• Journal of Yeungnam Medical Science
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• 제7권1호
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• pp.211-214
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• 1990

저자들은 최근 생후 3일된 dextrocardia와 situs inversus 및 십이지장 완전 폐색을 동반한 환아에서 십이지장 전방 문맥을 발견하였다. 이에 문헌 고찰과 함께 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제45권5호
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• pp.622-628
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• 2002

목 적 : 저자들은 이차적인 병변으로 인한 우심증이 아닌 환아들에서 그들의 임상양상, 동반 심장 또는 심장외 기형과 예후를 분석하고자 하였다. 방 법: 1996년 1월에서 2001년 7월까지 계명대학교 동산의료원 소아과에서 흉부 방사선 소견상 심장이 우측 흉곽에 위치하였던 환아 중 폐나 흉곽 병변에 의한 우전위를 제외한 24례를 대상으로 하였다. 모든 환아들에게 심에코 검사와 복부 초음파 검사를 시행하였고 필요에 따라 심장 조영술, 염색체 검사를 시행하였으며 이들의 진단 당시의 연령과 임상 증상, 이학적 소견, 동반하는 심기형 뿐 아니라 심장외 기형, 치료 후 예후를 조사하였다. 결 과: 총 24례 중 남아가 17례(79.8%)이었으며, visceroatrial situs로 보았을 때 situs solitus가 7례, situs inversus가 10례, situs ambiguous가 7례이었다. 우심증을 처음 발견한 시기는 생후 7일 이내가 21명(87.5%)으로 대부분을 차지하였다. 진단 당시의 증상으로는 청색증과 심잡음이 각각 11례(45.8%)로 가장 많았고 11례(45.8%)는 증상 없이 우연히 발견되었다. Situs solitus와 inversus의 경우는 우연히 발견된 경우가 증상을 보여 진단된 경우보다 많았으나 situs ambiguous의 경우는 청색증(71.4%) 등의 증상으로 발견된 경우가 많았다. 19례(79.2%)에서 심기형을 동반하고 있었으며 situs solitus와 ambiguous는 전례에서 심기형을 동반하였고 situs inversus는 50%에 서 심기형을 동반하였으며 동반된 심기형 중 폐동맥 협착이나 폐동맥 폐쇄가 12례로 가장 많았다. 심장외 다른 기형이 동반된 경우는 7례(29.2%)로 원선, 비후성 유문 협착증, 요도 하열, 수신증, 다지증, 만곡지 등이었으며 염색체 검사를 시행한 4례는 모두 정상이었다. 총 24례 중 11(45.8%)례는 심기형이나 동반된 다른 선천성 기형으로 사망하였으며, 특히 situs solitus와 ambiguous에서 사망률이 높았다. 결 론 : 우심증의 첫 진단 시기는 1주 이내가 많았고, 이들 중 증상 없이 우연히 발견된 경우도 약 반수로 많았으며, situs solitus와 ambiguous는 동반된 심장기형이 100%로 situs inversus의 50%보다 많았으며 예후 또한 불량하였다.

• Journal of Chest Surgery
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• 제11권3호
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• pp.342-347
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• 1978

A 8 year old male was admitted to the Department of Thoracic Surgery, Korea University Hospital on June 22, 1978. The chief complaints were cyanosis and exertional dyspnea since at birth. EKG shows BVH and dextrocardia, phonocardiogram revealed the accentuation of second heart sound in aortic area. Echocardiogram from the left ventricle to the base of the heart, there is a discontinuity between the ventricular septum and the anterior aortic margin with a large aortic root & aortic overriding. His cardiac catheterization data and cardiac angiogram shows situs inversus totalis, dextrocardia, right aortic arch, large ventricular septal defect etc., and finally diagnosed Truncus Arteriosus. Edwards type IV with retrograde aortogram and selective bronchial angiogram. This is the first operative case reported as Rastelli operation for Truncus Arteriosus type IV in the literatures in Korea. Authors have experienced I case of Truncus Arteriosus, Edward type IV and Rastelli operation with Dacron Arterial Conduit Graft under cardiopulmonary bypass on July 3, 1978. The procedures were as follows; 2] Cardiopulmonary bypass: Origin of bronchial arteries excised from descending aorta bilaterally; defects in aorta closed. 2] Horizontal incision made high in right ventricle. 2] Ventricular septal defect [Kirklin type I+II] closed with Teflon patch. 4] Bifurcated dacron arterial graft with pericardial monocusp sutured to the bilateral pulmonary arteries. [Diameter 9 mm: Length 7 cm]. 5] Proximal end of the conduit graft anastomosed to right ventricle. [Diameter 19 mm: Length 5 cm]..Total perfusion time was 220 min. The result of operation was poor due to anastomotic leakage and increased pulmonary vascular resistance resulting acute right heart failure. The patient was died on the operation table. Literatures were briefly reviewed.

