• Journal of Chest Surgery
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• v.20 no.3
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• pp.489-497
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• 1987

Six hundred and twenty two cases of open heart surgery were performed at Sejong General Hospital in 1986. And also, 117 cases of non open heart cardiac surgery had been performed during same period. Among the 622 open heart cases, 548 were congenital cardiac diseases and 74 were acquired heart diseases. In congenital heart patients, 422 were acyanotic and 126 were cyanotic. There were 52 cases of infant open heart Surgery below 12 months. Acyanotic group were consisted of 314 VSD, 66 ASD, 13 AVSD, 9 PDA, 8 ASD + PS, 4 AS, and 8 other rare cardiac cases. And cyanotic group were consisted of 84 TOF, 15 DORV, 5 Trilogy, 4 Ebstein`s anomaly, 3 PS + TR, 3 TGA, 3 TAPVR, 3 Pulmonary atresia and 6 other rare cardiac diseases. Majority of the acquired heart cases were valvular heart diseases. And there were also 4 cardiac myxoma and one endomyocardial fibrosis in acquired heart disease group. The operative results were as follows: Overall operative mortality, 5.3%: acyanotic 2.4%: cyanotic 15.8% and acquired heart disease, 8.5%.

• Journal of Chest Surgery
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• v.15 no.4
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• pp.373-380
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• 1982

Experience with intracardiac surgery in infants indicates that for most anomalies the operation and late mortality after primary total correction is lower than the combined mortality after early palliation and delayed correction. In addition, there are secondary benefits of primary total correction in terms of reversal of retarded physical growth and social development and alleviation of parental anxiety. One hundred and fifteen infants under 2 years of age with congenital cardiac anomalies underwent primary surgical intervention at Seoul National University Hospital from Jan. `78 to Sep. `82. There were 70 patients with VSD, 17 patients with TOF, 10 patients with TGA, 4 patients with ASD, 4 patients with TA, 3 patients with TAPVR, and the remainders are Coronary AV Fistula, partial ECD+COA, SV, DORV, PA, Trilogy+PDA. The overall surgical mortality was 18.3%. In acyanotic group 6 patients died among 76 operated patients, and surgical mortality was 6.6%. But in cyanotic group, the mortality was very high as41.0% [16 patients among 39 patients]. This poor surgical result in cyanotic group was due to Improper pre-, intra- and post-operative care, and we are convincing that these factors soon be improved as experiences accumulated.

• Journal of Chest Surgery
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• v.32 no.12
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• pp.1111-1114
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• 1999

Cor triatriatum is a rare congenital heart disease that is often lethal in children if not correctly identified and properly managed, Characteristically an anomalous membrane divides the left atrium into two chambers one located posterosuperiorly which is connected to the common pulmonary venous trunk and the other anteroinferiorly which is connected to the left atrial appendage and the mitral valve. Eight patients with Cor triatriatum were been seen at our hospital from 1984 to 1999. The clinical presentation diagnostic evaluation and surgical results are outlined in this retrospective review. Resection of the obstructing anomalous atrial membrane was performed using a hypothermic cardiopulmonary bypass in all cases. Right atriotomy was performed in all patients and left atriotomy was performed in a patient who had poor preoperative general conditions and serious cardiac defects(TAPVR & hypoplastic left ventricle) died of low cardiac output during the immediate postoperative period. The postoperative course has been excellent in the remaining. Cor triatriatum is amenable to surgical repair with excellent results when diagnosed early and in those who are not complicated by other complex cardiac anomalies.

• Clinical and Experimental Pediatrics
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• v.54 no.5
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• pp.183-191
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• 2011

Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD) is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary venous return (TAPVR), which the baby needs immediate diagnosis and management for survival. Unfortunately, these life threatening heart diseases may not have obvious evidence early after birth, most of the clinical and physical findings are nonspecific and vague, which makes the diagnosis difficult. High index of suspicion and astute acumen are essential to decision making. When patent ductus arteriosus (PDA) is opened Widely, many serious malformations may not be noticed easily in the early life, but would progress as severe acidosis/shock/cyanosis or even death as PDA constricts after few hours to days. Ductus dependent congenital cardiac lesions can be divided into the ductus dependent systemic or pulmonary disease, but physiologically quite different from each other and treatment strategy has to be tailored to the clinical status and cardiac malformations. Inevitably early presentation is often regarded as a medical emergency. Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (PPHN) and other pulmonary conditions are necessary. Urgent identification of the newborn at such high risk requires timely referral to a pediatric cardiologist, and timely intervention is the key in reducing mortality and morbidity. This following review deals with the clinical presentations, investigative modalities and approach to management of congenital cardiac malformations presenting in the early life.

• Journal of Chest Surgery
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• v.22 no.6
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• pp.944-950
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• 1989

Children with congenital cardiac defects associated with high pulmonary artery pressure may die despite accurate surgery. Postoperative mortality and morbidity have been attributed to acute rises in pulmonary artery pressure and resistance. Acute pulmonary hypertensive crisis is defined as a paroxysmal event in which pulmonary arterial systolic pressure rises to or above systemic levels followed by a rapid fall in systemic pressure and a minor pulmonary hypertensive event is defined as an acute rise in pulmonary arterial pressure to more than 80 % of systemic levels but without a fall in systemic pressure. From Oct. 1988 to Jul. 1989, we experienced 23 patients who showed many pulmonary hypertensive crises after operation in the Department of Thoracic and Cardiovascular Surgery, Seoul National University Children\ulcorner Hospital. Their preoperative PAP/SAPs were 53 to 123 %[mean 93.3%] and diagnoses were VSD[7], TAPVR[5], TGA[4], AVSD[3], MS[1], DORV[1], Truncus arteriosus[1], and AP window[l]. There were 9 deaths among 23 patients and they showed many pulmonary hypertensive crisis episodes during postoperative intensive care, which was managed by sedation, hyperventilation, oxygen, and acidosis correction and which decreased after using tolazoline. In view of our experience, we recommend that pulmonary artery pressure should be monitored in congenital heart defected patient with preoperative pulmonary hypertension to confirm and to manage the pulmonary hypertensive crisis accurately and using tolazoline is helpful in the treatment of pulmonary hypertensive crisis.

