• Journal of Chest Surgery
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• v.21 no.2
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• pp.211-222
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• 1988

Tetralogy of Fallot is a cyanotic congenital heart disease characterized by large ventricular septal defect[VSD] and stenosis of right ventricular outflow tract[RVOT] and the degree of RVOT stenosis and the state of pulmonary arteries are the major determinant of prognosis of this anomaly after operation. The sum of blood flow through RVOT and collateral flow from systemic arteries determine the total pulmonary blood flow and it is drained to left atrium and left ventricle. Therefore the degree of development of left ventricle not only reflects pulmonary blood flow and the status of peripheral pulmonary arteries but also affects postoperative prognosis as a systemic ventricle. In this article, left ventricular volume and its influence on postoperative cardiac function in tetralogy of Fallot were studied in 34 patients operated on at Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital in 1985. Mean age of the patients was 5 1/12*3 9/12 years[range 9/12 - 14 8/12 years], mean body surface area[BSA] 0.65*0.20m2[range 0.38 - 1.22m2], mean body weight 15.6k6.48kg[range 7.0 - 36kg]and mean hematocrit 50.6*9.77%[range 32.0 - 73.5%]. Left ventricular end-diastolic volume[LVEDV] of them were from 11.2 to 113cc and there was a good linear correlation between BSA[m2, X]and LVEDV[cc, Y][Y= - 20.0+923x, r= 0.84, p < 0.005]. Mean LVEDV/m2 was [57.6 * 18.3 cc / m2[range 28.7 - 95.8 cc / m2] and there was a significant reduction of volume compared with normal value. As body surface increases, there was a increasing tendency in LVEDV/m2 but there was no statistical significance. Mean total amount of postoperatively infused dopamine in these 33 patients[except one who expired postoperatively] was 65.6*74.5mg / kg and it was 40.6*44.0mg / kg in routine RVOT widening group [Group I] and 205*49.3mg / kg in transannular RVOT widening group[Group II]. There was a statistically significant difference between two groups. In group I patients there was a good linear inverse correlation between dopamine total amount[mg / kg, Y] and LV volume[cc / m2, X] [Y = 150 - 1.89 X, r = - 0. 77, p < 0.005]. But there were no correlations between dopamine total amount and Hct, cardiopulmonary bypass time and aorta cross clamp time. In conclusion, the patient with small preoperative left ventricular volume required more amount of dopamine as an inotropic agent for the maintenance of a cardiac function in postoperative period. But this is a result of immediate postoperative period and does not reflect the long term effect of left ventricular volume in tetralogy of Fallot. There must be more study for the evaluation of its long term effect.

• Journal of Chest Surgery
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• v.33 no.4
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• pp.290-300
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• 2000

Background: To evaluate the diagnostic accuracy of EBT(Electron Beam Tomography) in the diagnosis of conotruncal anomaly and to determine whether it can be used as a substitute for cardiac angiography. Material and Method: 20 patients(11M & 9F) with TOF(n=7, pulmonary atresia 2), DORV(n=7), complete TGV(n=4), & corrected TGV(n=2) were included. The age ranged from 7 days to 26 years(median 60 days). We analyzed the sequential chamber localization, the main surgical concenrn in each disease category (PA size, LVED volume and coronary artery pattern for TOF & pulmonary atresia, the LV mass, LVOT obstruction, coronary artery pattern for complete TGV, and type of VSD and TV-PV distance for DORV, etc) and other associated anomalies(e.g., VSD, arch anomalies, tracheal stenosis, etc). Those were compared with the results of echocardiography(n=19), angiography (n=9), and surgery(n=11). The interval between EBT and echocardiography/angiography was within 20/11 days, respectively except for an angiography in a patient with corrected TGV (48 days). Result: EBT correctly diagnosed the basic components of conotruncal anomalies in all subjects, compared to echocardiography, angiography or surgery. These included the presence, type and size of VSD(n=20), pulmonic/LV outflow tract stenosis(n=15/2), relation of great arteries and the pattern of the proximal epicardial coronary arteries(16 out of 20). EBT proved to be accurate in quantitation of the intrapericardial and hilar pulmonary arterial dimension and showed high correlation and no difference compared with echocardiography, angiography, or surgery(p>0.05) except for left pulmonary arterial & ascending arterial dimension by echocardiography. LVED volume in seven TOF(no difference: p>0.05 & high correlation: r=0.996 with echocardiography), and LV mass in 4 complete TGV were obtained. Additionally, EBT enabled the cdiagnosis of subjlottic tracheal stenosis and tracheal bronchus in 1 respectively. Some peripheral PA stenosis were not detected by echocardiography, while echocardiography appeared to be slightly more accurate than EBT in detecing ASD or PDA. Conclusion: EBT can be a non-invasive and accurate modality of for the evaluation of most anatomical alteration including peripheral PS or interruption in patients with conotruncal anomalies. Combined with echocardiography, EBT study provides sufficient information for the palliative or total repair of anomalies.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.4
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• pp.703-708
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• 2005

