• Title/Summary/Keyword: aortic arch anomaly

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Diffuse Supravalvar Aortic Stenosis Associated with Congenital Anomaly of the Aortic Valve(Williams Syndrome) -1 case report- (선천성 대동맥 판막 이상과 무명동맥 협착이 동반된 미만성 대동맥 판막상부 협착증 치험 1례)

  • 김수철;전순호
    • Journal of Chest Surgery
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    • v.33 no.9
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    • pp.748-751
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    • 2000
  • The diffuse form of supravalvar aortic stenosis represents a surgical challenge when the ascending aorta, aortic arch, proximal descending thoracic aorta and arch arteries are involved. It can be treated by a variety of surgical approaches. We report a case of severe diffuse supravalvar aortic stenosis combined with an aortic valve anomaly and occlusion of the right coronary artery ostium in a 14-year-old boy with Williams syndrome. We enlarged the aortic root(Nick's procedure), ascending aorta, aortic arch, proximal descending thoracic aorta, and innominate artery with patches and replaced aortic valve with 19 mm St. Jude valve. Deep hypothermic circulatory arrest and retrograde cerebral perfusion were used during repair of the arch and arch artery.

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Interruption of the Aortic Arch Associated with Single Ventricle, D-Transposition of Great Vessels, and Patent Ductus Arteriosus -Report of A Case- (대동맥전환증 및 단일심실과 동반된 대동맥궁 결손 1례 보고)

  • 유병하
    • Journal of Chest Surgery
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    • v.12 no.2
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    • pp.135-139
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    • 1979
  • Interruption of the aortic arch may be defined as discontinuity of the aortic arch in which either an aortic branch vessel or a patent ductus arteriosus supplies the descending aorta. This uncommon lesion was described first by Raphe Steidele in 1778 and was later classified into 3 types by Celoria and Patton. This anomaly rarely occurs as an isolated anomaly. Most commonly, a ventricular septal defect, patent ductus arteriosus, and abnormal arrangement of the brachiocephalic arteries occurs together with arch anomaly. Rarely, more complex anomaly, such as transposition of the great vessel, or single ventricle, is coexistent. We present the case of an 6 year-old boy with D-transposition of great vessel single ventricle, patent ductus arteriosus and patent foramen ovale with interruption of the aortic arch (Type A).

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A New Method of One Stage Correction of Taussig-Bing Anomaly with Interrupted Aortic Arch -1 case report- (대동맥궁 단절을 동반한 Taussig-Bing 기형에서 새로운 일차적 완전 교정술 - 1례 보고 -)

  • 정종필;서동만
    • Journal of Chest Surgery
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    • v.30 no.1
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    • pp.83-87
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    • 1997
  • Taussig-Bing anomaly is infrequently associated with interrupted aortic arch and size discrepancy of great arteries makes it difficult to undergo arch reconstruction and arterial switch operation. A 20-day old male infant was admitted with the diagnosis of Taussig-Bing anomaly with type B Interrupted aortic arch. Multi-organ failure, due to the diminution of ductal flow, was stabilized after 3 weeks of prostaglandin El and controlled ventilatory support. The surgical correction consisted of VSD closure, arterial swtich and extended aortic arch reconstruction. The marked disparity between the hypoplastic ascending aorta and the dilated main pulmonary artery was overcome by constructing distal neoaorta using both native ascending and descending aortic tissue. The patient was extubated on postoperative 2nd day Postoperative catheterization showed no left ventricular outflow obstruction, no intracardiac shunt, and no incompetence of neoaortic valve.

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Double Aortic Arch with Right Sided Descending Aorta - Report of 1 case - (우측 하행 대동맥을 동반한 중복 대동맥궁 - 1례 수술 치험 -)

  • 조경수
    • Journal of Chest Surgery
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    • v.23 no.1
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    • pp.201-204
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    • 1990
  • The double aortic arch is the commonest anomaly among the vascular rings are relatively rare congenital vascular anomalies. This anomaly is malformation of the aortic arch system may, by compression of the trachea and esophagus, cause respiratory distress and dysphagia. We experienced one case of double aortic arch with right sided descending aorta with predominant right anterior arch treated surgically at Kyung Hee University Medical Center. 1-year-old male patient with acute airway obstruction due to combination of double aortic arch and right descending aorta. The diagnosis was made by simple X-ray & confirmed by barium esophagogram & aortogram. The operative approach was through left thoracotomy & underwent division of the left aortic arch & division of ligamentum arteriosum & suspension of divided proximal end of anterior arch to anterior thoracic wall. The postoperative courses was uneventful and doing well on the 3 years.

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Anatomical Repair of Taussig-Bing Anomaly with Interrupted Aortic Arch and Intramural Left Coronary Artery (벽속관상동맥 기형과 대동맥궁 단절을 함께 동반한 Taussig-Bing심기형의 해부학적 완전교정)

  • 성시찬;김시호
    • Journal of Chest Surgery
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    • v.34 no.10
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    • pp.775-780
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    • 2001
  • We report a case of the successful anatomical correction of the Taussig-Bing anomaly associated with the interrupted aortic arch and intramural left coronary artery for an 38 day-old infant Aortic arch and neoaortic reconstructions were conducted without any prosthetic or pericardial patch. Intramural left coronary was separated from right one after partial detachment of aortic commissure and both coronary artery buttons were transferred separately to the proximal main pulmonary artery(nee-aorta). Delayed sternal closure was done 3 days after the operation and hospital discharge was delayed for a month because of postoperative pneumonia. Now he is 5 months old and free of symptoms and cardiac drugs.

