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A right-sided circumflex retroesophageal aortic arch combined with a ventricular septal defect and severe coarctation is an extremely rare condition. Moreover, surgical correction is significant when performing an anatomical reconstruction of the left aortic arch by end-to-side and native tissue-to-tissue anastomosis instead of using a bypass graft. The authors describe an unusual case of a 1 month-old neonate with a ventricular septal defect and coarctation of a right-sided circumflex retroesophageal aortic arch who underwent successful surgical correction.

The purpose of this study was to compare the outcomes of lateral tunnel (LT) and extracardiac conduit (ECC) Fontan procedures at a single institution. From April 1995 to December 2006, 165 Fontan procedures were performed (67 LT, 98 ECC). Pre-, intra- and postoperative variable values were compared between two different techniques. Operative mortality was 5 (3 LT, 2 ECC). Immediate postoperative transpulmonary gradient (LT 8.5+/-ECC 2.5 vs. 6.6+/-2.4 mmHg) and central venous pressure (LT 18.3+/-3.8 vs. ECC 15.6+/-2.4 mmHg) showed significant difference (P

Objective: This study was conducted to demonstrate that unilateral brain regional perfusion during congenital aortic arch anomaly surgery is neurologically safe. Methods: Fifteen patients who were diagnosed with congenital aortic arch anomaly between June 2004 and May 2006 were enrolled in this study. The mean age and body weight of the enrolled patients were 40.3 35.9 days and 3.7 1.0 kg, respectively. Underlying diseases included coarctation of the aorta (12) and an interrupted aortic arch (3). The pre- and postoperative neurological state of each patient was evaluated by a neurologist using an electroencephalogram and brain SPECT. During the operation, unilateral brain regional perfusion was performed using innominate arterial cannulation. Near-infrared spectroscopy (NIRS) and transcranial Doppler ultrasonographic evaluation of the cerebral artery was used to monitor the cerebral perfusion state during surgery. After being discharged a neurologist conducted regular follow-up evaluations of the patients to monitor their neurological development. Results: There were no operative mortalities. Based on NIRS data, there were no significant differences between left and right oxygen saturation during regional perfusion (left:right = 66.0 10.4%:69.8 1.0%, p = 0.72) or between the pre-regional and regional perfusion period (1. left side pre-regional perfusion:regional perfusion = 66.9 11.8%:66.0 10.4%,p = 0.92,2.rightsidepre-regionalperfusion:regionalperfusion = 70.2 11.1%:69.8 10.0%,p = 0.96).Additionally, there were no differences between pre- and postoperative findings in EEG and brain SPECT. For 17.5 9.0 months of follow-up duration, no patients showed abnormal neurological finding and development. Conclusions: Unilateral brain regional perfusion in neonates and children may be a useful technique with no significant neurological deficit. # 2007 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Objective: Primary repair of aortic arch obstructions and associated cardiac anomalies is a surgical challenge in neonates and infants. Deep hypothermic circulatory arrest prolongs myocardial ischemia and might induce cerebral and myocardial dysfunction. Methods: From March 2000 to December 2005, 69 neonates or infants with aortic arch anomaly underwent one-stage biventricular repair with continuous cerebral perfusion in the presence of a nonworking beating heart using the dual perfusion technique on the innominate artery and aortic root. Preoperative diagnoses of arch anomaly comprised aortic coarctation (n = 54) or an interrupted aortic arch (n = 15). Combined anomalies were ventricular septal defect (n = 52), anomalous origin of the right pulmonary artery from ascending aorta (n = 3), hypoplastic left heart syndrome (n = 2), truncus arteriosus (n = 2), atrioventricular septal defect (n = 2), double outlet right ventricle (n =1 ), total anomalous pulmonary venous return (n = 1), partial anomalous pulmonary venous return (n = 1), and aortic stenosis (n =1 ).Results: The mean regional perfusion time was 27.8 9.8 min. There was no operative mortality. Postoperative low cardiac output was present in four patients (5.8%). A neurologic complication was noted in one patient (1.5%) who developed transient chorea, but recovered completely. During 32.8 17.5 months of follow-up, one late death (1.5%) occurred. There was neither reoperation associated with arch anomaly nor recoarctation except in one patient. One patient developed left main bronchial compression necessitating aortopexy. Conclusions: Onestage total arch repair using our regional perfusion technique is an excellent method that may minimize neurologic and myocardial complications without mortality. Our surgical strategy for arch anomaly has a low rate of residual and recurrent coarctation when performed in neonates and infants. # 2007 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

