• Clinical and Experimental Pediatrics
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• 제55권1호
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• pp.29-33
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• 2012

PHACE association is a rare neurocutaneous condition in which facial hemangiomas associate with a spectrum of posterior fossa malformations, arterial cerebrovascular anomalies, cardiovascular anomalies, and eye anomalies. We reported a case of PHACE association in a premature infant showing facial, intracranial, and oropharyngeal hemangiomas with evidence of the Dandy-Walker variant and complicated cardiovascular anomalies, including a right-sided aortic arch and an atypical patent ductus arteriosus arising from a tortuous left subclavian artery. To our knowledge, intracranial hemangiomas are rare in PHACE association, and a concomitant oropharyngeal hemangioma has not been previously reported in the PHACE association literature. In infants presenting with large, plaque-like facial hemangiomas, it is important to conduct active cardiovascular and neurological evaluations. Special attention should be given to the laryngoscopic examination to search for additional hemangiomas in the airway.

• Journal of Chest Surgery
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• 제17권3호
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• pp.448-455
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• 1984

Coarctation of the Aorta is a congenital constriction of aorta of varying degree, usually located at or near the aortic ismuth with frequent associations of other cardiac anomalies. Various modes of surgical corrections, such as resection and end-to-end anastomosis, graft interposition, angioplasty using prosthetic patch or subclavian flap have been used according to the status of coarctation and age of the patient. We have experienced two cases of surgically treated coarctation of the aorta, one of which was preductal coarctation with hypoplastic aortic arch and ventricular septal defect in a 4 year old boy, and the other case was juxtaductal type with aortic regurgitation. Subclavian flap angioplasty with additional pulmonary artery banding procedure was done in the first case and wedge resection with end-to-end anastomosis and aortic valve replacement [St. Jude valve, 23mm] 20 days later of first operation in the other case. The first case developed massive tarry stool on 3rd POD, probably due to mesenteric arteritis with resultant bowl ecrosis, and expired the next day. Recovery was uneventful with the second case.

• Journal of Chest Surgery
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• 제26권1호
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• pp.36-43
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• 1993

Sixty four children [aged 2 days to 9 years] , 58 with complete transposition of the great arteries, 5 with Taussig-Bing double outlet right ventricle, and 1 with double outlet left ventricle plus left ventricular type single ventricle, have undergone anatomic correction from November 1987 to August 1992. Eleven underwent previous operations: pulmonary artery banding[7], modified Blalock-Taussig shunt[2], coarctoplasty[2], aortic arch reconstruction[1] . Of 58 patients with TGA, Type A coronary arteries of Yacoub were seen in 50[86%]. U-shaped coroanry arterial flaps were transfered to the neoaorta using trap door technique, and neopulmonary arterial tract was constructed using glutaraldehyde fixed autopericardium with Lecompte maneuver. There were 18 hospital deaths [28.1%] with no late mortality. Mean follow-up of 20.4\ulcorner11.9 months were achieved in all survivors. Postoperative cardiac catheterizations were done in 14 cases. Mean pressure gradients of pulmonary and aortic outflow tract were 15.0 $\pm$2.6 and 4.2$\pm$1.4mmHg, mild aortic valve insufficiencies were found in 2, and mean cardiac index was 5.18$\pm$0.19 L/min/M2. We conclude that we should continue anatomic correction for the complex congenital heart anomalies with the malposition of the great arteries because myocardial function seems to be well preserved, though we are still on the learning curve.