• Journal of Chest Surgery
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• v.25 no.1
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• pp.23-31
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• 1992

The ideal approach in the staged management of patients with pulmonary atresia has been a challenging problem and the result has not been always satisfactory. We reviewed our early result of initial palliative surgeries in fifteen cases of pulmonary atresia with interventricular communication Included are eight cases of simple pulmonary atresia with ventricular septal defect and seven cases of pulmonary atresia associated with other complex cardiac anomalies. The ages of the patients were less than one year except one. The morphology of pulmonary vasculature was highly variable and showed unfavorable conditions in most cases. Pulmonary artery was nonconfluent in two. Two-thirds of all cases showed significant problems such as juxtaductal stenosis or diffuse hypoplasia. The ductus arteriosus usually narrowed at its pulmonic end. Initial palliation was done by modified Blalock-Taussig shunt in six, central shunt with or without pulmonary angioplasty in five, right ventricular outflow tract [RVOT] reconstruction in three and direct connection of nonconfluent pulmonary arteries with bilateral cav-opulmonary shunt in one patient. There were 3 hospital deaths. Two of them underwent simultaneous repair of the associated anomaly of TAPVR. Among the six patients who received modified Blalock-Taussig shunt, three needed early second palliative procedure by central shunt, RVOT patch reconstruction and pulmonary angioplasty in each case, All patients who received central shunt showed marked clinical improvement. Among the twelve patients who survived the palliative procedures, two patients underwent total correction 13 months and 18 months after initial palliation respectively. We think that the choice of palliative procedure must be individualized according to the morphology of the pulmonary arteries. More experience and long term follow-up data are necessary to meet this challenging problem.

• Journal of Chest Surgery
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• v.38 no.5 s.250
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• pp.335-348
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• 2005

Background: This retrospective review examines the preoperative condition, postoperative course, mortality and cause of death for the patients who underwent modified Blalock-Taussig shunt for complex congenital heart defects in early infancy. Material and Method: Fifty eight patients underwent modified Blalock-Taussig shunts from January 2000 to November 2003. The mean age at operation was $23.1\pm16.2$ days ($5\~81\;days$), and the mean body weight was $3.4\pm0.7\;kg\;(2.1\~4.3\;kg)$. Indications for surgery were pulmonary atresia with ventricular septal defect in 12 cases, pulmonary atresia with intact ventricular septum in 17, single ventricle (SV) in 18, and hypoplastic left heart syndrome (HLHS) in 11. Total anomalous pulmonary venous return (TAPVR) was associated with SV in 4 cases. Result: There were 11 ($19.0\%$) early, and 5 ($10.6\%$) late deaths. Causes of early death included low cardiac output in 9, arrhythmia in 1, and multiorgan failure in 1. Late deaths resulted from pneumonia in 2, hypoxia in 1, and sepsis in 1. Risk factors influencing mortality were preoperative pulmonary hypertension, metabolic acidosis, use of cardiopulmonary bypass, HLHS and TAPVR. Twenty four patients ($41.4\%$) had hemodynamic instability during the 48 postoperative-hours. Six patients underwent shunt revision for occlusion, and 1 shunt division for pulmonary overflow. Conclusion: Modified Blalock-Taussig shunt for complex congenital heart defects in early infancy had satisfactory results except in high risk groups. Many patients had early postoperative hemodynamic instability, which means that continuous close observation and management are mandatory in this period. Aggressive management may appear warranted based on understanding of hemodynamic changes for high risk groups.

• Journal of Chest Surgery
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• v.39 no.6 s.263
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• pp.434-439
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• 2006

Background: Deep hypothermic circulatory arrest during repair of aortic arch anomalies may induce neurological complications or myocardial injury. So we surveyed if the regional cerebral and myocardial perfusion might eliminate those potential side effects. Material and Method: From March 2000 to December 2004, 62 neonates or infants with aortic arch anomaly underwent one stage biventricular repair using the regional perfusion technique by single surgeon. Preoperative diagnosis of the arch anomaly consisted of coarctation (n=46), interruption of the aorta (n=12), hypoplastic left heart syndrome (n=2) and truncus areteriosus (n=2). Combined anomalies were ventricular septal defect (n=51), TAPVR (n=1), PAPVR (n=1) and atrioventricular septal defect (n=2). Arterial cannula was inserted at the innominate artery. Result: The mean regional perfusion time of brain was $28{\pm}10min$. Operative mortality rates was 0 (0/62). Late death was 1 (1/62) during $11{\pm}7$ months of follow-up. Neurologic complications consisted of transient chorea in 1 case. There was no reoperation associated with arch anolamy. Pulmonary complication associated with arch repair occurred in f case which was managed by aortopexy. Conclusion: One-tage rch repair using the regional profusion is safe and effective in minimizing the neurologic and myocardial complications.