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OBJECTIVES We compared the clinical outcomes between tricuspid valve detachment (TVD) and non-TVD for ventricular septal defect (VSD) closure in infants METHODS From January 2004 to April 2020, 462 infants RESULTS The median age and body weight at operation were 1.9 months [interquartile range(IQR), 1.4–2.5] and 4.2 kg (IQR, 3.7–4.6). The median follow-up duration was 83.4 months (IQR, 43.5–130.4). After matching, 44 pairs were extracted from each group. There were no significant differences in all-cause mortality (P = 0.176), reoperation (P = 0.172), postoperative morbidities, including residual VSD, aortic regurgitation, atrioventricular block and significant tricuspid regurgitation (TR) (P = 0.346) between group D and group N. However, group D showed significantly less TR progression during follow-up (P = 0.019). CONCLUSIONS In infants

Though ventricular assist devices (VADs) are an important treatment option for acute heart failure, an extracorporeal membrane oxygenator (ECMO) is usually used in pediatric patients for several reasons. However, a temporary centrifugal pump-based Bi-VAD might have clinical advantages versus ECMO or implantable VADs. From January 2000 to July 2018, we retrospectively reviewed 36 pediatric patients who required mechanical circulatory support (MCS) for acute heart failure. Cases with postoperative MCS were excluded. Since 2016, we have tried to immediately add a right VAD rather than ECMO, when the patients begin to present features of right heart failure after left VAD support started in cases that the patients’ respiratory function did not require an oxygenator. Original diagnoses included dilated cardiomyopathy (n = 18), myocarditis (n = 11), and others (n = 7). Eleven patients were supported by Bi-VAD, and 25 patients were supported by ECMO; of these. Four patients were successfully weaned from VAD, and 10 patients were weaned from ECMO. Eleven patients underwent heart transplantation. Overall, we have 15 (41.7%) early mortalities. There were no significant differences in early mortality, morbidity, and weaning rate between the Bi-VAD group and the ECMO group. During the support, patients with Bi-VADs significantly required fewer platelets and showed less hemolysis than ECMO patients. Patients with myocarditis were successfully weaned from Bi-VAD support and bridged to transplantation thereafter. A temporary centrifugal pump-based Bi-VAD was clinically comparable to ECMO for pediatric patients with acceptable pulmonary function.

Background As a result of advances in pediatric care and diagnostic testing, there is a growing population of adults with congenital heart disease (ACHD). The purpose of this study was to better define the epidemiology and changes in the trend of hospitalizations for ACHD in Korean society. Methods We reviewed outpatient and inpatient data from 2005 to 2017 to identify patient ≥18 years of age admitted for acute care with a congenital heart disease (CHD) diagnosis in the pediatric cardiology division. We tried to analyze changes of hospitalization trend for ACHD. Results The ratio of outpatients with ACHD increased 286.5%, from 11.1% (1748/15,682) in 2005 to 31.8% (7795/24,532) in 2017. The number of ACHD hospitalizations increased 360.7%, from 8.9% (37/414) in 2005 to 32.1% (226/705) in 2017. The average patient age increased from 24.3 years in 2005 to 27.4 in 2017. The main diagnosis for admission of ACHD is heart failure, arrhythmia and Fontan-related complications. The annual ICU admission percentage was around 5% and mean length of intensive care unit (ICU) stay was 8.4 ± 14.6 days. Mean personal hospital charges by admission of ACHD increased to around two times from 2005 to 2017. (from $2578.1 to $3697.0). Total annual hospital charges by ACHD markedly increased ten times (from $95,389.7 to $831,834.2). Conclusions The number of hospital cares for ACHD dramatically increased more than five times from 2005 to 2017. We need preparations for efficient healthcare for adults with CHD such as a multi-dimensional approach, effective communication, and professional training.

OBJECTIVES We compared the surgical outcomes of infective endocarditis (IE) between early surgery and non-early surgery groups in children. METHODS From January 2000 to April 2020, we retrospectively reviewed 50 patients RESULTS The median age and body weight at operation were 7.7 years [interquartile range (IQR), 2.3–13.2] and 23.7 kg (IQR, 10.3–40.7), respectively. The median follow-up duration was 9.5 years (IQR, 4.0–14.5). In 28 patients with native valve endocarditis, the native valve was preserved in 23 (82.1%). The most common causative microorganism was Streptococcus viridans (32.0%). The operative mortality was 2.0%, and 13 (26.0%) patients required reoperation most commonly for prosthesis failure (n = 7). There were no significant differences in patient characteristics and perioperative data between early surgery (n = 9) and non-early surgery (n = 36) groups, except for the interval between diagnosis and surgery (early surgery < non-early surgery, P CONCLUSIONS Surgical outcomes for IE in children were acceptable irrespective of the time of surgery. Our results suggest that it may not be required to delay surgery for IE and the potential benefit of early surgery could be expected in children.

