• The Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.1
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• pp.36-40
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• 2018

CATCH22 syndrome or DiGeorge syndrome is a medical acronym of cardiac defects, abnormal facial appearances, thymic hypoplasia, cleft palate, and hypocalcemia. Patients with CATCH22 syndrome are susceptible to infection due to an absent or hypoplastic thymus and often have difficulties in maintaining good oral hygiene, which may require dental treatment. We present a case of dental treatment for the uncooperative child with CATCH22 syndrome under general anesthesia. A 4-year-old, 14.8 kg boy with CATCH22 syndrome visited Seoul National University Dental Hospital for dental check up. The patient had multiple caries requiring dental treatment. He experienced the corrective heart surgery due to Tetralogy of Fallot a few years ago. General anesthesia was planned because his heart rate and vital sign had shown unstable during the previous conscious sedation procedure. Dental restorative treatments were successfully performed and no complications were observed during and after the procedure. Safe and effective dental management of the patients with CATCH22 syndrome could be performed with the help of general anesthesia and careful monitoring.

• Clinical and Experimental Pediatrics
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• v.50 no.7
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• pp.665-671
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• 2007

Purpose : The purpose of this study was to investigate the clinical features and outcome in newborns undergoing cardiac surgery. Methods : Eighty two neonates underwent heart surgery for congenital heart defect at Kyungpook National University Hospital between March 2000 and February 2006. Patient characteristics (sex, age, diagnosis), pre-operative conditions, operation type, postoperative complications and mortality were reviewed retrospectively. Results : In 82 patients, 41 (50%) were male. The mean age and weight at operation were 12 days and 3.2 kg, respectively. The common cardiac anomalies were complete transposition of the great arteries (TGA), Tetralogy of Fallot (TOF), pulmonary atresia with intact ventricular septum, and single ventricle variants. Fifty seven operations were performed with cardiopulmonary bypass and corrective surgery was done on 54 patients. Arterial switch operation and modified Blalock-Taussig shunt were most frequently performed as corrective and palliative operations, respectively. The early hospital mortality rate was 7%; the late mortality was 3.9%. Complications were acute renal insufficiency, delayed sternal closure, wound infection, arrhythmia, and brain hemorrhage. Conclusion : During the last 6 years, the outcomes of cardiac surgery for congenital heart defects in neonates improved by progress in perioperative, anaesthetic, surgical, and postoperative care.

• Clinical and Experimental Pediatrics
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• v.51 no.11
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• pp.1236-1240
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• 2008

Smith-Lemli-Opitz syndrome (SLOS) is a rare, autosomal recessive disease caused by an inborn error in cholesterol synthesis. Patients with this disease suffer from multiple malformations due to reduced activity of 7-dehydrocholesterol reductase (DHCR7), which increases 7-dehydrocholesterol (7DHC) and 8-dehydrocholesterol (8DHC) concentrations and decreases cholesterol concentration in body fluids and tissue. The SLOS phenotypic spectrum ranges from a mild disorder with behavioral and learning problems to a lethal disease characterized by multiple malformations. Here, we describe a newborn male with ambiguous genitalia who was diagnosed to have type II SLOS during the neonatal period. A clinical examination revealed low levels of unconjugated estriol in the maternal serum, and a variety of fetal ultrasound anomalies, including prenatal growth retardation. After birth, the infant was diagnosed to have congenital heart disease (Tetralogy of Fallot with severe pulmonary artery stenosis), cleft lip and palate, micrognathia, postaxial polydactyly, ambiguous genitalia, and cataracts. Clinical investigation revealed extremely low plasma cholesterol levels and the presence of mutation (homozygote of p.Arg352Gln) in the DHCR7 gene. The patient underwent palliative heart surgery (to widen the pulmonary artery) and received intravenous lipid supplementation. Cholesterol levels increased slightly, but not to normal values. The patient died from cardiopulmonary failure and sepsis 72 days after birth. This report provides the first description of a Korean patient with SLOS confirmed by verification of DHCR7 gene mutation and illustrates the need for early recognition and appropriate diagnosis of this disease.