The Wilms tumor suppressor gene, WT1, plays an important role in the development of the urogenital system and the gonads, and clinical syndromes associated with WT1 mutations, such as WAGR syndrome, Denys-Drash syndrome and Frasier syndrome, typically manifest as renal and genitourinary abnormalities. WT1 may also play an important role in the development of the diaphragm, and recently several papers have reported an association between WT1 mutations and diaphragmatic hernias. In addition, WT1 mutations were also detected in some patients with Meacham syndrome, a rare malformation syndrome comprising congenital diaphragmatic hernia, double vagina, sex reversal, and cardiac malformations. Here, we report a case of an infant with typical clinical features of Deny-Drash syndrome and a heterozygous missense mutation, Arg366His, in the WT1 gene, in whom a diaphragm defect was detected after starting peritoneal dialysis. Diaphragmatic defects are rare but may be considered as clinical manifestations of WT1 mutation syndromes. In addition, we suggest that WT1 abnormalities should be suspected in patients with chronic renal failure who develop hydrothorax after peritoneal dialysis, especially in those with genitourinary abnormalities.

We grow a MnPt3-type trilayer ordered surface alloy on Pt(001), cs2 3 2d Mn-Pt/1ML Pt/cs2 3 2d MnPt/ Pts001d, of which atomic structure is investigated by dynamic low energy electron diffraction analysis. Magneto-optic Kerr effect measurements of the surface alloy at 45 K show no hysteresis, although bulk MnPt3 ordered alloy is ferromagnetic. Ab initio total energy calculations based on density functional theory, rather, predict that ferromagnetic order is not the ground state for the surface alloy. We propose that the nonferromagnetic order of the surface alloy originates mainly from surface effects in regards to nonferromagnetic ground state also found for the near-surface layers of the bulk-terminated MnPt3s001d.

Surgical correction of recurrent coarctation of the aorta presents a formidable technical challenge. If the lesion is diffuse or has long-segment aortic hypoplasia, it can be more difficult to manage. We present a successful case of repair using an extraanatomic bypass graft from the innominate artery to descending aorta. This technique can be an effective and easy approach for selected cases of complex or reoperative aortic obstruction.

Objective: Hemodynamic abnormalities and refractory atrial arrhythmias in patients late after the Fontan operation result in significant morbidity and mortality. We reviewed our experience with Fontan conversion and concomitant arrythmia surgery. Methods: Between January 1996 and February 2004, 16 patients underwent Fontan conversion and arrhythmia surgery. Mean age at the initial Fontan operation was 5.1G 3.5 (range: 2‐15) years and mean age at Fontan conversion was 17.0G5.8 (range: 6‐30). The initial Fontan operations were atriopulmonary connections in 14 patients, extracardiac lateral tunnel in 1, and intracardiac lateral tunnel in 1. The types of arrhythmia included atrial flutter in 10 patients and atrial fibrillation in 3. Fontan conversion operation was performed with intracardiac lateral tunnel in 5 patients and extracardiac conduit in 11. Arrhythmia surgery included isthmus cryoablation in 10 patients and right-sided maze in 3. Results: There has been no mortality. At Fontan conversion operation, 7 patients required permanent pacemaker. All patients have improved to New York Heart Association class I or II. With a mean follow-up of 26.9G30.6 (range:1‐87) months, 16 patients had sinus rhythm, 2 patients had transient atrial flutter which was well controlled, and 2 patients required permanent pacemaker during follow-up. Conclusions: Fontan conversion with concomitant arrhythmia surgery and permanent pacemaker placement is safe, improves New York Heart Association functional class, and has a low incidence of recurrent arrhythmias. In most patients, concomitant permanent pacemakers are needed. q 2004 Elsevier B.V. All rights reserved.