Background The occurrence of coronary artery disease (CAD) is unknown to date in patients who have undergone the right ventricular outflow tract (RVOT) reconstruction surgery for congenital heart disease using a bioprosthetic valve or conduit. Purpose We present the incidence and outcomes of CAD in patients who underwent RVOT reconstruction surgery in a single tertiary center. Method From April 1986 to February 2019, 544 patients underwent Rastelli operation or pulmonary valve replacement for conotruncal anomalies at the Seoul National University Children's Hospital in Korea. The patient's medical records and imaging findings were analyzed retrospectively. Results Significant coronary stenosis or occlusion were observed in 19 patients (3.5%) after RVOT reconstruction surgery. Their underlying diseases were pulmonary atresia in 8, tetralogy of Fallot in 3, double outlet right ventricle with pulmonary stenosis in 3, truncus arteriosus in 2, congenitally corrective transposition of great artery in 2, and congenital aortic stenosis in 1. Mechanism of CAD was compression of coronary artery by artificial conduit or valve in 10, intraoperative coronary injury in 7, and intramural course of coronary artery in 2 patients. The median number of RVOT reconstruction surgery before the diagnosis of CAD was 2 times (interquartile range: 2–3 times). The median age at diagnosis of CAD was 15 years (interquartile range: 9.5 - 25 years). The median time to detection of the CAD from the last RVOT reconstruction surgery was 9 years (interquartile range: 3.5–12.5 years). Involved coronary artery was right coronary artery (RCA) in 10, left anterior descending coronary artery (LAD) in 7, and left main coronary artery in 2 patients. Seven (36.8%) patients had underlying congenital coronary anomalies, such as single coronary artery, intramural course of the coronary artery, and anomalous origin of the coronary artery from an abnormal sinus. One patient died due to brain injury on extracorporeal membrane oxygenator after intraoperative coronary injury. Two patients with intramural course of coronary artery and one patient with high take-off of RCA which was compressed by adjacent calcified conduit underwent surgical repair of the CAD. One patient with recurrent ventricular tachycardia needed an implantable cardioverter-defibrillator. Nine patients are on medication for heart failure. Three patients with total occlusion of RCA or LAD showed well-developed collaterals from another normal coronary artery. Conclusion CAD after RVOT reconstruction surgery are not common but can be fatal. Because the clinical significance of CAD and long-term prognosis are not known yet, we need to closely track the patient after RVOT reconstruction surgery. Indications for the management of the CAD should be prepared to prevent sudden cardiac death, arrhythmia, and ventricular dysfunction in these patients. Funding Acknowledgement Type of funding source: None

OBJECTIVES Clinically, tetralogy of Fallot (TOF) patients who underwent repair late (older than 2 years) appears to have worse outcomes after pulmonary valve replacement than patients who underwent repair early. We proceeded to review the clinical features of late-repaired TOF patients who required pulmonary valve replacement. METHODS Fifty patients who underwent pulmonary valve replacement after TOF repair over the age of 2 years from 2000 to 2018 were retrospectively reviewed. Pre- and postoperative cardiac magnetic resonance imaging, cardiopulmonary exercise tests, and cardiac catheterization were analysed. RESULTS The median age of patients at the time of TOF repair and pulmonary valve replacement was 3.6 and 23.4 years, respectively. The median interval from TOF repair to pulmonary valve replacement was 20.5 years. Cardiac magnetic resonance imaging and cardiopulmonary exercise tests were performed at a median of 5.9 and 3.7 years after pulmonary valve replacement, respectively. Cardiac magnetic resonance revealed that there were significant changes in the indexed values of the right ventricle end-diastolic volume (164.7–106.9 ml/m2, P CONCLUSIONS Although pulmonary valve replacement after late TOF correction improves right ventricular volume status, only a minority of patients achieve normalization of right ventricular end systolic volume and a normal functional status.

BACKGROUND AND OBJECTIVES We reviewed the long-term outcomes after tetralogy of Fallot (TOF) repair with trans-annular incision; and evaluated the effectiveness of pulmonary valve replacement (PVR) on outcomes. METHODS This was a retrospective review of clinical outcomes of 180 of 196 TOF patients who underwent total correction with trans-annular incision from 1991 to 1997 (PVR group: 81; non-PVR group: 99). RESULTS The median age of the patients was 14.0 months (interquartile range [IQR], 10.7-19.8 months) at TOF repair. Ten in-hospital deaths (5.1%) occurred. During the follow-up, 81 patients underwent PVR at the median age of 13.5 years (IQR, 11.2-17.1 years). The patients in PVR group showed better outcomes than non-PVR group in overall survival rate (100% in PVR vs. 88.7% in non-PVR, p=0.007), in all adverse events (arrhythmia, neurologic complications, 95.5% in PVR vs. 74.6% in non-PVR, p=0.024) at 20 years. Age at TOF repair younger than 1 year (hazard ratio [HR], 2.265; p=0.01) and previous shunt history (HR, 2.195; p=0.008) were predictive for requiring PVR. During follow-up, 10 late deaths (5 sudden deaths) occurred in the non-PVR group, mainly due to ventricular arrhythmia and right ventricular failure; there was 1 late death (not a sudden death) in the PVR group. CONCLUSIONS Long-term survival after repair of TOF with trans-annular incision were acceptable. However, arrhythmias were frequently observed during 20 years of follow-up. The patient age

Additional file 1: Supplement table. Each diagnosis of adult patients with congenital heart disease was categorized as simple, complex, or unclassified based on the 32nd Bethesda Conference report [3].

