical entity, and it is usually found incidentally on radiological studies performed after trauma in patients with cervical pain. We report two cases of congenital absence of a cervical spine pedicle and present a review of the literature.

성인 선천성 심장기형의 개심수술 (Open Heart Surgery for Congenital Heart Disease in Adult)

  • 구본원;허동명
    • Journal of Chest Surgery
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    • 제29권9호
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    • pp.940-944
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    • 1996
  • 경북대학교 병원에서는 1990년 1월부터 1994년 10월까지 그 연령이 15세 이상인 선천성 심장병 환자 를 수술적 치료로 교정하였다 이 기간동안 총 628례의 선천성 심장병 환자중 22.4%인 143명이 성인 환자였다. 그중 10대가 23례, 20대가 58례, 30대가 34례, 40대가 18례, 50대가 10례였다. 가장 많은 질환은 전체의 51.1 % (73례)를 차지한 심방중격결손이었고, 39.9 % (57례)의 심실중격결손, 2.8 % (4례)의 활로씨 4징후순이었다. 술후 합병증은 10례 (6.9 %)에서 있었고 수술 사망은 없었다 이상의 결과로 성인에 있어서 수술이 가능한 예의 빈도를 알수 있었으며,이러한 경우에는적은수술 사망과 술후 합병증으로 수술이 가능하였다.

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혈관륜의 수술체험6례 보 (Surgical treatment of vascular ring: a report of 6 cases)

  • 김원곤
    • Journal of Chest Surgery
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    • 제17권2호
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    • pp.205-211
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    • 1984
  • Developmental anomalies of the aortic arch, commonly known as vascular rings, are relatively rare congenital vascular anomalies which can compress the trachea and esophagus. We have presented six patients with surgically treated vascular rings at Seoul National University Hospital during the period June 1970 through May 1984. Two patients had double aortic arches and four patients had right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum. Four patients had symptoms relating vascular ring and two patients were detected incidentally during diagnostic evaluation of symptomatic intracardiac defects. Associated congenital malformations were seen in four patients. The operative approach was through left thoracotomy in three patients and median sternotomy in remaining three patients associated with congenital cardiac defects. There were no postoperative deaths with excellent result in preoperative symptomatic patients. To our knowledge, successful surgical repair of vascular ring has been reported only once in the Korean literature.

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Double Outlet Right Ventricle: In-Depth Anatomic Review Using Three-Dimensional Cardiac CT Data

  • Hyun Woo Goo
    • Korean Journal of Radiology
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    • 제22권11호
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    • pp.1894-1908
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    • 2021
  • Double outlet right ventricle (DORV) is a relatively common congenital heart disease in which both great arteries are connected completely or predominantly to the morphologic RV. Unlike other congenital heart diseases, DORV demonstrates various anatomic and hemodynamic subtypes, mimicking ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, and functional single ventricle. Because different surgical strategies are applied to different subtypes of DORV with ventricular septal defects, a detailed assessment of intracardiac anatomy should be performed preoperatively. Due to high spatial and contrast resolutions, cardiac CT can provide an accurate characterization of various intracardiac morphologic features of DORV. In this pictorial essay, major anatomic factors affecting surgical decision-making in DORV with ventricular septal defects were comprehensively reviewed using three-dimensional cardiac CT data. In addition, the surgical procedures available for these patients and major postoperative complications are described.

폐동맥고혈압증을 동반한 선천성 심기형 환아들에서 술후 조기 혈류역학적 변화에 대한 연구 (A study on the Early Postoperative Hemodynamic Changes after Correction of Congenital Heart Defects associated with Pulmonary Hypertension)

  • 김용진;김기봉
    • Journal of Chest Surgery
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    • 제23권1호
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    • pp.32-40
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    • 1990
  • Surgical correction of congenital cardiac defects in infants and children with an elevated pulmonary arterial pressure or pulmonary vascular resistance carries a significant early postoperative mortality. And accurate assessments of cardiac output is critically important in these patients. From April 1988 through September 1989, serial measurements of cardiac index, ratio of pulmonary-systemic systolic pressure, ratio of pulmonary-systemic resistance, central venous pressure, left atrial pressure, and urine output during the first 48 hours after the cardiac operation were made in 30 congenital cardiac defects associated with pulmonary hypertension. Cardiac index showed significant increase only after 24 hour postoperatively and this low cardiac performance in the early postoperative period should be considered when postoperative management is being planned in the risky patients. There were no variables which showed any significant correlation with cardiac index. In 12 cases[40%], pulmonary hypertensive crisis developed during the 48 hours postoperatively, and they were treated with full sedation, hyperventilation with 100 % 0y and pulmonary vasodilator infusion. In all patient with preoperative pulmonary hypertension, surgical placement of a pulmonary artery catheter is desirable to allow prompt diagnosis of pulmonary hypertensive crisis and to monitor subsequent therapy.

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Practical stepwise approach to rhythm disturbances in congenital heart diseases

  • Huh, June
    • Clinical and Experimental Pediatrics
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    • 제53권6호
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    • pp.680-687
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    • 2010
  • Patients with congenital heart diseases (CHD) are confronted with early- and late-onset complications, such as conduction disorders, arrhythmias, myocardial dysfunction, altered coronary flow, and ischemia, throughout their lifetime despite successful hemodynamic and/or anatomical correction. Rhythm disturbance is a well-known and increasingly frequent cause of morbidity and mortality in patients with CHD. Predisposing factors to rhythm disturbances include underlying cardiac defects, hemodynamic changes as part of the natural history, surgical repair and related scarring, and residual hemodynamic abnormalities. Acquired factors such as aging, hypertension, diabetes, obesity, and others may also contribute to arrhythmogenesis in CHD. The first step in evaluating arrhythmias in CHD is to understand the complex anatomy and to find predisposing factors and hemodynamic abnormalities. A practical stepwise approach can lead to diagnosis and prompt appropriate interventions. Electrophysiological assessment and management should be done with integrated care of the underlying heart defects and hemodynamic abnormalities. Catheter ablation and arrhythmia surgery have been increasingly applied, showing increasing success rates with technological advancement despite complicated arrhythmia circuits in complex anatomy and the difficulty of access. Correction of residual hemodynamic abnormalities may be critical in the treatment of arrhythmia in patients with CHD.