• Journal of Chest Surgery
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• 제11권3호
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• pp.333-341
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• 1978

A 8 year old male was admitted to the Department of Thoracic Surgery, Korea University Hospital on June 22, 1978. The chief complaints were cyanosis and exertional dyspnea since at birth. EKG shows BVH and dextrocardia, phonocardiogram revealed the accentuation of second heart sound in aortic area. Echocardiogram from the left ventricle to the base of the heart, there is a discontinuity between the ventricular septum and the anterior aortic margin with a large aortic root & aortic overriding. His cardiac catheterization data and cardiac angiogram shows situs inversus totalis, dextrocardia, right aortic arch, large ventricular septal defect etc., and finally diagnosed Truncus Arteriosus. Edwards type IV with retrograde aortogram and selective bronchial angiogram. This is the first operative case reported as Rastelli operation for Truncus Arteriosus type IV in the literatures in Korea. Authors have experienced I case of Truncus Arteriosus, Edward type IV and Rastelli operation with Dacron Arterial Conduit Graft under cardiopulmonary bypass on July 3, 1978. The procedures were as follows; 2] Cardiopulmonary bypass: Origin of bronchial arteries excised from descending aorta bilaterally; defects in aorta closed. 2] Horizontal incision made high in right ventricle. 2] Ventricular septal defect [Kirklin type I+II] closed with Teflon patch. 4] Bifurcated dacron arterial graft with pericardial monocusp sutured to the bilateral pulmonary arteries. [Diameter 9 mm: Length 7 cm]. 5] Proximal end of the conduit graft anastomosed to right ventricle. [Diameter 19 mm: Length 5 cm]..Total perfusion time was 220 min. The result of operation was poor due to anastomotic leakage and increased pulmonary vascular resistance resulting acute right heart failure. The patient was died on the operation table. Literatures were briefly reviewed.

• Tuberculosis and Respiratory Diseases
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• 제47권2호
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• pp.259-264
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• 1999

저자들은 흉부 불쾌감 및 호흡곤란을 주소로 내원한 19세 여자환자에서 우연히 흉부사진상 반월도(scimitar) 모양의 음영 및 우측 폐 형성부전이 발견되어 심초음파, 전산화 단층촬영 혈관조영술, 심도자 및 심혈관 조영술 등으로 확진하여 보존적 치료를 시행한 성인형 Scimitar 증후군을 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• 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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• 제48권4호
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• pp.277-280
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• 2015

We report the case of a 37-year-old man who suffered from biventricular failure due to left isomerism, inferior vena cava interruption with azygos vein continuation, bilateral superior vena cava, double outlet of right ventricle, complete atrioventricular septal defect, pulmonary stenosis, and isolated dextrocardia. Heart transplantation in patients with systemic venous anomalies often requires the correction and reconstruction of the upper & lower venous drainage. We present a case of heart transplantation in a patient with left isomerism, highlighting technical modifications to the procedure, including the unifocalization of the caval veins and reconstruction with patch augmentation.

• Journal of Chest Surgery
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• 제24권10호
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• pp.960-966
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• 1991

From February 1984 through July 1991, 104 infants less than 1 year of age with congenital heart defects underwent open heart repair with conventional cardiopulmonary bypass which occupied 10.7% of all patients with congenital heart defects operated on during same period. There were 66 boys and 38 girls 7 days to 12 months [mean age, 8.2 months]. Four patients were neonates, 8 were 1 to 3 months, 23 were 4 to 6 months, and 69 were 7 to 12 months of age. Mean body weight at repair was 6.9kg and mean BSA, 0.36m2 Indications for operation were intractable congestive heart failure and severe pulmonary hypertension in patients with VSD and severe cyanosis and anoxic spells in patients with TOF. Conditions corrected were VSD[79], TOF[8], AVSD[4], PS[2], PA+IVS[2], TAPVC [2], MR[2], DOLV[l], Truncus arteriosus[1], D-TGA[1], and PA-VSD[1]. Twenty-three of 79 patients with VSD had associated cardiovascular anomalies which included PDA in 16 patients, PS in 9 patients, ASD in 5 patients, LSVC in 2 patients, MR in 1 patient, dextrocardia in 1 patient, and single coronary artery in 1 patient. The hospital mortality rate was 24.0% which was much higher than that of 6% in patients over 1 year of age. The greatest mortality occurred in babies of low weight under 6 months of age, There was no late death. Surviving infants showed marked symptomatic improvement and change in growth patterns. These surgical results were to be overcome with proper pre- and post-operative management and improvement of surgical technique

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제5권2호
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• pp.192-198
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• 2002

저자들은 지속적인 황달을 주소로 연세의대 세브란스병원에 입원하였던 10개월 된 남아에서 특징적인 얼굴모양, 심혈관계 이상, 간 생검 조직검사상 담즙 정체, 거대세포 형성 및 소염관 담관의 수가 감소되어 있었고, 퇴원 후 간경변으로 이행되어 간 이식수술을 받았던 Alagille증후군 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of Genetic Medicine
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• 제3권1호
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• pp.1-4
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• 1999

Scimitar syndrome is a rare congenital anomaly that is characterized by hypoplasia of the right lung and the right pulmonary artery with anomalous pulmonary venous drainage to the inferior vena cava. The scimitar vein is usually visible on chest radiographs, but may be obscured by the heart. It is essential for surgical correction to establish the point of drainage of the anomalous vein and associated anomalies. There are recent reports of familial total anomalous pulmonary venous return suggesting heritable forms of this anomaly. Although genetic factors are believed to have important roles in congenital heart disease, few genes involved in heart development have been located. We report a case of familial chromosome 9 inversion with Scimitar syndrome in an offspring who presented with dextrocardia. Evaluation with magnetic resonance cineangiograph imaging demonstrated an anomalous pulmonary vein draining into the inferior vena cava above the diaphragm and hypoplasia of the right lung and the right pulmonary artery. Chromsome analysis showed pericentric inversion of chromosome 9, inv 9 (p13, q21), in the patient and his mother as well. A brief review of the related literature is also included.