• Tuberculosis and Respiratory Diseases
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• v.56 no.4
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• pp.420-425
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• 2004

Fistula between coronary artery and pulmonary artery is a type of coronary artery anomalies. It can cause atypical chest pain and fatigue, angina pectoris, endocarditis, finally myocardial steal can result in heart failure and myocardial infarction. But only 0.1-0.2% of coronary angiographic studies reveal the communications between coronary artery and other spaces. (heart chamber, pulmonary artery etc.) It is frequently congenital, but acquired types are increasing because chest and heart manipulations such as opertion of tetralogy of Fallot, endomyocardial biopsy, radiation therapy, or penetrating blunt trauma are increasing. There are reports about repair of fistula using thrombogenic tips, coil embolization and surgical intervention. We report a connection between coronary artery and pulmonary artery in 79 years old female. She was 30 pack-years smoker and suffered from dyspnea several years with chronic obstructive pulmonary disease. She presented with atypical chest pain and palpitation after admission. Electrocardiography showed ST-T wave abnormality. Emergency coronary angiography and chest CT scan revealed coronary-pulmonary artery fistula. Transcatheter embolization was performed and she was relieved from discomforts.

• Korean Journal of Radiology
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• v.22 no.5
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• pp.706-713
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• 2021

Objective: To evaluate the impact of surgical simulation training using a three-dimensional (3D)-printed model of tetralogy of Fallot (TOF) on surgical skill development. Materials and Methods: A life-size congenital heart disease model was printed using a Stratasys Object500 Connex2 printer from preoperative electrocardiography-gated CT scans of a 6-month-old patient with TOF with complex pulmonary stenosis. Eleven cardiothoracic surgeons independently evaluated the suitability of four 3D-printed models using composite Tango 27, 40, 50, and 60 in terms of palpation, resistance, extensibility, gap, cut-through ability, and reusability of. Among these, Tango 27 was selected as the final model. Six attendees (two junior cardiothoracic surgery residents, two senior residents, and two clinical fellows) independently performed simulation surgeries three times each. Surgical proficiency was evaluated by an experienced cardiothoracic surgeon on a 1-10 scale for each of the 10 surgical procedures. The times required for each surgical procedure were also measured. Results: In the simulation surgeries, six surgeons required a median of 34.4 (range 32.5-43.5) and 21.4 (17.9-192.7) minutes to apply the ventricular septal defect (VSD) and right ventricular outflow tract (RVOT) patches, respectively, on their first simulation surgery. These times had significantly reduced to 17.3 (16.2-29.5) and 13.6 (10.3-30.0) minutes, respectively, in the third simulation surgery (p = 0.03 and p = 0.01, respectively). The decreases in the median patch appliance time among the six surgeons were 16.2 (range 13.6-17.7) and 8.0 (1.8-170.3) minutes for the VSD and RVOT patches, respectively. Summing the scores for the 10 procedures showed that the attendees scored an average of 28.58 ± 7.89 points on the first simulation surgery and improved their average score to 67.33 ± 15.10 on the third simulation surgery (p = 0.008). Conclusion: Inexperienced cardiothoracic surgeons improved their performance in terms of surgical proficiency and operation time during the experience of three simulation surgeries using a 3D-printed TOF model using Tango 27 composite.

• Journal of Chest Surgery
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• v.36 no.7
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• pp.483-488
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• 2003

There are still debates in the literature on the relative benefits of blood cardioplegia and crystalloid cardioplegia in pediatric cardiac surgery. We performed a clinical trial to compare the myocardial protective effect between HTK solution and blood cardioplegic solution in congenital heart surgery. Material and Method: 15 patients who underwent HTK solution cardioplegia (group 1) and 15 patients who underwent blood cardioplegia(group 2) were included in this study. Preoperative and postoperative serial serum cardiac enzyme levels (troponin I, CK-MB, LDH) were measured in all patients. Clinical data were analyzed and compared between the two groups. Result: There were no differences in age and body weight between the two groups. Operative diagnosis included ventricular septal defect (VSD, n＝4), atrial septal defect (ASD, n＝1), tetralogy of Fallot (TOF, n＝4), and other complex heart diseases (n＝6) in group 1, VSD (n＝7), ASD (n＝5), and TOF (n＝3) in group 2. Cardiopulmonary bypass times were 99.1$\pm$48.1 minutes in group 1, and 69.3$\pm$27.3 minutes in group 2 (p＝0,02). Aortic clamping times were 52.1$\pm$23.6 minutes in group 1, and 37.9$\pm$20.5 minutes in group 2 (p＝0.07). There was no mortality and spontaneous defibrillation was possible in all patients. No differences were observed in the serial enzyme levels between the two groups. There were no differences in the duration of inotropic support and ventilator time between the two groups. Conclusion: HTK solution provided comparable myocardial protection compared with blood cardioplegic solution. A single high dose of HTK solution may be safely and conveniently used for an extended periods as well in congenital heart surgery.

