• Journal of Chest Surgery
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• v.25 no.4
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• pp.406-411
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• 1992

Thirty-four patients were received bidirectional cavopulmonary shunt[BCPS] from Aug. 1989 to Apr. 1991 at Sejong General Hospital, Puchon, Korea. Their ages were from 43 days to 21 years old with 19 cases of infant, 10 from 1 to 5 years old and 5 cases above 6 years old. Their diagnoses were as follows: 13 cases with uni-ventricular heart, 9 tricuspid atresia, 6 double outlet of right ventricle, 4 pulmonary atresia with intact ventricular septum, and 2 transposition of great arteries with pulmonary stenosis. Among them, 10 patients had received other palliative operations before. The BCPS operations were performed under the cardiopulmonary bypass and 10 patients who had bilateral superior vena cava received bilateral BCPS. Other associated procedures were 9 cases of takedown of Blalock-Taussig shunt, 3 pulmonary artery angioplasty, 1 unifocalization, 1 repair of total anomalous pulmonary venous return, 1 Damus procedure, 1 relief of sub-aortic stenosis, 1 right ventricular outflow tract reconstruction and one case of tricuspid valve obliteration. There were 3 operative deaths[8.8%] and two late deaths. The remainders show good postoperative state and their oxygen saturation was increased significantly. Conclusively, the bidirectional cavopulmonary shunt is very effective and safe palliative or pre-Fontan stage operation for the many complex congenital anomalies with low pulmonary blood flow especially for the patients who have the risk of Fontan repair.

• 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.24 no.1
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• pp.1-7
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• 1991

This report describes our 10-year experience with intracardiac repair in 42 patients older than 16 year with tetralogy of Fallot. The mean age was 22.0$\pm$5.18 years[range 16~41]. The preoperative clinical manifestations were cyanosis & clubbing[93%], frequent URI [55%], anoxic spell[40%], pulmonary tuberculosis[21%], tuberculous empyema[7%], chronic renal failure[7%], congestive heart failure[7%] and subacute bacterial endocarditis [2%], etc. The previous shunt procedure for palliation had been performed in 7 patients. The type of VSD were typical perimembranous type[67%], total canal defect[28%] and combined type[5%]. The right ventricular outflow tract obstruction were combined type[69%], infundibular type[21%] and valvular type[10%]. Transannular patch was used in 50% of patients. Associated cardiovascular anomalies were ASD[33%], PFO[31%], Rt. aortic arch[10%], Lt. SVC[10%], single Rt. pulmonary artery, single Lt. coronary artery, ASI, proximal stenosis of Rt. pulmonary artery and anomalous systemic venous return, etc. Hospital mortality was 7.1%[3 cases]in overall. The causes of hospital deaths were revealed low cardiac output & acute renal failure[2 cases], postoperative bleeding[1 case]. There were 2 late deaths 3 & 68 months after surgery. Residual intracardiac shunt was detected in 2 patients. one patient was successfully reoperated and another patient had Qp /Qs ratio less than 1.5.

• Journal of Chest Surgery
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• v.35 no.12
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• pp.862-870
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• 2002

The surgical results of the patients with single ventricle(SV) associated with total anomalous pulmonary venous connection(TAPVC) has been reported with high mortality and morbidity due to their morphologic and hemodynamic complexity. A retrospective review was undertaken to report the outcome of the first-stage palliative surgery in our institution and to determine the factors influencing early death. Material and Method: Between January 1987 and June 2002, 39 patients with SV and TAPVC underwent surgical intervention with or without TAPVC repair. Age at operation ranged from 1day to 10.7months (median age, 2.4month), and 29 patients were male. Preoperative diagnosis included 20 right-dominant SV, 15 SV with endocardial cushion defect, 3 left-dominant SV, and 1 tricuspid atresia. The pulmonary venous connection was supracardiac in 22, cardiac in 5, infracardiac in 11, and mixed in 1, Obstructed TAPVC was present in 11. First-stage palliative surgery was performed in 37. Repair of TAPVC, either alone or in association with other procedures, was performed during the initial operation in 31. Univariate and multivariate analyses were performed to analyze the risk factors influencing the operative death. Result: A mean follow-up period of survivors was 34.3 $\pm$ 43.0(0.53 ~ 146.2)months. Overall early operative mortality was 43.6%(17/39). The causes were low cardiac output in 8, failure of weaning from cardiopulmonary bypass in 3, sepsis in 2, pulmonary hypertensive crisis in 1, pulmonary edema in 1, pneumonia in 1, and postoperative arrhythmia in 1. Risk factors influencing early death in univariate analysis were body weight, surgical intervention in neonate, obstructive TAPVC, preoperative conditions including metabolic acidosis, and need for inotropic support, TAPVC repair in initial operation, operative time, and cardiopulmonary bypass(CPB) time. In multivariable analysis, body weight, age at initial operation, surgical intervention in neonate, preoperative conditions including metabolic acidosis, need for inotropic support and CPB time were the risk factors. Conclusion: In this study, we demonstrated that the patients with SV and TAPVC had high perioperative mortality. Preoperative poor condition, young age, the length of operative and CPB time, the presence of obstructive TAPVC had been proven to be the risk factors. This fact suggests that the avoidance of unnecessarily additional procedures may improve the surgical outcomes of the first-stage palliative surgery. However further observation and collection of the data is mandatory to determine the ideal surgical strategy.