Pulmonary vein stenosis (PVS) is still a frustrating disease with extremely high mortality, especially in children with multiple severe PVS. Hybrid pulmonary vein stenting (HPVS) is a rescue treatment for recurrent and malignant PVS. The aim of this study is to share our successful experience with intraoperative HPVS for recurrent PVS after total anomalous pulmonary venous connection (TAPVC) repair in infant. Six patients were identified between 2013 and January 2018, who were diagnosed with recurrent PVS and underwent HPVS in the operating room. The mean age at the time of the HPVS was 10.3 ± 2.7 months (range 7-14 months) and the mean body weight was 7.9 ± 2.6 kg (range 4.1-10.5 kg). Prior pulmonary vein surgery had been performed on average 2.7 times (range 2-3) in all patients. We used a bare-metal stent (BMS) of 6-8 mm diameter in 15 veins of five patients and a drug-eluting coronary stent (DES) in two veins of one patient. All patients had undergone several elective further pulmonary vein in-stent balloon dilatations or another stent insertion after HPVS. Over a mean follow-up of 17.3 ± 13.7 months (range 6-44 months), all patients maintained patency of stents although two patients died due to respiratory failure not associated with PVS. HPVS is a useful treatment modality for recurrent PVS patient that could save the life and achieve longer freedom from restenosis than repetitive surgical pulmonary vein widening only. Even though the prognosis of severe multiple PVS is very poor, planned HPVS could be a good palliation in this patients group.

Despite improvements in valve repair techniques, conditions in which infants and children need for mechanical valve replacement (MVR) are still present. We analyzed supra-annular MVR outcomes in infants and children with small annulus and compared them with conventional annular MVR outcomes. Data were collected retrospectively from medical records of infants and children (weighing

OBJECTIVES Mitral valve (MV) repairs have been performed in paediatric patients with congenital MV stenosis. However, congenital MV stenosis lesions are a heterogeneous group of lesions, and their repair remains challenging. METHODS From March 1999 to September 2014, MV repair was performed in 22 patients with congenital MV stenosis. The median age was 10.3 months (ranging from 22 days to 9.1 years), and the mean body weight was 7.9 ± 4.0 kg at the time of the operation. Multiple-level left-side heart obstructions were present in 9 (45%) patients. RESULTS The main aetiology of the mitral stenosis was a supravalvular mitral ring in 8 patients, valvular stenosis in 4 patients, a parachute deformity of the papillary muscles in 4 patients and other abnormal papillary muscles in 6 patients. The mean MV pressure gradient improved from 10.4 ± 3.9 mmHg to 3.4 ± 1.7 mmHg after MV repair (n = 18, P

Background Patients with congenital complete atrioventricular block often require lifelong pacemaker therapy, which has a risk of left ventricular (LV) dysfunction. The aim of our study was to determine the long-term effects of epicardial pacing on LV function and dyssynchrony in children with congenital heart block. Methods We conducted a retrospective study at a single tertiary center in which 34 pediatric patients with isolated congenital complete heart block who underwent epicardial pacemaker implantation from 1987 to 2016 were enrolled. Results Median age during pacemaker insertion was 2.5 years (range, 0-16.7 years) and the median follow-up duration was 12.3 years. The initial pacing sites were the right ventricle (RV) free wall for 10 patients, RV apex for 16, and LV apex for eight. LV dysfunction developed in 7 patients, of whom 4 underwent lead relocation to the RV apex (n = 2) and cardiac resynchronization therapy upgrade (n = 2). RV free-wall pacing had a significantly higher risk of LV dysfunction than either LV or RV apical pacing (odds ratio 52.5; P = .003; 95% confidence interval 3.9-700). All 4 patients who underwent lead repositioning showed improvement of LV function (median ejection fraction, 24%-62.7%). Conclusions Our study suggests that RV free-wall pacing may be a significant risk factor for LV dysfunction. Pacemaker-induced LV dysfunction was improved via lead relocation to the RV apex or cardiac resynchronization therapy upgrade. Future studies with a larger sample size and longer-term follow-up are required to confirm our results.