Cleidocranial Dysplasia(CCD) is a congenital disorder of skeletal and dental anomaly with an autosomal dominant mode of inheritance. CCD Shows a generalized defect in intramembranous bones, such as the skull, clavicles, and endochondral bones, such as the long bones and the remainder of the skeleton. The specific clinical feature of CCD is an aplasia & hypoplasia of one or both clavicles, frontal & parietal bone bossing, incomplete fontanels and sutures closure of cranial bone. Generally, relative mandibular prognathism is seen, because maxillar is underdeveloped. Dental anomalies of CCD are prolonged primary teeth, delayed eruption of the permanent teeth and multiple supernumerary teeth. Almost patients of CCD can not recognize their dental abnormality until the permanent teeth eruption was begining. So it is difficult to decide the proper timing of the treatment of patients of CCD. Pedodontists should understand the development of the dentition in CCD patient and start the treatment of CCD patient in proper time.

• Journal of Chest Surgery
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• v.32 no.1
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• pp.5-9
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• 1999

Background: Ebstein anomaly is a rare congenital disease distinguished by its unique deformity in tricuspid valve and right ventricle & atrium. In its surgical treatment , tricuspid valve reconstruction and valve replacement are well known method, but various surgical methods were suggested. Material and Method : From January 1984 to December 1995, 8 patients with Ebstein anomaly underwent surgical correction. Age and sex distribution, clinical symtoms, radiologic findings, preoperative studies, operative findings, operative methods and its results were analyzed. Result: The sex ratio was 5 to 3(male : female). Patients' ages were averaged 17.6(2-28) years. In all cases, it showed typical deformities of the tricuspid valve. Associated anomalies were permenant foramen ovale, atrial septum defect, pulmonary stenosis. Surgical procedures included tricuspid valve replacement(n=4) and tricuspid valve reconstuction(n=4). Two cases of sinus tachycardia and complete AV block occured postoperatively. There were two hospital death and no late death. All survivors are in NYHA class I or II with median follow up of 64.8 months. 2-D echocardiogram disclosed improvement tricuspid regurgitation during the follow up period. Conclusion: Even though operative method of Ebstein anomaly should be decided according to each anatomical characteristics, we recommended that tricuspid valvuloplasty and plication can be one of the good methods method in the selective cases.

• Archives of Plastic Surgery
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• v.34 no.1
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• pp.52-59
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• 2007

