• Title/Summary/Keyword: 선천성 기형

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Congenital Polyvalvular Disease; Report of A Case (선천성 다발성 판막질환 1예 보고)

  • 김정원;민경석;윤태진;서동만;윤소영;김영휘;고재곤;박인숙;김규래
    • Journal of Chest Surgery
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    • v.34 no.8
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    • pp.626-629
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    • 2001
  • Congenital polyvalvular disease is a connective tissue disorder affecting more than one heart valve with variable involvement of the entire valvular and subvalvular apparatus. It is frequently associated with the Trisomy 18 and trisomy 13-15 or ventricular septal defect and patent ductus arteriosus. We present an isolated case of congenital polyvalvular disease in a new born baby with a review of the pertinent literatures, which has not been described in Korea. The mass was discovered as a right atrial mass in the prenatal ultrasonography and it was thought to be either a hematoma or a myxoma in the preoperative echocardiography. Microscopic examination of the surgically resected mass showed irregular thickening, nodulation, and additional features of calcification and ossification in the valvular connective tissue on the body of anterior and septal leaflet of tricuspid valve. Congenital polyvalvular disease should be included in the differential diagnosis in cases showing valvular calcification or ossification in the fetal echocardiography.

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Results of Mitral Valve Repair in Patients with Congenital Mitral Disease (선천성 승모판막 기형 환자에서 승모판막 성형술)

  • Jang, Hee-Jin;Lee, Jeong-Ryul;Rho, Joon-Ryang;Kim, Yong-Jin;Kim, Woong-Han
    • Journal of Chest Surgery
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    • v.42 no.2
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    • pp.175-183
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    • 2009
  • Background: Mitral valve abnormalities in the pediatric population are rare. Mitral valve replacement or pediatric mitral lesions can cause problems such as a lack of growth potential. There re only limited experiences with mitral valve repair at any institution, so the purpose of his study is to evaluate the outcomes of mitral valve repair n pediatric patients. Material and Method: Sixty-four consecutive children (28 males and 36 females) with a mean age of $5.5{\pm}4.7$ years underwent mitral valve repair for treating their congenital mitral valve disease between January 1996 and December 2005. The patients were divided into two groups: group 1 (34 patients (53.1%)) had isolated disease (mitral anomaly with or without trial septal defect or patent ductus arteriosus) and group 2 (30 patients (46.9%)) had complex disease (mitral anomaly with concurrent intracardiac disease, except atrioventricular septal defect). Result: The overall in-hospital mortality was 6.3%; group 1 had 5.9% mortality and group 2 had 10.0% mortality. The postoperative morbidity was 18.8%; group 1 and 2 had 14.7% and 23.3% postoperative morbidity, respectively, and there as no significant difference among the groups. The median follow-up was 4.6 years range: $0.5{\sim}12.2$ years). The 10-year survival rate was 95.3%. The 10-year freedom from re-operation rate was 76.1% with 10 re-operations. The majority of the functional classifications were annular dilatation and leaflet prolapse. A mean of $2.1{\pm}1.1$ procedures per patient were performed. The echocardiography that was done at the immediate postoperative period showed a significant improvement in the mitral valve function. The follow-up echocardiographic results were significantly improved. However, mitral stenosis newly developed over time, and there ere significant differences according to the repair strategies. Conclusion: The patients who underwent mitral valve repair for congenital mitral anomalies showed good results. The follow-up echocardiography revealed satisfactory short-term and long-term results. Close follow-up is necessary to detect the development of postoperative mitral stenosis or regurgitation.

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

  • 구본원;허동명
    • Journal of Chest Surgery
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    • v.29 no.9
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    • pp.940-944
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    • 1996
  • Patients over 15 years of age who have undergone a surgical correction of congenital heart disease at Kyungpook University Hospital during the period of January 1990 through October 1994 have been reviewed . One hundred forty three, 22.4 % of 628 operations, which have repaired congenital heart diseases during this period were adult patients. There were 23 patients under 20 years of age, 58 between 20∼29 years, 34 between 30∼39 years, 18 between 40∼49 years, and 10 between 50∼59 years. The most common defects were atrial septal defects which accounted for 73 cases (51.1 %) and other common anomalies were ven- tricular septal defects (57 cases, 39.9 %), tetralogy of Falloffs(4 cases, 2.8%) in order of incidence. There were 10 non-fatal operative complications (6.9 %) but there was no operative mortality. This study shows the incidence of operable congenital heart diseases in adults and the fact that it could be corrected surgically with low mortality and morbidity.