We reviewed our surgical experience with anomalous origin of one pulmonary artery from the ascending aorta (AOPA). From 1989 to 2012, 12 children (five neonates) aged 3-734 days (mean 152 ± 222) with AOPA underwent operations. Eight patients had right AOPA, and four patients had left AOPA. The majority of the patients had elevated right ventricular pressure, with 58 % (7 of 12) demonstrating suprasystemic right ventricular pressure. Surgery was performed by direct anastomosis (group 1) in seven patients and by employing an autologous patch (group 2) in five patients. There were two postoperative mortalities caused by heart failure and pulmonary hypertensive crisis. The mean follow-up duration was 12.6 ± 8 years. Catheterization showed that the right ventricle-to-systemic pressure ratio decreased following operation (preoperative vs. postoperative; 1.13 ± 0.19 vs. 0.48 ± 0.03, p = 0.043). There was no difference in the perfusion of the affected lung as measured by the final lung perfusion scan, between the two groups (group 1 vs. group 2; 50.0 ± 10.3 vs. 42.7 ± 28.7 %, p = 0.158). Two patients required reoperations for pulmonary regurgitation and pulmonary artery stenosis. There were two catheter-based interventions. At 20 years, survival by the Kaplan-Meier was 91.7 ± 8.0 %, freedom from reoperation was 80.0 ± 17.9 %, and freedom from catheter intervention was 80.8 ± 12.2 %. Early repair of AOPA improves right ventricular pressure and overall hemodynamics with excellent survival and low risk of reintervention. The type of surgical repair did not significantly affect the long-term outcomes (measured via lung perfusion scan).

Background : Atrial tachyarrhythmia is a major late complication in adult Fontan patients. This study examined the clinical features and risk factors of late intra-atrial reentrant tachyarrhythmia (IART) in adult patients after Fontan surgery and the mid-term outcome of Fontan conversion with or without antiarrhythmic surgery in these patients. Methods : We conducted a retrospective study on adult patients who were born before 1994 and survived at least 3months after a Fontan operation at Seoul National University Children's Hospital. Results : We followed 160 patients over 20.9±4.1years. Sustained atrial tachycardia was identified in 51 patients, and IART was found in 41, appearing a mean 13.6years after surgery. By the 25year follow-up, 40% had developed IART. The incidence of IART significantly increased over time. Patients with an atriopulmonary connection (APC) (n=65) had significantly longer follow-up duration and higher incidence of IART than patients with a lateral tunnel (n=86) or extracardiac conduit Fontan (n=9). On multivariate analysis, APC, sinus node dysfunction, and nonsustained atrial tachycardia were found to be significantly associated with IART. Twenty-four patients with IART underwent Fontan conversion. Over the follow-up period, IART severity scores in the 22 patients who survived after Fontan conversion decreased significantly, and New York Heart Association functional class significantly improved. On multivariate analysis, protein losing enteropathy and ventricular dysfunction were found to be significant risk factors for mortality. Conclusions : IART was common in adult Fontan patients, and Fontan conversion with or without antiarrhythmic surgery and pacemaker placement helped to control it.

The occurrence of ‘algal bloom’, caused by the mass proliferation of phytoplankton, causes serious problems in streams and lakes in Korea. Therefore, in this study, the phytoplankton filter-feeding trait of Unio douglasiae, a type of freshwater clam, was used to reduce the algal bloom in outdoor water tanks during the summer. This involved the construction of a U. douglasiae cultivation apparatus, wherein 1,000 clams were divided into 8 rectangular baskets arranged in the shape of an empty square. The control tank was manufactured in exactly the same shape within the water tank, but without the addition of clams. The algal bloom-reducing effect of U. douglasiae was confirmed by the measurement of (and comparing between) the water quality at the center and periphery of the test and control cultivation apparatus. Water quality measurements included the measurement of water temperature, pH, turbidity, dissolved oxygen (DO) content, and chlorophyll-α concentrations; the water quality was measured twice a month between June and November 2014.The results of these analyses did not show a significant difference in water quality (temperature, pH, turbidity, DO) between the center and periphery of the test and control tanks. However, the chlorophyll-α concentration was observed to be much lower at the center of the test tank compared to that at the center and periphery of the control tank, as well as at the periphery of the test tank. This was believed to be a result of the U. douglasiae surrounding the center of the test tank, which prevented the influx of plankton from the periphery. Accordingly, the results of these analyses suggest the possibility that U. douglasiae cultivation could reduce the proliferation of algal blooms in lakes and streams during the summer. In particular, these results indicate possible improvements in U. douglasiae activity (reduction in algal blooms) by their effective arrangement in the water bodies.