• Journal of Chest Surgery
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• v.38 no.4 s.249
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• pp.277-283
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• 2005

Arterial oxygen saturation $(SaO_2)$ instability frequently takes place after systemic-pulmonary shunt without shunt occlusion. We analyzed actual incidence and risk factors for $SaO_2$ instability after shunt operations, and possible mechanisms were speculated on. Material and Method: Ninety three patients, who underwent modified Blalock-Taussig shunt from January 1996 to December 2000, were enrolled in this study. Adequacy of shunt was verified in all patients, either by ensuing one ventricle or biventricular repair later on or by appropriate pulmonary artery growth on postoperative angiogram. Age, body weight, hemoglobin level at operation were 3 day to 36 years (median: 1.8 months), 2.5kg to 51kg (median: 4.1kg) and $10.7\~24.3$ gm/dL (median: 15.2 gm/dL) respectively. Preoperative diagnoses were functional single ventricle with pulmonary stenosis or atresia in 39, tetralogy of Fallot in 38 and pulmonary atresia with intact ventricular septum in 16. Pulmonary blood flow (PBF) was maintained pre-operatively by patent ductus or previous shunt in 64 and by forward flow through stenotic right ventricular outflow tract (RVOT) in 29. $SaO_2$ instability was defined as $SaO_2$ less than $50\%$ for more than 1 hour with neither anatomic obstruction of shunt nor respiratory problem. Result: 10 patients $(10.7\%)$ showed $SaO_2$ instability after shunt operation. After shunt occlusion was ruled out by echocardiogram, they received measures to lower pulmonary vascular resistance (PVR), which worked within a few hours in all patients. Risk factors for $SaO_2$ instability included older age at operation (p=0.039), lower preoperative $SaO_2$ (p=0.0001) and emergency operation (p=0.001). PBF through stenotic RVOT showed marginal statistical significance (p=0.065). Conclusion: $SaO_2$ instability occurs frequently after shunt operation, especially in patients with severe hypoxia pre-operatively or unstable clinical condition necessitating emergency operation. Temporary elevation of pulmonary vascular resistance is a possible mechanism in this specific clinical setting.

• Journal of Chest Surgery
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• v.38 no.3 s.248
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• pp.181-190
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• 2005

The results of biventricular repair for double outlet right ventricle have been improved in recent series. We studied the surgical and long term results for total correction of double outlet right ventricle by the type of ventricular septal defect. Material and Method: Between November 1979 and December 2003, 126 patients had biventricular repair for double outlet right ventricle. The mean age was 1.8 years (range 1$\~$44) and 86 patients ($68.3\%$) were male. We classified and studied this disease by the type of VSD. Result: The locations of VSD were subaortic in 79 ($62.7\%$), subpulmonary in 17 ($13.5\%$), doubly committed in 16 ($12.7\%$) and noncommitted in 14 ($11.1\%$). 28 patients had palliative operation before total correction and the mean interval to total correction was 41.0$\pm$45.1 months. The methods of total correction were intraventricular baffling in 37 ($29.4\%$), intraventricular baffling with patch enlargement of right ventricular outflow tract in 49 ($38.9\%$), intraventricular baffling with Rastelli procedure in 15 ($11.9\%$), arterial switch operation in 8 ($6.3\%$) and REV procedure in 4 ($3.2\%$), etc. Hospital mortality rate was $10.3\%$ (13 patients) and 25 reoperations were performed in 24 patients ($19.0\%$). The risk factors for hospital mortality and reoperation were cardiopulmonary bypass time (p=0.020) and previous palliative operation (p=0.013), respectively. Follow up was possible in 98 patients and mean follow up period was 118.9$\pm$70.7 months. The percent survival and survival for freedom from reoperation at 15 years were $82.5\%$ and $66.7\%$, respectively. The survival rate was significantly lower (p=0.003) in transposition of great artery type and remote type than in simple ventricular septal defect type and tetralogy of Fallot type, but there was no statistical differences in survival rate for freedom from reoperation. Conclusion: It is thought to be that acceptible surgical and long term results can be obtained with application of appropriate methods of repair for double outlet right ventricle.

