• Journal of Yeungnam Medical Science
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• v.7 no.1
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• pp.211-214
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• 1990

Portal vein anomalies include absence, duplication, and malposition(preduodenal portal vein). Duplication of the portal vein or a preduodenal portal vein are hazards at the time of biliary or duodenal surgery. or liver transplantation. Preduodenal portal vein, which was first reported by knight in 1921, is extremely rare congenital anomaly and may cause duodenal obstruction. Recently, we experienced a case of preduodenal portal vein associated with dextrocardia, situs inversus, and duodenal obstruction in a 3 days old male newborn and report with review of the references.

• Clinical and Experimental Pediatrics
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• v.45 no.5
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• pp.622-628
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• 2002

Purpose : The purpose of this study is to ascertain the clinical manifestations and associated cardiac anomalies of dextrocardia. Methods : Twenty-four pediatric patients, who were admitted to, or visited, Dongsan Medical Center Keimyung University and were diagnosed with dextrocardia between January 1996 and July 2001, were enrolled in this study. All patients received echocardiogram and abdominal ultrasonogram to identify structural cardiac anomalies and visceral position. Results : Among 24 patients, 7 cases were situs solitus(29.2%), 10 cases(41.6%) were situs inversus and 7 cases(29.2%) were situs ambiguous. Most were diagnosed within a week of life(87.5%). They were most commonly cyanotic(45.8%), but eleven cases(45.8%) had no specific symptoms or signs. Nineteen cases(79.2%) had accompanied cardiac anomalies, and pulmonary stenosis or atresia were the most common among them. All the cases of situs solitus and ambiguous had associated cardiac anomalies, but half of the situs inversus had that. Eleven cases were dead by cardiac or extracardiac anomalies during follow up and the mortality rate was higher in situs solitus or ambiguous group than situs inversus group. Conclusion : Dextrocardia accompanies different cardiac and extracardiac anomalies. It's very important to diagnose dextrocardia and associated cardiac or extracardiac anomalies in the early stages of life to improve prognosis.

• Journal of Chest Surgery
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• v.11 no.3
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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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• v.11 no.3
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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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• v.47 no.2
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• pp.259-264
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• 1999

The scimitar syndrome, a rare complex anomaly, is defined as an anomalous right pulmonary venous drainage, partial or complete, to the inferior vena cava. The shape of the Turkish curved sword (scimitar) has provided the name of this syndrome. Additional characteristics of this syndrome such as hypoplasia of the right lung and of the right pulmonary arterial tree, anomalous arterial supply of the right lung from the aorta, dextrocardia and bronchial anomalies are common. Recently we experienced a case of scimitar syndrome (adult form) in a 19-year-old woman patient, so we report the case with a brief review of the literature.

• Journal of Chest Surgery
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• v.23 no.2
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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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• v.48 no.4
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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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• v.24 no.10
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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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• v.5 no.2
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• pp.192-198
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• 2002

Alagille syndrome is characterized by paucity of interlobular bile ducts, chronic cholestasis, characteristic facial abnormalities, cardiovascular abnormalities, posterior embryotoxon, vertebral arch defects, skeletal abnormalities, and glomerular renal involvement. We experienced a case of Alagille syndrome in a 10 month-old male presenting with jaundice. He had chronic cholestasis, characteristic face, cardiovascular abnormalities (aortic stenosis, dextrocardia, double chamber of left ventricle), and situs inversus. Histological examination of liver biopsy specimen revealed paucity of interlobular bile ducts with septal fibrosis, cirrhotic transformation and severe cholestasis. He underwent liver transplantation, but died of cardiopulmonary arrest associated with cardiac anomaly.

• Journal of Genetic Medicine
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• v.3 no.1
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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.