Background Translocation of the coronary artery to the neoaorta is essential in the arterial switch operation. The goal of this study is to investigate (1) the frequency of commissural malalignment in complete transposition of the great arteries, (2) the usefulness of echocardiography in diagnosis of commissural malalignment, and (3) the impact of commissural malalignment on surgery. Methods We retrospectively reviewed the medical records of 28 patients with complete transposition of the great arteries who underwent an arterial switch operation from February 2000 to August 2001. Results Commissural malalignment was expected preoperatively in 11 patients by echocardiography and was confirmed in 13 patients intraoperatively. Four patterns of commissural malalignment were present: (1) sinus-facing of the pulmonary valve, (2) sinus-facing of the aortic valve, (3) sinus-facing of both valves, and (4) bicuspid pulmonary valve (functionally sinus-facing). Two patterns of severity were present: major and minor. To avoid torsion and stretching of the coronary arteries during surgery, various methods were needed: more extensive dissection of the coronary artery, trap door incision, supracommissural or juxtacommissural transfer, both coronary transfer to the same sinus, tube reconstruction of the coronary artery, and neoaorta dextrorotation anastomosis. One patient who had severe commissural malalignment died during the operation, and the cause of death was probably stretching or torsion of the coronary artery. Conclusions The recognition of malalignment of the facing sinus in transposition of the great arteries can be detected preoperatively by echocardiography. The surgical procedure of the arterial switch operation is influenced by the presence of commissural malalignment. Preoperative awareness of commissural malalignment seems to be helpful for surgeons to predict the need for an alternative operational procedure.

Background: Sinus node dysfunction (SND) is reported to be a troublesome complication following various types of Fontan operations. The correlation of post-Fontan SND with surgical methods was evaluated in this study. Methods: By reviewing the medical records, surface ECGs, and Holter monitoring, the range of heart rate (HR) and the risk of SND at intermediate term after Fontan type operation (follow up: 41.3±13.1 months) were analyzed between two age matched groups of patients, consisting of the extracardiac conduit group (EC, n=33) and the lateral tunneling group (LT, n=35). Results: Junctional rhythm was observed in nine out of 35 patients in LT and five out of 33 patients in EC during the follow-up period. Resting HR was faster in EC than that in LT (108±15 vs. 82±21, P

Objectives: Deep hypothermic circulatory arrest during repair of aortic arch anomalies may induce neurological complications or myocardial injury. Regional cerebral and myocardial perfusion may eliminate those potential side effects. Methods: From March 2000 to March 2002, 48 neonates or infants with complex arch anomaly were operated on using the regional perfusion technique. Thirty-three patients were male and the median age was 24 days (range 5-301 days). Preoperative diagnosis consisted of coarctation or interruption of the aorta associated with ventricular septal defect (group I, n = 26) and arch anomaly with complex intracardiac defects such as hypoplastic left heart syndrome or its variants (group II, n = 22). Arterial cannula was inserted through the innominate artery and the flow rate was regulated to about 50-100 ml/kg per min during regional perfusion. Simultaneous myocardial perfusion was maintained using a Y-connected infusion line. Cardioplegia was applied during intracardiac repair. Results: Cardiopulmonary bypass and aortic cross-clamp times were 154 ± 49 and 39 ± 34 min, respectively. Temporary circulatory arrest for intracardiac procedures was performed in eight patients. However, the mean arrest time was minimized (range 1-18 min). The descending aorta clamping time was 33 ± 16 min. Operative mortality rates in each group were 0 and 18.2% (0/26 and 4/22). Late mortality rates were 0 and 11.1% (0/26 and 2/18) during 9.1 months of follow-up. Complications consisted of low cardiac output in eight cases, transient neurological problems in two cases, and transient renal insufficiency in two cases, respectively. Conclusions: Regional perfusion is feasible and can be used with acceptable results. It may reduce potential complications following aortic arch reconstruction using circulatory arrest. However, repair of aortic arch in the patients with complex intracardiac defects still imposes a significant rate of mortality and morbidity.