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Cone Reconstruction for Tricuspid Valve Repair in a Patient with Ebstein's Anomaly - A case report - (Cone 재건술을 이용한 엡스타인 기형의 삼첨판막 성형술 - 1예 보고 -)

  • Lee, Cheul;Kwak, Jae-Gun;Lee, Chang-Ha
    • Journal of Chest Surgery
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    • v.42 no.4
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    • pp.509-512
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    • 2009
  • Ebstein's anomaly is a complex congenital defect of the tricuspid valve and right ventricle. Various surgical methods to repair the regurgitant tricuspid valve have been reported, and most of them depend on monocuspidalization with using the anterior leaflet. We report here on our first experience with Ebstein's anomaly in a 31-year-old female patient who underwent cone reconstruction of the tricuspid valve with using three leaflets.

A Case of Microtia (선천성 외이기형)

  • 김세훈;원상희;황명순;한주호;김선우
    • Proceedings of the KOR-BRONCHOESO Conference
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    • 1981.05a
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    • pp.40.2-40
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    • 1981
  • The deformities of the auricle is rare and classified roughly into two categories as "Hyperplasia and Hypoplasia". Microtia occurs about once in every 6000 births and is twice as frequent in males as in females and the unilateral to bilateral ratio is roughly 8 : 1. Significant malformation of the auricle frequently involves the external auditory canal and the contents of the middle ear. Recently, the authors experienced a case of hypoplasia of the superior third of the right auricle of 24 year old male, with neither hearing impairment nor any other associated defect, who was surgically corrected by rotation flaps through post-auricular incision.

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Congenital Quadricuspid Semilunar Valve - A case report - (선천성 사엽성 반월형 판막 - 1예 보고 -)

  • Seo, Min-Bum;Seo, Hong-Joo
    • Journal of Chest Surgery
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    • v.42 no.3
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    • pp.361-363
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    • 2009
  • A 17-year-old male patient was referred with symptoms of dyspnea. Multi-detector computerized tomography (MDCT) and echocardiography evaluation revealed quadricuspid aortic and pulmonary valves, an atrial septal defect (ASD), and pulmonary stenosis. We closed the ASD using a bovine patch and performed a commissurotomy of the pulmonary valve. Quadricuspid semilunar valves are very rare congenital abnormalities that are reported to occur nine times more frequently in the pulmonic valve than in the aortic valve. According to the Hurwitz Roberts classification, the aortic valve was type A, and the pulmonic valve was type B. The aortic valve normal function, but the pulmonic valve was stenotic and had abnormal function.

Type 4 Dual Left Anterior Descending Artery: A Case Report of a Rare Congenital Coronary Anomaly (제4형 이중 좌전하행 관상동맥: 드문 선천성 관상동맥 기형에 대한 증례 보고)

  • Seon Woong Jang;Ki Hwan Kim;Byung Hoon Lee
    • Journal of the Korean Society of Radiology
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    • v.85 no.2
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    • pp.428-433
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    • 2024
  • Dual left anterior descending artery (LAD) is a rare congenital coronary artery anomaly with a prevalence of approximately 1% in the general population. To date, 10 types of dual LAD artery anomalies have been reported. Among these, type 4 is one of the rarest. Knowledge and recognition of the dual LAD artery are important for correct diagnosis and planning of coronary bypass surgery and percutaneous coronary intervention. We report a case of a 59-year-old male with type 4 dual LAD artery who presented with dyspepsia and sweating for several months and had approximately 50%-70% stenosis in a major diagonal branch off the short LAD artery.

Congenital Cardiac Anomaly Combined with the VATER Association - A case report - (VATER 증후군에 동반된 선천성 심장기형 치험 1례)

  • Ban, Dong-Gyu;Kim, Hyuck;Jee, Heng-Ok;Chung, Won-Sang;Kang, Jung-Ho;Kim, Young-Hak;Lee, Chul-Bum;Ham, Shi-Young;Kim, Nam-Su
    • Journal of Chest Surgery
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    • v.34 no.11
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    • pp.858-860
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    • 2001
  • VATER is a complex anomaly of multiple organs and often combined with cardiac anomalies. However, it can be cured with active surgical intervention. We achieved successful result with aggressive surgery in the patient with VATER and report it with references.

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Congenital Pulmonary Vein Stenosis with Normal Anatomical Connection -One case report- (정상적인 해부학적 연결을 가진 선천성 폐정맥 협착증 -치험 1예 보고-)

  • 박준석;장윤희;정미진;강이석;전태국
    • Journal of Chest Surgery
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    • v.37 no.4
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    • pp.364-368
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    • 2004
  • Congenital pulmonary vein stenosis is a rare anomaly and related to high mortality due to progressive pulmonary hypertension and heart failure in infancy. Aggressive anti-failure medication and surgical treatment is recommended. Surgical options are balloon dilatation, endovascular stent, pneumonectomy, lung transplantation, patch grafting, and sutureless repair. We report a case of congenital pulmonary vein stenosis with normal anatomical connection successfully treated with sutureless technique and using pulmonary vasodilators, such as Sildenafil, lloprost and iNO postoperatively.