• 제목/요약/키워드: Cardiac malformation

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Rastelli 씨 수술을 이용한 선천성 교정형 대동맥 전치증 (I.D.D.) ; 1예 보고 (Rastelli Operation In Congenitally Corrected Transposition Of Great Arteries (I.D.D.) -A Case Report-)

  • 조범구
    • Journal of Chest Surgery
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    • 제12권3호
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    • pp.215-224
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    • 1979
  • Corrected transposition of great arteries associated with dextrocardia [I.D.D.] is a very rare congenital cardiac malformation in which the atria and ventricles are in discordant relation, as are the ventricles and great arteries, but the aorta is to the right of the pulmonary artery. A 13 year old male patient who complained cyanosis and dyspnea on exertion for 8 years, was diagnosed as congenitally corrected transposition of great arteries associated with dextrocardia [I.D.D.], large ventricular septal defect, patent foramen ovale and pulmonary stenosis. He was operated on Nov. 22 1978. The ventricular septal defect was closed with Teflon felt and the pulmonary hypoplasia was corrected with Rastelli operation successfully. On 34th postoperative days, complete heart block was occurred and permanent epicardial pacemaker was implanted with good result.

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개방성 동맥관 합병 심실중격결손증 -7례 보고- (Ventricular Septal Defect Associated With Patent Ductus Arteriosus -A Report Of 7 Cases-)

  • 이상호
    • Journal of Chest Surgery
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    • 제12권3호
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    • pp.233-239
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    • 1979
  • Ventricular septal defect associated with patent ductus arteriosus is well known cardiac anomaly that can be treated successfully by operation. This anomaly is found at low frequency. Seven cases of the combined malformation were operated on during the period from 1959 to 1978 in the Department of Thoracic Surgery, Seoul National University Hospital. Right heart catheterization and retrograde aortogram can diagnose the disease correctly. Staged operation in one case and one-stage operation in 5 patients were done. In all cases of the open heart surgery the closure of the ductus was done by transpulmonary suture of the PDA opening. Among 6 patients of the open heart surgery 2 operative death [33.3%] and one late death [total 50%] were noted. Four type II and one type I and one Type IV VSDs were found. In one case the anomaly was accompanied with aortic insufficiency by herniation of the right coronary cusp through Type I VSD.

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좌심실-우심방 단락 치험 1례 (Left ventricular-right atrial communication (One case report))

  • 오세웅
    • Journal of Chest Surgery
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    • 제19권4호
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    • pp.683-687
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    • 1986
  • A direct communication between the left ventricle and right atrium is a relatively uncommon defect. Familiarity with this anomaly has become increasingly important, however, since the preoperative findings may be identical with those of an atrial septal defect. The left ventricle is directly related to the right atrium over. an area of the membranous ventricular septum which extends superior to the septal attachment of the right atrioventricular valve in the LV-RA communication. The clinical triad of a ventricular septal defect murmur, cardiac enlargement, and an arteriovenous shunt at atrial level is characteristic of the malformation. A 2-year-old boy with left ventriculo-right atrial communication has operated at the Maryknoll Hospital. Under the cardiopulmonary bypass, the atrium was opened, there was a jet-blood stream just above the atria-ventricular portion adjacent to the septal leaflet of the tricuspid valve.

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Berry 증후군의 신생아기 일차완전교정술 (One Stage Repair of Berry Syndrome in a Neonate)

  • 최창휴;곽재건;김진현;정요천;김동진;오세진;이정렬;김용진;노준량
    • Journal of Chest Surgery
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    • 제37권11호
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    • pp.918-921
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    • 2004
  • 대동맥궁단절과 우폐동맥이 대동맥에서 기시하는 형태의 대동맥폐동맥창의 조합인 Berry증후군은 매우 드문 복합심기형의 하나이다. 14일된 신생아에 있어서 국소순환하에 순환정지 없이 일차완전교정술을 시행하여 좋은 결과를 얻었기에 보고하는 바이다.

고양이에서 삼첨판과 승모판의 형성이상 진단 증례 (Diagnostic Imaging of Tricuspid and Mitral Valve Dysplasia in a Cat)

  • 정주현;채웅주;장진화;김대용;윤정희;최민철
    • 한국임상수의학회지
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    • 제24권3호
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    • pp.444-448
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    • 2007
  • An intact female, 2 month old, domestic short hair cat, weighing 0.82kg with a history of respiratory distress, anorexia, and depression for 3 days was referred. In laboratory examination, there were no remarkable abnormalities. Radiographic findings mainly included pleural effusion, dilation of pulmonary vessels, pulmonary edema, and ascites. Echocardiography showed dilation of four cardiac chambers, malformation of the tricuspid and mitral valve, and atrioventricular valve regurgitant jet flow. The cat was euthanized by client's request and confirmed to the tricuspid and mitral valve dysplasia by necropsy and histopathologic examination.

Echocardiographic features of indirect Gerbode defect in a cat

  • Lee, Sang-Kwon;Lee, Namsoon;Cho, Kyoung-Oh;Soliman, Mahmoud;Yun, Munsu;Choi, Jihye
    • 대한수의학회지
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    • 제59권3호
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    • pp.161-163
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    • 2019
  • This report describes the echocardiographic features of an indirect Gerbode defect in a young cat. Echocardiography revealed high-velocity, turbulent systolic flow directed from left ventricle to right ventricle through a ventricular septal defect. The flow immediately entered the right atrium through a tricuspid septal leaflet. The indirect-type Gerbode defect was confirmed through necropsy. When a high-velocity turbulent flow in the RA without pulmonary hypertension is observed on echocardiography, Gerbode defect should be considered.