Purpose: The venous flap is used as an alternative method to the standard free flap for the management of small and thin soft tissue defects. Especially, the venous flap has advantages of being thin, easy harvest and various donor sites, as well as it having lower morbidity. Yet their clinical applications have been limited by their unstable postoperative course and also by their complications such as partial necrosis. The aim of this study is to extend the clinical indications of venous flaps with using various methods. Methods: From May 2005 to March 2006, total of 19 patients(21 cases) underwent various venous flaps for soft tissue defects as a result of trauma(15 cases), facial skin cancer(3 cases), chronic ulcer(1 case) and surgical wound for congenital anomaly(2 cases). The arterialized venous free flap were applied in 18 cases, the pure venous free flap was applied in 1 case and the pure venous pedicled flap were applied in 2 cases. Among them, two flow-through arterialized venous free flaps were applied that used the great saphenous vein to reconstructed major arteries as well as the injured skin and soft tissues in the arm. All the flap were harvested from the volar wrist(11 cases), dorsum of foot(5 cases), thenar(2 cases), and medial thigh(3 cases). Results: The sizes of the flap ranged from $0.75cm^2$ to $264cm^2$(mean size: $40.06cm^2$). The follow-up period ranged from two to twelve months. In the majority of cases, we obtained satisfying results, which was the excellent reconstruction of skin and soft tissue defects and especially in the case of limb salvage, replantation and cancer reconstruction. However, there were 5 cases of partial necrosis and 2 cases of complete failure. The donor sites were closed primarily in 7 cases and wound closure with skin graft were in 14 cases. Conclusion: We conclude that the venous flap will not only be useful for reconstruction of small defect in the hand and foot, but also be useful for various other clinical indications.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.71-78
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• 2010

Purpose : Hydronephrosis is found about 30% of children with urinary tract infection (UTI). It can be caused by various conditions, although most childhood hydronephrosis is congenital. This study was performed to investigate the relationship between febrile UTI and hydronephrosis. Methods : We retrospectively reviewed the medical charts of 183 patients diagnosed as UTI between January 2007 and May 2009 at Korea University Guro Hospital. Inclusion criteria were as followings; 1) fever more than $37.5^{\circ}C$ measured in the axilla, 2) positive urine culture, 3) no history of urinary tract anomaly on antenatal sonography and urinary tract infection. We classified the enrolled children into two groups of patients with hydronephrosis (HN) and those without hydronephrosis (NHN). Results : The 80 patients were HN and 103 patients NHN. Hydronephrosis was found in 58 patients with left kidney, 8 right and 14 both kidneys. Most of hydronephrosis were of low grade. Compared with NHN group, initial renal cortical defects on DMSA scan significantly increased in HN group (HN 37.5%, NHN 16.5%, P<0.05). The incidence of VUR was not different between the two groups (HN 22%, NHN 12.1%). White blood cell counts and C-reactive protein were not different between the two groups. Follow-up DMSA scan (about 6 months later after UTI) showed no difference of renal scarring in both two groups. Conclusion : Our data suggests that hydronephrosis in febrile UTI patients is clinically useful for detecting renal cortical defects, but is not associated with follow-up renal scar.

• Journal of the korean academy of Pediatric Dentistry
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• v.46 no.2
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• pp.147-157
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• 2019

This study aimed to evaluate skeletodental characteristics of patient with maxillary lateral incisor agenesis (MLIA) in mixed dentition. It involved the children in early mixed dentition who visited Pusan National University dental hospital for orthodontic purposed and had intact primary canines. 38 children with MLIA and 38 controls with the same chronological age satisfying the inclusion criteria were selected. The craniofacial structures and dental arch dimensions of the MLIA were evaluated using model & cephalometric analysis and compared to controls. The rate of unilateral MLIA was high in male and the rate of bilateral MLIA was high in female. In model analysis, the width / length ratio of maxillary anterior portion of the MLIA group were higher and arch perimeter of the maxilla of the MLIA group were smaller than those of the control group (p = 0.003, 0.04). Cephalometric analysis showed that there were no significant differences in terms of skeletal, dental analysis. In soft tissue profile, nasolabial angle was larger in MLIAs than in controls (p = 0.039). Considering these skeletodental characteristics of MLIA, early diagnosis and proper management is highly recommended to minimize the possibility of functional defect.