OBJECTIVES: Acute kidney injury (AKI) is common after paediatric cardiac surgery and associated with increased morbidity and mortality. Aortic arch surgery may be an independent risk factor for AKI because of circulatory arrest below the descending thoracic artery during anastomosis. We investigated the surgical outcomes associated with AKI after aortic arch repair in paediatric patients. METHODS: We retrospectively analysed 120 paediatric patients who underwent aortic arch repair between 2003 and 2012. AKI was defined according to the paediatric-modified risk, injury, failure, loss and end-stage kidney disease criteria. The incidence, clinical outcomes and risk factors for AKI were analysed. RESULTS: Aortic arch repair was performed for coarctation of aorta in 97 patients and interrupted aortic arch in 23 patients. The median age and body weight at the time of surgery were 16.5 days and 3.3 kg, respectively. The mean duration of the follow-up was 3.9 years. AKI developed in 42 patients (36.8%) and peritoneal dialysis (PD) was applied in 20 patients (16.7%). The recovery of renal function began a mean of 3.8 days after conservative management, and full recovery occurred a mean of 6.7 days after conservative management. A lower body weight (

The study aim was to evaluate the immune reaction, difference of degenerative calcification, and anti-calcification effect of decellularization with or without α-galactosidase in bovine pericardium and porcine heart valves, using an α1,3-galactosyltransferase (α-Gal) knockout (KO) mouse model.In order to elucidate the anti-calcification effect of decellularization with or without α-galactosidase, bovine pericardium and porcine heart valve tissues were assigned to four groups according to the tissue preparation method: (i) glutaraldehyde (GA) fixation only; (ii) decellularization + GA fixation (Decell); (iii) α-galactosidase + GA fixation (α-galactosidase); and (iv) decellularization +α-galactosidase + GA fixation (Decell + α-galactosidase). Each prepared tissue was implanted subcutaneously into α-Gal KO mice. Anti-α-Gal immunoglobulin (Ig) G and IgM antibody titers were monitored prior to implantation and at four, eight and 12 weeks after implantation using an enzyme-linked immunosorbent assay. Calcium contents of explanted tissues were measured at 12 weeks after implantation.There were no significant differences in the anti-α-Gal IgG antibody titers according to the type of bioprosthetic material or tissue preparation method (p0.05). The calcium content was significantly lower in porcine heart valves than in bovine pericardium when implanted in α-Gal-KO mice (p0.001). Calcium contents in bovine pericardium and porcine heart valves were significantly lower in the Decell, α-galactosidase and Decell + α-galactosidase groups than in the GA group (all p0.05).The porcine heart valve induced lower levels of calcium deposition than did the bovine pericardium, but the anti-α-Gal IgG antibody titers did not differ significantly between the bioprosthetic tissues. Decellularization had significant anticalcification effects in both the bovine pericardium and porcine heart valves, though there was no significant difference in the anti-α-Gal IgG antibody titers among tissue preparation methods.

Objectives We aimed to investigate the effect of a combination of anticalcification treatments, which were effective for preventing calcification in a small animal experiment, on glutaraldehyde-fixed xenopericardial valves using a large-animal long-term circulatory model. Methods Valved conduits were made of porcine pericardium as a leaflet and bovine pericardium as a conduit and were implanted into the right ventricular outflow tract of goats under cardiopulmonary bypass. The goats were divided into study (glutaraldehyde + combined anticalcification treatment, n = 6) and control (glutaraldehyde alone, n = 9) groups. Upon euthanization at 1 year, echocardiography and cardiac catheterization were performed. Explanted tissues were microscopically examined and analysed for measuring the calcium content. Results Haemodynamic data were obtained from 3 and 2 goats in the study and control groups, respectively. All valves, except 1, which was limited in motion, were functioning well on echocardiography; pressure gradients across the right ventricular outflow tract were lower in the study group on cardiac catheterization. On gross inspection, all leaflets remained mobile without calcific deposits in the study group, while most leaflets were heavily calcified in the control group. The calcium content in the leaflets remained low (≤4 µg/mg) in the study group. Among the leaflets explanted from goats that survived longer (>3 months), the calcium concentration was higher in the control group than in the study group [15.1 µg/mg (n = 5) vs 2.7 µg/mg (n = 5), respectively; P = 0.008). Conclusions Porcine pericardial leaflets treated with our anticalcification protocol showed better function and less calcification than those treated with glutaraldehyde alone in the pulmonary position.

Background Many Down syndrome (DS) patients have an atrial septal defect (ASD) and associated pulmonary hypertension (PH) from early childhood. ASD closure in DS patients with PH is often controversial due to concerns regarding exacerbation of PH. The aim of this study was to investigate the clinical outcome following surgical ASD closure in children with DS. Methods We retrospectively reviewed the medical records of DS patients who underwent surgical ASD patch closure from January 2000 to December 2016. Results A total of 15 patients underwent surgery for ASD. Prior to ASD patch closure, nine patients were diagnosed with PH, three of whom took medications for PH. The mean age of patients at ASD patch closure was 17.3 months, and the mean diameter of the ASD was 10.2 mm. Three patients who took medications for severe PH underwent ASD patch closure at ages 7, 12, and 25 months. Two patients continued medication for an additional 13 and 21 months, and one patient remained on medication 52 months after ASD closure. PH did not recur following discontinuation of selective pulmonary vasodilators in two patients. Although a moderate degree of PH remained in one patient due to a chronic lung problem, it was improved compared to before ASD closure. No PH was observed in the remaining 12 patients following ASD closure. Conclusions A large ASD can be closed even in DS patients with severe PH during early childhood with the support of multiple selective pulmonary vasodilators.