• Journal of Chest Surgery
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• 제19권1호
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• pp.165-173
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• 1986

During the 4 year period from 1982 through 1985, twelve patients have undergone operations for discrete subaortic stenosis with good short-term clinical result at Department of Thoracic and cardiovascular Surgery, S.N.U.H. According to the cineangiographic and operative findings, nine of the 12 patients were classified as Deutsch type I, the other 3 as type II, and eleven of the 12 had one or more associated anomalies of the cardiovascular system such as PDA[5], VSD[5], left SVC[2], MS[1], COA[1], supramitral membrane[1], DORY[1], right aortic arch[1], DCRV[1], and TOF[1] [one with Shone`s complex], and three of them had secondary cardiac disorders such as aortic regurgitation[3],mitral regurgitation[2], and tunnel shaped dynamic obstruction of left ventricular outflow tract[2]. We have performed membrane resection via oblique aortotomy with retraction of the aortic cusps in 7 cases and via VSD from right cardiac chamber in 5 cases with large VSD and have also performed the operations on the correctable associated anomalies. There was only one operative death in patient with associated TOF due to neurologic complication and no other postoperative difficulties except in one patient with transient heart block resolved spontaneously on postoperative 3rd day. To our knowledge, this article is the first report of operation for discrete subaortic stenosis in Korean literature.

• Journal of Chest Surgery
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• 제30권4호
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• pp.363-372
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• 1997

본 연구는 1986년 6월 3일부터 1995년 12월 31일까지 서울대학교 어린이 병원 흉부외과에서 경험한 144례의 대동맥 교약증 환아를 대상으로, 술후 결과에 영향을 미치는 여러 가지 요소들을 분석하였다. 환아의 평균 연령은 15.73+32.43(범 위 0.1-191.0, 중앙값=3.3)개월이 었고, 이중 11)례(78.5%)의 환아는 영아, 25례(17.4%)는 신생아였다. 환아를 대동맥궁 협 착 부위와 동반 심기형에 따라(제 1형:국소 협착, 제2형:대동맥 헙부 협착, 제3형 :대동맥 협부 및 횡대동맥궁 협착, h:심실중격결손증 동반, B:기타 복 잡 심기 형 동반) 9가지로 분류한 결과, 1, IA, IB, I, IIA, IIB, III, IIIA, IIIB형이 각각 25, 9, 6, 24, 35, 15, 4, 19, 7례 였다. 수술방법은 쇄골하동맥편 교약성형술(subclavian-flap coarctoplasty, SFC: 60례), 절단 및 문합술(resection and anastomo sis, R&A: 44례), 확장된 대동맥성형술(extended aortoplasty, Ex-Ao: 26 례), 덮개포편술(onlay patch, Onlay: 14례)이 사용되었다. 총수술 사망률은 16.0%(23/144)였고, 수술 사망률을 높이는 인자로 협착부위(I, II, III형) 동반 심기형(0, A, B), 환아의 연령, 수술방법, 단계수술 \ulcorner부 등이 분석되었는데 이중 횡대동맥궁협착을 동반하는 III형(I형 사망률=2.5%(1/40), II형=17.6% (13/74), III형=30%(9/30); p(0.01), B형(0형 사망률=3.8%(2/53), A형 =15.9%(10/63), B형 =39.3%(11/28); p(0.01) 등이 의미있는 위험 인자였다. 생존환아 121례는 술후 평균 29.1+28.8(범위 0-129.2)개월 외래 추 적되 었으며 이중 술후 협착을 보였던 경우는 18례로 14.9%의 헙착률은 보였다. 생존환자중 77례의 환자 (I형 20명. ll형 42명, 111형 15명)에서 술전, 술후 3개월 이내, 술후 6개월이후의 심에코도상의 대동맥 각 부위 크기에 관한 자료의 입수가 가능하였으며 이를토대로무명동맥 직근위부의 상행대동맥 직경에 대 한 대동맥협부 직경의 비율(대동맥협부지수)및 경동맥부위의 횡대동맥 직경비율(횡대동맥지수)을구하 여 형태 별로 술전, 수술 직후 6개월이상 경과후의 대동맥 크기 변화의 양상을 관찰하였다. 제 I, II, III형 모두에서 술전에 비하여 술후 평균 대동맥협부지수의 의미있는 증가가 관찰되었으며(p<0.01),제 III형에서는 횡대동맥지수의 의미있는 증가도 관찰되었다(p<0.01). 재헙착으로 진단된 I, II형의 수술직후 대 동맥협부지수와 111형의 횡대동맥 지수는 비협착군에 비하여 의미있게 작았는데 이는 아마도수술 당시 협 착 부위의 완전제거가 이루어지지 않은 것이 원인이라고 사료되었다. 본 분석에서는 어린 연령(3개월 이하), 3개월이하에 시행한 쇄골하동맥편 교약성형술이 의미있는 재협착의 위험요소로 밝혀졌다. 결론 적으로 저자등은 본연구를 통하여 대동맥협부지수, 횡대동맥 지수 등이 개개 대동맥교약 환아의 해부학 적, 임상적 특징을 파악하는데 도움이 되는 도구라는 사실을 발견하였고 아울러 교약의 해부학적 특성, 동반 심기 형, 연령, 수술방법 등이 수술사망 및 재협착에 영향한다는 사실을 입증하였다.