• Journal of the Korean Society of Radiology
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• v.79 no.5
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• pp.271-275
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• 2018

The falciform ligament is a hepatic suspensory ligament that extends from the umbilicus to the diaphragm, containing the ligamentum teres and a vestigial remnant of the umbilical vein. Among the rarely-occurring pathologies of the falciform ligament, which include ligament cyst, tumor, abnormal vascularization, and congenital ligament defect, a falciform ligament abscess is even more sporadic. Accordingly, the definitive diagnosis of the falciform ligament abscess is rather challenging and may easily be misinterpreted as an infected choledochal cyst or a liver abscess. We present a 25-day-old infant with the falciform ligament abscess, which developed after the umbilical venous catheter insertion and was successfully treated with percutaneous drainage and antibiotic administration.

• Journal of Chest Surgery
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• v.55 no.3
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• pp.206-213
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• 2022

Background: Surgical repair of partial anomalous pulmonary venous return (PAPVR) to the superior vena cava (SVC) using the Warden procedure has favorable outcomes. However, there remain some concerns after the Warden procedure, such as sinoatrial nodal dysfunction and systemic or pulmonary venous stenosis. We investigated the outcomes of the Warden procedure for repair of PAPVR to the SVC. Methods: This retrospective study included 22 consecutive patients who underwent the Warden procedure for PAPVR between 2002 and 2018. The median age and body weight at operation were 27.5 months (interquartile range [IQR], 5.0-56.8 months) and 13.2 kg (IQR, 6.5-16.0 kg), respectively. The median follow-up duration was 6.2 years (IQR, 3.5-11.6 years). Results: There were no cases of early or late mortality. No patients had postoperative heart rhythm problems, except 1 patient who showed transient sinoatrial nodal dysfunction in the immediate postoperative period. Procedure-related complications requiring reintervention occurred in 5 patients, including 3 of 4 SVC stenosis cases and 2 pulmonary venous stenosis cases during follow-up. The rate of freedom from reintervention related to the Warden procedure was 75.9% at 10 years. Conclusion: In cases requiring extension or creation of an atrial septal defect to achieve a sufficient venous pathway, or interposition of an entire circumferential conduit between the SVC and right atrium due to the shortness of the SVC in the Warden procedure, stenotic complications of the venous pathway occurred. Careful observation of changes in the pressure gradient or anatomical stenosis is required in such patients.

• Journal of Chest Surgery
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• v.37 no.9
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• pp.727-734
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• 2004

The Lecompte procedure for transposition of the great arteries has an advantage because it obviates the need for an extracardiac conduit for the reconstruction of the pulmonary outflow tract. We evaluated the effectiveness and the application of the Lecompte procedure. Material and Method: A retrospective review was conducted of the records of 46 patients who underwent the Lecompte procedure during the past 15 years. Mean age at operation was 29.2$\pm$20.3 (range: 3∼83) months. The diagnoses involved anomalies of the ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction, such as transposition of the great arteries, double-outlet right ventricle, and double-outlet left ventricle. Result: Early mortality was 4.4% (2 of 46 patients) and late mortality was 6.8% (3 of 44). The mean follow-up was 11.2$\pm$6.9 years. Eighteen patients (43.9% of survivors, n=41) had pulmonary stenosis (pressure gradient above 30 mmHg), the main reason for which was a calcified monocusp valve (n=15, 83.3%). Seventeen of 46 patients (37.0%) underwent reoperation: 15 for pulmonary stenosis, 5 for residual ventricular septal defect, 4 for left ventricular outflow tract obstruction, 3 for pulmonary insufficiency, and 4 for other causes. The cumulative survival rates were 91.3$\pm$4.2%, and 87.0$\pm$5.8% at 10 and 15 years, respectively. The actuarial probabilities of freedom from reoperation for pulmonary stenosis were 90.6$\pm$4.5%, 73.9$\pm$7.3%, and 54.0$\pm$10.4% at 5, 10, and 15 years, respectively. Conclusion: The Lecompte procedure is an effective treatment modality. Repair in early age is possible with acceptable morbidity and mortality, but recurrent right ventricular outflow tract obstruction caused by degeneration of the monocusp valve is a problem that needs resolution.