Purpose: We investigated the quality and usefulness of spiral computed tomography and threedimensional reconstruction in children with obstruction of the central airways as a complication of congenital heart disease. Materials and methods: Spiral computed tomography with three-dimensional reconstruction was performed in 49 children with obstruction of the central airways seen as a complication of congenital heart disease. Spiral scanning was performed during breathing in 40, and after sedation with chloral hydrate in 38. Contrast medium was administered through a pedal venous route in 42. We analyzed the motion artifact, additional information provided by, and clinical usefulness, of the three-dimensional images. We also investigated the factors influencing the quality of the images. Results: Stenoses were seen in the trachea in 21 patients, and in bronchuses in 28. Their causes were an anomaly of the aortic arch in 6, posterior displacement of the aortic arch in 7, posterior displacement of the ascending aorta in 5, compression of the brachiocephalic artery in 5, absent pulmonary valve syndrome in 6, displaced or dilated cardiovascular structure in 17, and pulmonary arterial sling in 2. Motion artifact caused mild or negligible degradation of images in all patients except 6. Breath-holding in non-sedated children produced more severe motion artifact than did cardiovascular pulsation. Threedimensional images provided additional information over two-dimensional images in 11, and provided clinically useful information in 10. Contrast injection via the pedal route was better for the quality of threedimensional images than brachiocephalic injection (p = 0.013). Conclusions: Three-dimensional computed tomography is useful in evaluation of obstruction of the central airways in children with congenital heart disease. Despite the fact that motion artifact is unavoidable, the quality of three-dimensional images is acceptable for making a proper and accurate diagnosis. A pedal route is recommended for injection of contrast medium.

Background. We encountered several patients with posteriorly displaced ascending aorta and bronchial compression associated with congenital heart disease. We describe the helical computed tomography (CT) findings and explore the mechanism of airway compression. Methods. We retrospectively reviewed the clinical data and CT findings of 8 patients with posterior displacement of the ascending aorta. The bronchial stenosis was quantified on reformatted images perpendicular to the main-stem bronchi. On an axial image at the level of main bronchi, we measured depth of retrosternal space, interaortic distance, and aorto-spinal distance. To compare with control, we measured the same variables in 10 control patients. Results. In 7 patients, the main bronchus on the side of the aortic arch was squeezed between the ascending and descending aorta and showed slit-like stenosis. The right pulmonary artery was elongated around the ascending aorta in 5 patients and showed slit-like stenosis in 3. Patients with posterior displacement had significantly larger retrosternal space, smaller interaortic distance, and smaller aorto-spinal distance than did the control group. Aortopexy was undertaken in 3 patients. Follow-up computed tomograms of 2 patients showed improvement. Conclusions. The posteriorly displaced ascending aorta may compress the main bronchus on the side of the aortic arch and right pulmonary artery against the descending aorta or spine. Even if the bronchial compression is mild with tolerable airway symptoms, these patients must be closely observed. When airway symptoms are severe, aortopexy should be considered.

Background and Objectives Milrinone is often used in children to treat acute heart failure and prevent low cardiac output syndrome after cardiac surgery. Due to the lack of studies on the long-term milrinone use in children, the objective of this study was to assess the safety and efficacy of the current patterns of milrinone use for ≥3 days in infants and children with heart diseases. Subjects and Methods We retrospectively reviewed the medical records of patients aged

A 5-month-old male patient presented with right-dominant unbalanced atrioventricular septal defect and left-sided parachute valve, and underwent successful biventricular repair. Because of the presence of a small left ventricle, left atrium, and a single left papillary muscle, an additional orifice was created in the left-sided atrioventricular valve with artificial partitioning of the right-sided atrioventricular valve. There was no evidence of mitral stenosis or regurgitation on follow-up echocardiography.