Reducing the cardiopulmonary bypass (CPB) priming volume in congenital cardiac surgery is important because it is associated with fewer transfusions. This retrospective study was designed to compare safety and transfusion volumes between the mini-volume priming (MP) and conventional priming (CP) methods. Between 2007 and 2012, congenital heart surgery using CPB was performed on 480 infants (≤5 kg): the MP method was used in 331 infants (MP group, 69.0%), and the CP method was used in 149 infants (CP group, 31.0%). In the MP group, narrow-caliber (3/16″) tubing was used, and the pump heads were vertically aligned to shorten the tubing lengths. The smallest possible oxygenators and hemofilters were used, and vacuum drainage was applied. Ultrafiltration was vigorously applied during CPB to avoid excessive hemodilution. The mean age and body weight of the patients were 48 ± 41 (0-306) days and 3.8 ± 0.8 (1.3-5.0) kg, respectively. The total priming and transfusion volumes during CPB were lower in the MP group than in the CP group (141 ± 24 mL vs. 292 ± 50 mL, P

Extrinsic airway compression often complicates the course of congenital heart disease (CHD) repair. This study investigated the risk factors and outcome of airway compression evaluated using computed tomography (CT) in CHD patients.Of the 2,729 patients who underwent heart surgery for CHD between 1999 and 2007, airway compression was confirmed using CT in 58 (2.1%) patients. The patients were divided into groups according to the underlying CHD, and their medical records and CT scans were reviewed retrospectively.Airway compression was found more frequently in the vascular ring or absent pulmonary valve syndrome (8 of 11) and repaired aortic arch (22 of 213) groups than in the other groups (28 of 2,505) (p0.001). Patients with more severe respiratory manifestations showed greater airway compression on CT (p0.001) and had a higher rate of additional surgery to relieve airway compression using multivariate analysis (p = 0.005). Airway compression was ameliorated in 13 of 17 patients after surgery for airway compression. Funnel chest deformity worsened after aortic arch repair and was associated with the need for surgical relief of airway compression. Pulmonary overflow disease could be followed up without additional surgery for airway compression.Early airway compression detection and management may reduce further morbidity, especially after aortic arch repair. The patient's respiratory manifestation and the underlying disease characteristics must be considered when determining the need for additional surgery for airway compression.

We investigated the mid-term surgical results of Fontan conversion with antiarrhythmias surgery and permanent pacemaker implantation, which were complications of a previous Fontan operation.From January 1996 to November 2011, we performed Fontan conversion in 31 of 260 Fontan cases (M:F = 17:14, mean age = 19.0 years). The Fontan conversion from atriopulmonary connection (APC) to extracardiac conduit (ECC) was performed in 20 patients, APC to lateral tunnel in 5 and lateral tunnel to ECC in 6. The clinical outcomes and improvements of arrhythmias were analysed. The types of arrhythmias included atrial flutter in 21 patients, atrial fibrillation with flutter in 4 patients and junctional tachycardia in 3 patients.Twenty-six patients (83.9%) required antiarrhythmia surgery (isthmus cryoablation = 9, right-sided maze = 13, bilateral maze = 4). In addition, 23 patients (74.2%) received a permanent pacemaker. The New York Heart Association functional class (NYHA Fc) was statistically improved after the surgery during the 6.5-year median follow-up duration (preoperative NYHA Fc = 1.77, postoperative NYHA Fc = 1.13, n = 15, P = 0.001). There were 4 late mortalities. Actuarial 5-year survival was 90.0 ± 5.5%. And freedom from arrhythmia was 91.8 ± 5.5%, at 5 years. Normal sinus rhythm was maintained in 12 patients (38.7%), pacing-dependent rhythm in 10 patients (32.3%) and intermittent pacing-dependent rhythm in 4 patients (12.9%).Fontan conversion with antiarrhythmia surgery and permanent pacemaker implantation is safe and improves the clinical outcome and arrhythmias.