• Journal of Chest Surgery
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• v.14 no.4
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• pp.381-387
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• 1981

Fourteen Infants with congenital cardiac anomalies underwent primary surgical Intervention within the first 12 months of life. There were eight patients with ventricular septal defect, two with total anomalous pulmonary venous return [TAPVR], and the remainders with tetralogy of Fallot, transposition of great arteries [d-TGA], Taussing-Bing malformation, and coronary A-V fistula. The age of the patients ranged from 5 to 12 months, with a mean age of 9.9 months. The mean weight was 6.7 Kg [3.8 to 9.5 KS]. Congestive heart failure persisting despite intensive medical treatment was present In 8 patients [56%], and was the most common indication for operation. Early operation was necessary in 5 of these patients [35%], because of failure to thrive and recurrent pulmonary infection. In one patient with TOF, frequent hypoxic spell prompted the necessity for early operation. In cases of VSD, TAP. VR, TOF, and coronary A-V fistula, Intracardiac repair was done with conventional cardiopulmonary bypass, chemical cold cardioplegia, and topical myocardial cooling. Deep hypothermic circulatory arrest with surface induced cooling, followed by core cooling and core rewarming, was employed .for better exposure in the cases of d-TGA and Taussing-Bing malformation. The results were however, not satisfactory. The overall mortality was 28 per cent. There were no deaths in the eight patients with VSD. The one with coronary A-V fistula survived. The other 5 cases all expired either on the table or immediately after operation. The non-fatal post-operative complications included low cardiac output, respiratory insufficiency, bleeding, and temporary A-V block. The causes of death were prolonged circulatory arrest time in d-TGA, complete A-V block and low cardiac output in TOF and Taussing-Bing malformation and prolonged bypass time and Inadequate correction in TAPVR.

• Journal of Chest Surgery
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• v.14 no.4
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• pp.388-398
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• 1981

Fourteen Infants with congenital cardiac anomalies underwent primary surgical Intervention within the first 12 months of life. There were eight patients with ventricular septal defect, two with total anomalous pulmonary venous return [TAPVR], and the remainders with tetralogy of Fallot, transposition of great arteries [d-TGA], Taussing-Bing malformation, and coronary A-V fistula. The age of the patients ranged from 5 to 12 months, with a mean age of 9.9 months. The mean weight was 6.7 Kg [3.8 to 9.5 KS]. Congestive heart failure persisting despite intensive medical treatment was present In 8 patients [56%], and was the most common indication for operation. Early operation was necessary in 5 of these patients [35%], because of failure to thrive and recurrent pulmonary infection. In one patient with TOF, frequent hypoxic spell prompted the necessity for early operation. In cases of VSD, TAP. VR, TOF, and coronary A-V fistula, Intracardiac repair was done with conventional cardiopulmonary bypass, chemical cold cardioplegia, and topical myocardial cooling. Deep hypothermic circulatory arrest with surface induced cooling, followed by core cooling and core rewarming, was employed .for better exposure in the cases of d-TGA and Taussing-Bing malformation. The results were however, not satisfactory. The overall mortality was 28 per cent. There were no deaths in the eight patients with VSD. The one with coronary A-V fistula survived. The other 5 cases all expired either on the table or immediately after operation. The non-fatal post-operative complications included low cardiac output, respiratory insufficiency, bleeding, and temporary A-V block. The causes of death were prolonged circulatory arrest time in d-TGA, complete A-V block and low cardiac output in TOF and Taussing-Bing malformation and prolonged bypass time and Inadequate correction in TAPVR.

• Journal of Chest Surgery
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• v.39 no.5 s.262
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• pp.347-353
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• 2006

Background: Despite recent advances in surgical technique and perioperative care of total anomalous pulmonary venous return (TAPVR), post-repair pulmonary vein stenosis (PVS) remains as a serious complication. We thought that the most important factors of TAPVR repair to prevent PVS were good exposure, proper alignment, and sufficient stoma size. We analyzed our experience retrospectively. Material and Method: Between Jan. 1995 and Feb. 2005, we studied 74 patients diagnosed with TAPVR suitable for biventricular repair. Supra-cardiac type (n=41, 55.4%) was the most common. Mean CPB time, ACC time, and TCA (40.5%, 30/74) time were $92.1{\pm}25.9\;min,\;39.1{\pm}10.6\;min$, and $30.2{\pm}10.7\;min$, respectively. Mean follow-up duration was $41.4{\pm}29.1$ months and follow-up was possible in all patients. Result: The median age and body weight at operation were 28.5 days ($0{\sim}478$ days) and 3.4 kg $(1.4{\sim}9\;kg)$. Early mortality was 4.1% (3/74). Causes of death were pulmonary hypertensive crisis, sepsis, and sudden death. There was PR-PVS in 2 patients (early: 1, late: 1). Both patients were cardiac type TAPVR drained to coronary sinus. Re-operations were done but only one patient survived. Cumulative survival rate in 5 year and percent freedom from PVS were $94.5{\pm}2.7%\;and\;97.2{\pm}2.0%$, respectively. Conclusion: There was no PVS in patients who underwent extra-cardiac anatomosis between LA and CPVC. Therefore it could be said that our principle might be effective in preventing PR-PVS in patients suitable two-ventricle.