The objective of this study was to investigate the usefulness of helical CT angiography in the evaluation of total anomalous pulmonary venous connections.Fourteen patients with total anomalous pulmonary venous connections underwent helical CT angiography and subsequent three-dimensional (3D) reconstruction. They ranged in age from 3 days to 8 months (median age, 2.3 months) and in weight from 2.3 to 7.1 kg (median weight, 4.3 kg). The types of total anomalous pulmonary venous connections and the number of pulmonary veins were evaluated on axial and 3D images. Qualitative evaluations were performed for extent of pulmonary vascular enhancement and contrast- or motion-induced artifacts.In all patients, helical CT angiography correctly depicted total anomalous pulmonary venous connections. Seven cases were the supracardiac type, four cases were the cardiac type, one case was the infracardiac type, and two cases were the mixed type. The detection rate of the pulmonary vein in 3D reconstruction images (95-98%) was slightly lower than that of the pulmonary vein in the axial images (100%), but the difference between axial and 3D reconstruction images was not statistically significant (p0.1). No statistically significant differences were noted among 3D reconstruction images in the detection rates of the pulmonary vein (p0.1). The extent of contrast enhancement of the pulmonary vein was good or excellent in all patients. In five patients, there were contrast-induced artifacts that made some surrounding vascular distortion but did not interfere with the pulmonary vein analysis, except in one patient. Motion-induced artifacts were observed in nine patients. One of them had an obstacle in pulmonary vein analysis.The combination of axial and 3D images in helical CT angiography is helpful in the assessment of a total anomalous pulmonary venous connection containing the individual pulmonary vein, and this combination can be a good diagnostic tool in preoperative evaluation of neonates and infants with a total anomalous pulmonary venous connection.

A 9-year-old boy with a permanent epicardial screw-type ventricular pacemaker lead (VVI mode), fitted due to a complete atrioventricular block after ventricular septal defect closure at 5 months of age, showed ventricular tachycardia during maximal exercise testing. The pacemaker lead was found to have fractured at 30 mm from the tip, and the screw tip penetrated the right ventricular cavity. Pace mapping at the anterior free wall of the right ventricle detected a 12/12 matched ventricular tachycardia focus around the site of penetration. The fractured lead was extracted under cardiopulmonary bypass support and the lead insertion site cryoablated. Subsequently, ventricular tachycardia was not induced during maximal exercise testing.

A 5-month-old boy was admitted to hospital presenting with cardiogenic shock. The echocardiographic examinations revealed a severe ventricular dysfunction. Despite the aggressive medical therapy, his hemodynamic status progressively deteriorated. Mechanical circulatory support was initially provided with extracorporeal membrane oxygenation (ECMO). Because his hemodynamic condition had not improved, the ECMO was removed and a biventricular assist device (BVAD) was instituted. After 10 days, the ventricular function had recovered and the BVAD could be removed. This report describes a successful bridge to recovery using a BVAD in an infant suffering from acute myocarditis.

Woong-Han Kim*, Jeong-Wook Seo, Soo-Jin Kim, Jinyoung Song, Jaeyoung Lee, Chan-Young Na Department of Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital, College of Medicine, Seoul National University, Seoul National University Medical Research Center, Seoul, South Korea Department of Pathology, Seoul National University Children’s Hospital, College of Medicine, Seoul National University, Seoul National University Medical Research Center, Seoul, South Korea Department of Pediatric Cardiology, Sejong General Hospital, Sejong Heart Institute, Pucheon-Shi, Kyonggi-Do, South Korea Department of Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute, Pucheon-Shi, Kyonggi-Do, South Korea

537 PREDICTION OF LIVER-RELATED MORTALITY AND RISK STRATIFICATION IN PATIENTS HOSPITALIZED FOR ALCOHOLIC HEPATITIS W. Kim, M. Lee, Y. Choi, S. Kim, D. Kim, S.J. Yu, H.Y. Kim, Y.J. Jung. Seoul Metropolitan Government Seoul National University Boramae Medical Center, Seoul National College of Medicine, Medical Research Collaborating Center, Seoul National University Hospital, Seoul National University Hospital Healthcare System Gangnam Center, Department of Internal Medicine and Liver Research Institute, Seoul National College of Medicine, Department of Internal Medicine, Seoul Metropolitan Government Seoul National University Boramae Medical Center, Seoul, Republic of Korea E-mail: wonshiri@yahoo.com