• Journal of Chest Surgery
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• 제24권7호
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• pp.693-700
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• 1991

A rare case of the association of distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum, patent ductus arteriosus and interrupted aortic isthmus in a 40-day-old infant is reported. The infant was suffered from two operations with an interval of nine days. At the first operation a 10mm polytetrafluoroethylene prosthesis was inserted instead of the interrupted aortic isthmus and ductus was ligated via the left posterolateral thoracotomy. But the patient could not be weaned from the respirator because of large amount of left-to-right shunt. So the total correction was subsequently performed after an interval of nine days. At the second operation, tunneling of the right pulmonary artery to the main pulmonary artery through the aortopulmonary septal defect was performed using the Dacron patch via a longitudinal transaortic approach and a separate autologous pericardial patch was applied to the longitudinally incised margins of the anterior wall of the ascending aorta. The second postoperative course was relatively uneventful except some respiratory distress and nutritional problems. Now he is at 6 months of age and thrives well without any symptom. Because the success of the surgical repair of this complex anomalies depends upon the accurate diagnosis and meticulous design of each step of procedure prior to operation these problems are also discussed.

• Journal of Chest Surgery
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• 제23권2호
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• pp.253-259
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• 1990

Between January, 1970 and August, 1989, a total of 81 patients whose age were more than 20 years of life, received total correction for tetralogy of Fallot. This report analyzed 70 patients among them and excluded the remaining 11 patients whose clinical data could not be found. Their mean age was 25.750.39 years[range 20 \ulcorner50]. The clinical manifestations were cyanosis and clubbing [64 pts], frequent URI[40 pts], anoxic spell [19 pts], infective endo-carditis[4 pts], brain abscess[3 pts], pulmonary tuberculosis[3 pts] and CHF, chest tightness, nephrotic syndrome, left hemiplegia, and tamponade. The types of right ventricular outflow tract obstruction were combined[46 pts], pure infundibular [21 pts] and pure valvular[3 pts]. Associated cardiovascular anomalies were PFO [27 pts], ASDi8 pts], LSVC[8 pts], aortic regurgitation [5 pts], right aortic arch, coronary artery anomalies, PDA and dextrocardia. Hospital mortality was 5.7%. The causes of death ware low cardiac output [2 pts], aggravation of CRF[1 pts] and brain damage[1 pts]. There was one late death because of residual intracardiac shunt and congestive heart failure. During the follow-up period, 16 patients were lost and the remaining 49 patients were asymptomatic and leading normal lives. Residual intracardiac shunt was detected in 5 patients with radionuclide single pass study but all of them had Qp / Qs ratio less than 1.5.