• Clinical and Experimental Pediatrics
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• v.52 no.12
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• pp.1364-1369
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• 2009

Purpose : To analyze abnormal ventricular activation in childhood congenital heart disease induced by postoperative changes in ventricular volume and pressure and ventricular scar formation using signal-averaged electrocardiography (SAECG). Methods : Fifty-two patients who had undergone open heart surgery (OHS) were enrolled. Patients were divided into the following 3 groups: right ventricular volume overload (atrial septal defect, group1), left ventricular volume overload (ventricular septal defect, group2), and right ventricular pressure overload (tetralogy of Fallot, group 3). The patients were monitored by standard 12-lead ECG and SAECG before and 2 months after the operation. QRS duration, QT and QTc intervals, filtered QRS (f-QRS), high frequency low amplitude potential (HFLA), and root mean square (RMS) voltage in the terminal 40 ms of SAECG were determined. Results : In the preoperative period, group1 showed significant increase in QRS (P=0.011) compared to those of the other 2 groups. In the postoperative period, group3 showed significant increase in the QTc interval (P=0.004) compared to those in the other 2 groups. SAECG parameters showed no significant differences among the groups in the pre- or postoperative period. Of the 52 patients, 12 (23%) in the preoperative period and 21 (40%) in the postoperative period had at least 1 SAECG abnormality. The prevalence of SAECG abnormalities was significantly higher in the postoperative group 2 and group 3 (preoperative: 20% versus postoperative: 28%, P<0.001, preoperative: 14% versus postoperative: 64%, P<0.001, respectively). Conclusion : Abnormal SAECG patterns may be attributed to postoperative scars, OHS itself, and/or ventricular overload.

• Journal of Chest Surgery
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• v.31 no.12
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• pp.1183-1194
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• 1998

Background: From Sept. 1985 to Sept. 1997, 2,000 cases of open heart surgery(OHS) were performed in the Department of Thoracic & Cardiovascular Surgery, Pusan Paik Hospital, College of Medicine, Inje University. Material and Method: Among the total of 2,000 cases of OHS, 1532 cases were congenital heart disease(CHD) and 468 cases were acquired heart disease(AHD). The age distribution was 9 days(4.0kg) to 68 years in CHD and 11 to 66 years in AHD. In 1532 cases of CHD, there were 1403 acyanotic cases and 129 cyanotic cases. Result: The CHD cases consisted of 940 ventricular septal defects(61.4%), 324 atrial septal defects(21.1%), 112 tetralogy of Fallot(7.3%), 46 pulmonary stenosis(3%), 38 endocardial cushion defects(2.5%), 15 valsalva sinus ruptures(1%), 4 transposition of great arteries (0.3%), 4 double outlet right ventricles(0.3%), and etc. Corrective operations were applied for congenital heart disease with a result of 3.1% hospital mortality. Of 468 AHD, 381 cases were valvular heart diseases, 48 ischemic heart diseases, 12 cardiac tumors, 8 annuloaortic ectasias, 16 dissecting aortic aneurysms and etc. In the 381 valvular heart diseases, there were 226 single valve replacements(36 aortic valve replacements(AVR), 188 mitral valve replacements(MVR), and 2 tricuspid valve replacements(TVR), among these were 71 cases of double valve replacements(AVR & MVR), 54 cases of MVR with tricuspid valve annuloplasty(TVA), and 18 cases of AVR, MVR with TVA. The total implanted prosthetic valves were 466. In MVR, 123 St. Jude Medical valves, 90 Carpentier-Edwards valves, 65 CarboMedics valves, 42 Sorin valves and 16 other valves were used. In AVR, 68 St. Jude Medical valves, 36 CarboMedics valves, 14 Carpentier-Edwards valves and 9 other valves were used. Coronary Artery Bypass Surgery(CABG) were performed in 48 cases. The patterns of bypass graft were 14 patients of single vessel graft, 21 patients of two vessels graft, 10 patients of three vessels graft and 3 patients of four vessels graft. Conclusion: The hospital operation mortality rate of congenital acyanotic, cyanotic and acquired heart diseases were 2.0%, 15.5%, and 5.1% respectively. The overall mortality rate was 3.6%(72/2,000).