The conventional fuzzy set model uses the MIN and MAX operators for the evaluation of boolean operators AND and OR, respectively. It has been criticized to generate incorrect ranked output in many cases. This is because the MIN and MAX operators do not well correspond with human behavior for document ranking. There are a variety of fuzzy operators to replace the MIN and MAX operators. In this paper we first investigate the behavioral aspects of the fuzzy operators and address important issues to affect retrieval effectiveness. We then present the enhanced fuzzy set model based on positively compensatory operators, which are suitable for achieving high retrieval effectiveness. It is also shown through experiments that the enhanced fuzzy set model provides similar retrieval effectiveness and higher retrieval efficiency in comparison with the extended boolean model.

Regardless of the preoperative morphology and the type of operation, left ventricular outflow tract obstruction (LVOTO) after biventricular repair of double outlet right ventricle (DORV) may develop. This report presents our 10-yr experience with surgical management of LVOTO after biventricular repair of DORV. Between 1996 and 2006, 15 patients underwent reoperation for subaortic stenosis after biventricular repair of DORV. The mean age at biventricular repair was 23.3+/-18.3 months (1.1-64.2). Biventricular repairs included tunnel constructions from the left ventricle to the aorta in 14 cases and an arterial switch operation in one. The mean left ventricle-to-aorta peak pressure gradient was 54.0+/-37.7 mmHg (15-140) after a mean follow-up of 9.5+/-6.3 yr. We performed extended septoplasty in nine patients and fibromuscular resection in six. There were no early or late mortality. There was one heart block and one aortic valve injury after an extended septoplasty, and two and one after a fibromuscular resection. No patient required reoperation for recurrent subaortic stenosis. The mean pressure gradient was 11.2+/-11.4 mmHg (0-34) after a mean follow-up of 5.6+/-2.7 yr. Extended septoplasty is a safe and effective method for the treatment of subaortic stenosis, especially in cases with a long-tunnel shaped LVOTO.

Background and Objectives: The ability to study microvessels of a beating heart in real time at the level of the capillary is essential for research. However, there are no proven methods currently available to achieve this. The conventional absorption-contrast agents have limitations for studying capillaries. Microangiography with using synchrotron phase-contrast X-ray technology and no contrast agent has recently been reported on. We tried to verify this previous report, and we wanted to visualize the microvessels of a rat heart using air as a contrast agent. Materials and Methods: We made the Langendorff apparatus in a hutch of the Pohang Accelerator Laboratory. The images were obtained with a white beam and a monochromatic beam. The visual images were magnified using 3× and 20× optical microscope lenses, and the images were captured with a charge-coupled device camera. Results: We could not duplicate the previously reported findings in which microvessels were visualized without the use of contrast agent. But with using air as a contrast agent, the microvasculature of rat hearts was clearly identified at a spatial resolution of 1.2 μm. Air being absorbed inside a capillary was also observed. Vessels under 10 μm diameter were unable to be visualized with using iodine as a contrast agent. Conclusion: Phase contrast imaging already allows spatial resolution of 1 μm, which is enough to inspect capillaries. We were able to obtain images of cardiac capillaries with using air as a contrast agent. Yet air has the fatal limitations in that it causes embolism and ischemia. A more suitable contrast agent or imaging method needs to be developed in order to study the microvessels of a beating heart. (Korean Circ J 2008;38:462-467)