Vein of Galen Aneurysmal Malformation : Endovascular Management of 6 Cases in a Single Institute

  • Moon, Jung-Hyeon;Cho, Won-Sang;Kang, Hyun-Seung;Kim, Jeong-Eun;Lee, Seung-Jin;Han, Moon-Hee
    • Journal of Korean Neurosurgical Society
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    • 제50권3호
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    • pp.191-194
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    • 2011
  • Objective : The aim of this study was to analyze the treatment outcome of patients with vein of Galen aneurysmal malformations (VGM). Methods : Clinical and angiographic data of six consecutive patients with VGM were retrospectively reviewed. VGMs were angiographically classified by Yasargil's method. Treatment outcomes were evaluated. Results : Mean age at initial treatment was $4.4{\pm}5.7$ months. Angiographic types of VGMs were type II in two patients and type III in four. Three patients had cardiac symptoms and the others were asymptomatic. Two patients were treated with transvenous embolization, three with transarterial embolization, and one was managed conservatively. Two patients died due to venous hypertension few days after transvenous approach. Of three patients who were transarterially embolized, one was completely occluded with Onyx and two were incompletely occluded. During the follow-up period (range, one to six years) two of three patients treated with transarterial approach were asymptomatic and the other showed mild symptoms. One patient who was managed conservatively showed normal performance. Conclusion : Transarterial embolization of VGMs may be better than transvenous approach in terms of the treatment outcome and complication. Further studies are needed because of the rarity of the disease and rapid advancement of endovascular techniques.

Anorectal Malformations Associated with Esophageal Atresia in Neonates

  • Byun, Shin Yun;Lim, Ryoung Kyoung;Park, Kyung Hee;Cho, Yong Hoon;Kim, Hae Young
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제16권1호
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    • pp.28-33
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    • 2013
  • Purpose: Anorectal malformations are often associated with other anomalies, reporting frequency with 40-70%. Gastrointestinal anomalies have been known to be relatively less common than associated anomalies of other organ system. This study was performed to assess a distinctive feature of cases associated with esophageal atresia. Methods: Clinical data (from January 2000 through December 2011) on the 196 subjects with anorectal malformations, managed in our Hospital, were reviewed. Total 14 neonates were identified with accompanying esophageal atresia and retrospective analysis was conducted. Results: The incidence was 7.1% and there were 8 male and 6 female subjects. Only 2 cases were associated with esophageal atresia without tracheoesophageal fistula. Although variable cases of anorectal malformation in female subjects, almost cases were anorectal malformations with rectourethral fistula in male. Other associated anomalies were identified in all cases, with more than 3 anomalies in 10 cases. There were 4 VACTERL (Vertebral abnormalities, Anal atresia, Cardiac anomalies, Tracheoesophageal fistula, Esophageal atresia, Renal and Limb anomalies) associations accounting for 28.6%, but could not identify chromosomal anomaly. Most cases were managed with staged procedure, usually primary repair of esophageal atresia and diverting colostomy. Overall mortality rate was 21.4%, mainly caused by heart problems. Conclusion: This study shows that early diagnosis and rational surgical approach with multidisciplinary plan are mandatory in managing anorectal malformations with esophageal atresia, when considering a high frequency of associated anomaly and a relative high mortality.

신생아에서 Berry 증후군의 자가동맥 피판을 이용한 일차 완전교정술 (One Stage Repair of Berry Syndrome in a Neonate Using an Autologous Arterial Flap)

  • 박진수;성시찬;장윤희;이형두;손봉수;김영규
    • Journal of Chest Surgery
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    • 제41권4호
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    • pp.499-503
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    • 2008
  • 원위부 대동맥-폐동맥창, 우폐동맥 대동맥기시, 온전한 심실중격결손, 개방성동맥관, 대동맥궁차단으로 구성된, 소위 Berry syndrome이라고 불리는 이 기형은 매우 드문 복잡 심기형이다. 생후 19일된 신생아에서 자가동맥 피판을 이용한 일차 완전교정술과 추적관찰에서 발생한 우폐동맥 협착 및 그의 풍선성형술에 의한 성공적 치료를 문헌고찰과 함께 보고한다.

속발성 무월경 외 비정상 표현형이 없는 Trisomy 8 Mosaicism의 증례 보고 (A Case of Trisomy 8 Mosaicism in a Patient with Secondary Amnorreha without Abnormal Phenotype)

  • 강혜심;손영수;김성엽;박철민;심순섭
    • Journal of Genetic Medicine
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    • 제8권1호
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    • pp.67-70
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    • 2011
  • 체질성 8삼염색체 모자이시즘은 비교적 드문 염색체 이상으로 특이한 두개안면 모양 (변형 두개골, 돌출된 이마, 낮거나 변형된 귀), 골격계 기형, 심장 기형, 신장 기형, 잠복고환, 다양한 정도의 발달지체 등의 특징적인 표현형을 갖는다. 그 표현형과 세포유전학적 표현의 다양성 때문에 특정 질환이 생기기 전까지 진단이 늦어지기도 한다. 전형적인 표현형 없이 속발성 무월경을 동반한 28세 여성에서 발견된 체질성 8삼염색체 모자이시즘(47,XX,+8[9]/46,XX[41])을 보고한다.