Although significant atrioventricular valve regurgitation (AVVR) is well known for its association with increased morbidity and mortality in patients with single-ventricle physiology, there is a lack of consensus in management of AVVR. The purpose of this study was to analyze the clinical outcomes in patients receiving AVV repair or replacement. From 2001 to 2010, a total of 33 patients (25 male and 8 female) with more than moderate-degree AVVR among 160 patients who underwent staged single-ventricle palliation were included. The median follow-up duration was 6.0 years (range 0.1-14.1). Valve repair (n = 27) or valve replacement (n = 6) was performed at the initial surgery. There were six late mortalities (18.18 %): five in the repair group and one in the replacement group and seven morbidities. Among patients with valve repair, 11 were required to undergo redo-valve operations (valve repair n = 6, valve replacement n = 5) due to deteriorated valve function. Initial shunt procedure (p = 0.04) and arrhythmia (p = 0.01) were risk factors for survival. Freedom from reoperation in the valve replacement group was higher than that in the valve repair group (67.0 ± 9.7 and 44.6 ± 11.2 % at 5 and 6 years, respectively, p = 0.03). Need for early repair (p = 0.02), presence of mitral- or tricuspid-dominant AVV (p = 0.005), and male sex (p = 0.04) were risk factors for valve durability. Early valve regurgitation affects valve durability. Thus, successful repair in the early stage may improve later outcomes. Therefore, aggressive valve surgery was required and AVV replacement might be one of the options for selected patients.

Background and Objectives We hypothesized that fetal echocardiography (echoCG) is an accurate diagnostic tool reflecting well postnatal echoCG findings and outcomes. Subjects and Methods We reviewed the medical records of 290 pregnant women, including 313 fetuses, who were examined by fetal echoCG at the Seoul National University Children's Hospital from January 2008 through April 2011. Results The mean gestational age at diagnosis was 26.2±5.2 weeks. The mean age of mothers at diagnosis was 31.7±3.8 years. We identified indications for fetal echoCG in 279 cases. The most common indication was abnormal cardiac findings in obstetrical screening sonography (52.0%). Among the 313 echoCG results, 127 (40.6%) were normal, 13 (4.2%) were minor abnormalities, 35 (11.2%) were simple cardiac anomalies, 50 (16.0%) were moderate cardiac anomalies, 60 (19.2%) were complex cardiac anomalies, 16 (5.1%) were arrhythmias, and 12 (3.8%) were twin-to-twin transfusion syndrome. The most common congenital heart disease was tetralogy of Fallot (23 fetuses, 15.9%). One hundred forty-eight neonates were examined by echoCG. We analyzed differences between fetal echoCG and postnatal echoCG. In 131 (88.5%) cases, there was no difference; in 15 (10.1%), there were minor differences; and in only 2 (1.4%) cases, there were major differences. Conclusion There is a recent increase in abnormal cardiac findings of obstetric ultrasonography screenings that indicate fetal echoCG. Fetal echoCG is still a good, accurate diagnostic method for congenital heart disease.

Background Refractory atrial arrhythmias and hemodynamic abnormalities are responsible for significant morbidity and mortality after the Fontan operation. We evaluated the long-term feasibility, safety, and efficacy of prophylactic atrial arrhythmia surgery performed concomitantly with the lateral tunnel Fontan operation. Methods From 1997 August to 2003 December, 27 patients underwent a initial lateral tunnel Fontan with an interventional atrial incision and cryoablation from the atriotomy to the coronary sinus and right atrioventricular valve annulus. This novel surgical technique consists of (1) right atriotomy extending to the coronary sinus to block the slow rate conduction isthmus; (2) cryoablation between right atriotomy and right atrioventricular valve annulus; (3) baffling to avoid injury to the crista terminalis; and (4) use of a sandwich technique with closure of right atriotomy incorporating the Gore-Tex (W. L. Gore & Associates, Flagstaff, AZ) patch to reduce atrial suture line. Results There has been no early death after operation and one late death, which was not arrhythmic in etiology. At late follow-up of 15.2 ± 2.9 years (range, 5.5 to 18.0) after Fontan, spontaneous intraatrial reentrant tachycardia occurred in 1 patient, and inducible intraatrial reentrant tachycardia in 1 patient who required beta-blocker medication without ablation attempts. There was no evidence of early or late complications related to the interventional atrial incision and cryoablation. Four patients required late pacemaker implantation for sinus node dysfunction after Fontan operation. Conclusions The prophylactic arrhythmia surgery with our novel modification of the lateral tunnel Fontan procedure to reduce the development of intraatrial reentrant tachycardia was feasible and safe. Long-term follow-up results also demonstrated that this novel modification is effective for the prophylaxis of intraatrial reentrant tachycardia.