Background and Objectives: This study aimed to evaluate the usefulness of cardiopulmonary exercise test (CPET) in children with various congenital heart diseases (CHDs). Subjects and Methods: Forty-eight children and adolescents (18 girls and 30 boys; mean±SD age, 12.6±0.5 years) with CHD who had undergone corrective surgery performed CPET using a programmable treadmill. The participants were divided into 4 groups based on the surgery performed: Fontan operation (12 patients), total correction for tetralogy of Fallot (13 patients), repair for coarctation of aorta (11 patients), and corrective surgery for simple CHDs (12 patients). Patients with simple CHDs who had undergone an early surgery were selected as controls. Results: Progressive exercise time was the longest in the simple CHD group than in the other groups (10.6±2 versus 8.2-8.7 min, p=0.122). Absolute and predicted values of mean peak oxygen consumption were the lowest in the Fontan operation group (p=0.220 and 0.091, respectively). The coarctation of aorta group exhibited a prominent blood pressure response, that is, an elevation in the systolic blood pressure relative to that in the other groups at rest (131.7±18.8 versus 114.4121.3 mmHg) and at peak exercise (196.5±41.5 versus 146.8-184.3 mmHg). Conclusion: CPET can be a useful tool to clarify the exercise capacity and reflect the hemodynamic status of patients who have undergone surgery for various CHDs and other heart diseases. (Korean Circulation J 2007;37:489-496)

Background and Objectives:Based on our previous studies on pulmonary atresia with ventricular septal defect (VSD) and major aorto-pulmonary collateral arteries (MAPCAs), a management strategy for this disease was formulated at our hospital in 1999. To evaluate this strategy, it was applied to the new patients and their outcomes were measured. Subjects and Methods:The subjects were a cohort of newly diagnosed patients who were without any prior interventions and these patients were treated at our hospital from January 2000 to December 2003. The management strategy focused on promoting the growth of the pulmonary arterial confluence (PAC), if present, by performing a right ventricle-pulmonary artery conduit operation (RV-PA conduit). If the PAC was large or absent, then the management plan was discussed among the surgeons after performing an exhaustive work-up. Results:Seventeen patients were enrolled and their age at the time of diagnosis ranged from 8 days to 34 months with a median age of 3 months. The initial surgical method varied from one-stage total correction (4 cases) to a conduit operation with or without additional MAPCAs procedures (13 cases). All but three of the patients survived their initial surgery. There were two late deaths. In addition to the 3 survivors from one-stage total correction, 4 patients underwent a staged total corrective operation. The 5 remaining patients are still a subtotal correction state or a palliated state. Conclusion:When a large PAC is present, one-stage total correction is the best surgical option. However, when the PAC is small, then RV-PA conduit without any vascular procedures may be a good alternative. This staged approach makes a larger PAC, which enables surgeons to create a much easier vascular anastomosis later on. (Korean Circulation J 2007;37:348-352)

Purpose : Late hypertension (HT) after coarctoplasty occurs commonly. This study was performed to evaluate the relationships between late HT and associated factors to it. Methods : Retrospective analysis of the medical records of 22 apparently healthy patients who had coarctoplasty during infancy and were followed up for more than 10 years was done. Casual blood pressure (BP) was obtained in all and 24 hour BP monitoring was obtained in 16. Echocardiogrphic Doppler flow velocity at coarctation of aorta (COA) repair site and left ventricular (LV) indexes were also obtained. The ratio of the narrowest diameter of aortic arch and the diameter of the descending aorta at the diaphragmatic level was obtained through 3-dimentional computed tomography. Results : HT by casual BP was detected in 7 of 22 and HT by 24 hour BP monitoring in 10 of 16. Three were normotensive by casual BP, but hypertensive by 24 hour BP monitoring. Males predominated in patients with HT by casual BP (P=0.022) and by 24 hour BP monitoring (P=0.036). Doppler velocity at COA repair site was higher in HT group by casual BP (P=0.006) and also in HT group by 24 hour BP monitoring (P=0.041). Especially, patients with a velocity of over 3 m/sec were more in HT group by casual BP (P=0.026). Conclusion : Even if casual BP is normal, there can be hypertension by 24 hour BP monitoring. So every postoperative patient has to get 24 hour BP monitoring. Especially in patients with Doppler velocity at COA site over 3 m/sec, 24 hour BP monitoring should be done to define HT.