• Journal of Chest Surgery
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• v.32 no.6
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• pp.510-517
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• 1999

Background: Total anomalous pulmonary venous connection (TAPVC) is still one of the more challenging congenital heart defects in newborns and young infants. The purpose of the study is to evaluate the early and midterm results of the surgical corrections for patients in early infancy with isolated TAPVC. Material and Method: Hospital records of 15 consecutive patients in early infancy (January 1993 to August 1998) were retrospectively reviewed. There were 8 boys and 7 girls whose ages ranged from 4 days to 3.5 months (median age 22 days). Their body weight ranged from 1.75 kg to 4.9 kg (mean 3.54 kg). The abnormal anatomical connections were supracardiac in 11, cardiac in 3, and infracardiac in 1. In 6 patients (40%), the pulmonary venous drainage was obstructive. Total circulatory arrest was used in 13 patients. Anastomosis between the common pulmonary vein and the left atrium was performed with a continuous suture technique using a fine nonabsorbable polypropylene suture through a lateral approach behind the right atrium. Result: There was one hospital death (6.5%) caused by a sepsis 17 days after the operation in a neonate who had supracardiac drainage and was dependent on a ventilator preoperatively. There were 2 late deaths. One died sudde`nly of an unknown cause at home 2.5 years after the operation and the other died of a recurrent pulmonary hypertension 3 months after the reoperation due to pulmonary venous obstruction (PVO). Two patients required reoperations because of PVO 5 months and 10 months respectively after the initial operation. Of these patients, one patient is alive at the present time with persistent pulmonary hypertension. All survivors without postoperative PVO (78.6%) were in NYHA functional class I at mean follow-up of 25.8 months (0.5∼67 months). Conclusion: Surgical correction of TAPVC in early infancy can be performed at low risk. However, there were 2 postoperative PVOs (14.3%) which had bad results. The survivors without postoperative PVO had excellent functional status.

• Journal of Chest Surgery
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• v.37 no.6
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• pp.504-510
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• 2004

Background: Vasodilatory shock after cardiac surgery may result from the vasopressin deficiency following cardio-pulmonary bypass and sepsis, which did not respond to usual intravenous inotropes. In contrast to the adult patients, the effectiveness of vasopressin for vasodilatory shock in children has not been known well and so we reviewed our experience of vasopressin therapy in the small babies with a cardiac disease. Material and Method: Between February and August 2003, intravenous vasopressin was administrated in 6 patients for vasodilatory shock despite being supported on intravenous inotropes after cardiac surgery. Median age at operation was 25 days old (ranges; 2∼41 days) and median body weight was 2,870 grams (ranges; 900∼3,530 grams). Preoperative diag-noses were complete transposition of the great arteries in 2 patients, hypoplastic left heart syndrome in 1, Fallot type double-outlet right ventricle in 1, aortic coarctation with severe atrioventricular valve regurgitation in 1, and total anomalous pulmonary venous return in 1. Total repair and palliative repair were undertaken in each 3 patient. Result: Most patients showed vasodilatory shock not responding to the inotropes and required the vasopressin therapy within 24 hours after cardiac surgery and its readministration for septic shock. The dosing range for vasopressin was 0.0002∼0.008 unit/kg/minute with a median total time of its administration of 59 hours (ranges; 26∼140 hours). Systolic blood pressure before, 1 hour, and 6 hours after its administration were 42.7$\pm$7.4 mmHg, 53.7$\pm$11.4 mmHg, and 56.3$\pm$13.4 mmHg, respectively, which shows a significant increase in systolic blood pressure (systolic pressure 1hour and 6 hours after the administration compared to before the administration; p=0.042 in all). Inotropic indexes before, 6 hour, and 12 hours after its administration were 32.3$\pm$7.2, 21.0$\pm$8.4, and 21.2$\pm$8.9, respectively, which reveals a significant decrease in inotropic index (inotropic indexes 6 hour and 12 hours after the administration compared to before the administration; p=0.027 in all). Significant metabolic acidosis and decreased urine output related to systemic hypoperfusion were not found after vasopressin admin- istration. Conclusion: In young children suffering from vasodilatory shock not responding to common inotropes despite normal ventricular contractility, intravenous vasopressin reveals to be an effective vasoconstrictor to increase systolic blood pressure and to mitigate the complications related to higher doses of inotropes.

• 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.