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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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Interrupted Aortic Arch [Type A] associated with ventricular septal defect, patent ductus arteriosus and patent foramen ovale (심실중격결손증, 개방성 대동맥관 및 개방성난원공과 동반한 대동맥궁 결손증: 1례보고)

  • 김한용
    • Journal of Chest Surgery
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    • v.24 no.2
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    • pp.206-211
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    • 1991
  • Interruption of the aortic arch may be defined as discontinuity of the aortic arch in which either an aortic vessel or a patent ductus arteriosus supplies the descending aorta. This anomaly is a rare congenital malformation that usually occurs with severe associated intracardiac congenital anomalies, such as ventricular septal defect, patent foramen ovale and abnormal arrangement of the brachiocephalic arteries. Rarely, transposition of the great vessel, truncus arteriosus are coexistent. We experienced a case of the interrupted aortic arch [Type A] associated with VSD, PDA and patent foramen ovale in a 16 years old female. One stage total correction was done under profound hypothermia with total circulatory arrest. Aortic continuity was established using patent ductus arteriosus with anterior wall of main pulmonary artery, which was anastomosed obliquely to anteromedial side of the ascending aorta. Ventricular septal defect was closed using Dacron patch and patent foramen ovale was closed directly. Postoperative course was uneventful, except mild hoarseness.

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Translocation of the Aortic Arch with Norwood Procedure for Hypoplastic Left Heart Syndrome Variant with Circumflex Retroesophageal Aortic Arch

  • Lee, Chee-Hoon;Seo, Dong Ju;Bang, Ji Hyun;Goo, Hyun Woo;Park, Jeong-Jun
    • Journal of Chest Surgery
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    • v.47 no.4
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    • pp.389-393
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    • 2014
  • Retroesophageal aortic arch, in which the aortic arch crosses the midline behind the esophagus to the contralateral side, is a rare form of vascular anomaly. The complete form may cause symptoms by compressing the esophagus or the trachea and need a surgical intervention. We report a rare case of a hypoplastic left heart syndrome variant with the left retroesophageal circumflex aortic arch in which the left aortic arch, retroesophageal circumflex aorta, and the right descending aorta with the aberrant right subclavian artery encircle the esophagus completely, thus causing central bronchial compression. Bilateral pulmonary artery banding and subsequent modified Norwood procedure with extensive mobilization and creation of the neo-aorta were performed. As a result of the successful translocation of the aorta, the airway compression was relieved. The patient underwent the second-stage operation and is doing well currently.

Persistent Fifth Aortic Arch with Coarctation

  • Kim, Sue Hyun;Choi, Eun-Suk;Cho, Sungkyu;Kim, Woong-Han
    • Journal of Chest Surgery
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    • v.49 no.1
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    • pp.39-41
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    • 2016
  • Persistent fifth aortic arch (PFAA) is a rare congenital anomaly of the aortic arch frequently associated with other cardiovascular anomalies, such as tetralogy of Fallot and aortic arch coarctation or interruption. We report the case of a neonate with PFAA with coarctation who successfully underwent surgical repair.

Arterial Switch Operation in Complex Congenital Heart Diseaes (Application, procedure analysis,risk assessment,and results) (복잡 심기형에서의 동맥 전환술에 대한 연구)

  • 김용진
    • Journal of Chest Surgery
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    • v.28 no.3
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    • pp.237-246
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    • 1995
  • Between March 1989 and December 1994, one-stage repair was performed for correction of the intracardiac malformations associated with aortic coarctation in 34 patients or interrupted aortic arch in 8 patients via median sternotomy. There were 26 male and 16 female patients, and their body weight ranged from 1.8 to 8 kg [mean weight, 4.0 1.4 kg . The age at the operation ranged from 7 days to 18 months [mean age, 3.1 $\pm$ 3.8 months . The repair of aortic coarctation or interrupted aortic arch was performed using extended end-to-end anastomosis in most of the patients [86%, 36/42 , and six patients underwent ductal tissue excision and patch aortoplasty. Intracardiac defects were corrected concomitantly through the right atrium unless the anatomy dictated otherwise. Obstructive outlet septum was resected whenever necessary. There were seven early deaths [16.8 % , and three late deaths with a mean follow-up period of 25 months [range from 1 to 65 months . Three patients were reoperated upon residual subaortic stenosis, stenosis at the RPA origin, and subacute bacterial endocarditis respectively. None showed any significant residual or anastomotic stenosis postoperatively. One stage repair of the aortic coarctation and interrupted aortic arch associated with intracardiac defect leaves no native coarctation shelf tissue or residual hypoplasia in the repaired segment, has low incidence of recurrent or residual stenosis, minimizes reoperation and incisions, and manages arch hypoplasia easily. We concluded that surgical results of one-stage repair for the intracardiac malformation associated with aortic coarctation or interrupted aortic arch are reasonable.

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