• Journal of Chest Surgery
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• 제42권6호
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• pp.749-756
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• 2009

배경: 혈관륜은 대동맥궁의 드문 기형으로 저자들이 12년 간 경험한 16예의 수술 결과를 정리하여 그 경험을 공유하고자 한다. 대상 및 방법: 1995년 11월부터 2007년 9월까지 혈관륜으로 수술을 받은 16명을 대상으로 하여 이중 대동맥궁(n=5), 우측 대동맥궁-동맥관 인대(n=7), 폐동맥 슬링(n=4)의 세 가지 그룹으로 분류하였다. 각 그룹의 수술 당시 평균 연령은 이중 대동맥궁 그룹에서 $5.7{\pm}5.5$세, 우측 대동맥궁-동맥관 인대 그룹에서 $6.1{\pm}13.4$세, 폐동맥 슬링 그룹에서 $2.9{\pm}2.6$세였다. 우측 대동맥궁-동맥관 인대 그룹의 71% (n=5)에서 Kommerell 게실이 동반되어 있었다. 기관 협착은 이중 대동맥궁 그룹 중 2예(40%), 우측 대동맥궁-동맥관 인대 그룹 중 2예(28.6%), 폐동맥 슬링 그룹 중 4예 (100%)에서 동반되어 있었다. 함께 진단된 심장 기형은 전체의 50% (n=8)였다. 결과: 사망한 예는 없었고, 증상 재발 및 문합부 협착 등으로 재수술을 시행한 예도 없었다. 평균 재원 기간은 $27.1{\pm}38.2$일이었다. 결론: 혈관륜은 드문 질환이지만 수술적 교정이 필요하므로, 원인이 명확하지 않은 호흡기 증상이 반복될 경우에는 심초음파 등으로 반드시 확인해 보아야 하며, 수술 전후의 전산화 단층촬영 및 기관지 내시경은 기도 협착 및 주변 해부학적 구조와의 관계 평가에 유용하게 이용할 수 있다.

• Journal of Korean Neurosurgical Society
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• 제62권2호
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• pp.175-182
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• 2019

Objective : Aberrant right subclavian artery (ARSA) is a rare anatomical variant of the origin of the right subclavian artery. ARSA is defined as the right subclavian artery originating as the final branch of the aortic arch. The purpose of this study is to determine the prevalence and the anatomy of ARSA evaluated with computed tomography (CT) angiography. Methods : CT angiography was performed in 3460 patients between March 1, 2014 and November 30, 2015 and the results were analyzed. The origin of the ARSA, course of the vessel, possible inadvertent ARSA puncture site during subclavian vein catheterization, Kommerell diverticula, and associated vascular anomalies were evaluated. We used the literature to review the clinical importance of ARSA. Results : Seventeen in 3460 patients had ARSA. All ARSAs in 17 patients originated from the posterior aspect of the aortic arch and traveled along a retroesophageal course to the right thoracic outlet. All 17 ARSAs were located in the anterior portion from first to fourth thoracic vertebral bodies and were located near the right subclavian vein at the medial third of the clavicle. Only one of 17 patients presented with dysphagia. Conclusion : It is important to be aware ARSA before surgical approaches to upper thoracic vertebrae in order to avoid complications and effect proper treatment. In patients with a known ARSA, a right transradial approach for aortography or cerebral angiography should be changed to a left radial artery or transfemoral approach.

• 대한수의학회지
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• 제64권2호
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• pp.11.1-11.5
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• 2024

A 4-month-old intact male Sapsaree dog was referred due to a history of postprandial regurgitation following consumption of solid food. Thoracic radiography revealed focal leftward displacement of the thoracic trachea at T1 to T4 vertebrae levels. Barium contrast radiography revealed focal dilation of the cranial thoracic esophagus at the heart base level. Persistent right aortic arch (PRAA) with an aberrant left subclavian artery branching from the patent ductus arteriosus was diagnosed by computed tomography angiography (CTA). Although barium contrast radiography can presumptive diagnose PRAA, CTA should be considered for identifying additional vascular anomalies, specific types, and surgical planning.