BACKGROUND AND OBJECTIVES Mechanical circulatory support with extracorporeal membrane oxygenation (ECMO) and ventricular assist device has always been the optimal choice for treating the majority of medically intractable low cardiac output case. We retrospectively investigated our institution's outcomes and variables associated with a high risk of mortality. SUBJECTS AND METHODS From 1999 to 2014, 86 patients who were of pediatric age or had grown-up congenital heart disease underwent mechanical circulatory support for medically intractable low cardiac output in our pediatric intensive care unit. Of these, 9 grown-up congenital heart disease patients were over 18 years of age, and the median age of the subject group was 5.82 years (range: 1 day to 41.6 years). A review of all demographic, clinical, and surgical data and survival analysis were performed. RESULTS A total of 45 (52.3%) patients were successfully weaned from the mechanical assist device, and 25 (29.1%) survivors were able to be discharged. There was no significant difference in results between patients over 18 years and under 18 years of age. Risk factors for mortality were younger age (

OBJECTIVES: The arterial switch operation (ASO) has become the standard surgical procedure for transposition of the great arteries (TGA) or variants with an excellent early outcome. However, there are concerns regarding neopulmonary stenosis, neoaortic regurgitation (neoAR) associated with neoaortic root dilatation and coronary artery disease. METHODS: A total of 220 early survivors of the ASO were included in this retrospective study between November 1987 and June 2011. The median age and weight at operation were 13 days (0–1768 days) and 3.52 kg (1.69–19 kg), respectively. The indications for the ASO included TGA with intact ventricular septum in 113 patients, TGA with ventricular septal defect in 90 and Taussig–Bing anomaly in 17 patients. The median follow-up period was 103.2 months (0.4–277.4 months). Statistical analyses with the Kaplan–Meier and Cox proportional hazards models were performed. RESULTS: The actuarial late survival rate and freedom from reoperation at 23 years were 96.6 ± 1.3 and 81.9 ± 3.8%, respectively. Twenty-four (10.9%) patients underwent reoperations for right ventricular outflow tract obstruction in 10 patients, neoAR in four and coronary artery stenosis in three, etc. Freedom from neoAR of Grades IV, III and II at 23 years was 90.2 ± 6.6, 70.9 ± 9.6 and 20.3 ± 5.5%, respectively. The risk factors for neoAR were size discrepancy of the great arteries, aortic root dilatation after the ASO and follow-up duration after the ASO. NeoAR was significantly correlated with the size of aortic sinus and aortic sinotubular junction over time. Freedom from pulmonary stenosis (PS) of ≥36 and ≥20 mmHg at 23 years was 34.8 ± 18.0 and 17.7 ± 9.6%, respectively. The risk factors for PS were Taussig–Bing and arch anomalies. Coronary artery evaluation was performed in 95 (43.2%) patients with angiography, computed tomography or single-photon emission computed tomography, and five (5.3%) patients had abnormal coronary morphology or perfusion. Three patients underwent reoperation for coronary artery stenosis, and two had reversible perfusion defects in various regions, which were clinically not significant. Freedom from coronary events was 88.1 ± 6.4% at 22 years. A risk factor for coronary events was the single coronary artery. CONCLUSIONS: The survival and functional outcomes of the ASO were excellent in the long-term. Strict serial surveillance is required to evaluate the long-term functional outcome of the ASO, particularly in a high-risk anatomy.

Pulmonary valve replacement (PVR) is performed to reduce right ventricular (RV) volume overload, resulting in improved ventricular function and clinical status. Significant pulmonary regurgitation (PR) after tetralogy of Fallot (TOF) repair could result in RV dysfunction, exercise intolerance, arrhythmia and sudden death. The present study was conducted to investigate the mid-term clinical outcomes of PVR after TOF repair.Between 2001 and 2010, we retrospectively reviewed the outcomes of 131 (89 males and 42 females) PVRs with PR or pulmonary steno-insufficiency after TOF repair. PVR was performed at a mean age of 14.8±6.7 years. The mean interval from total correction of TOF to PVR was 12.5±5.2 years. Surgical indications of PVR were more than moderate PR with/without pulmonary stenosis, right ventricle dilatation, right ventricle dysfunction and reduced exercise capacity. Hancock II (n=58), Carpentier-Edwards Perimount (n=49) and St Jude Biocor (n=35) bioprosthetic valves were used. The mean z-score at implantation was 1.1±0.8. The mean valve size implanted was 25.1±1.5 mm.There was no early or late mortality in this study. RV end-diastolic and end-systolic volume indices (from 111.3±34.7 to 64.6±23.6, P0.01) (preoperative n=70, postoperative n=17) were markedly decreased PVR during the 13.2±16.1 months follow-up period. Eleven patients (male=10, female=1) required a repeat PVR operation due to prosthetic valve failure. The rate of freedom from reoperation at 10 years was 66.4±4.4%. Implanted valve type (Carpentier-Edwards bovine valve), young age, and large-sized valve implantation (z-score2.0) were risk factors for a repeat PVR in the univariate analysis. There was no risk factor in the multivariable analysis.PVR reduced the RV volume and improved the RV function within the first postoperative year. The rate of freedom from reoperation during the 10-year follow-up period in our series was acceptable. However, a longer follow-up will be necessary to determine the long-term outcomes of bioprosthetic valves in PVR.

Background and Objectives: Vascular rings refer to anomalies of the great arteries that cause respiratory or feeding problems. The purpose of this study was to analyze a series of patients with vascular rings and evaluate associated risk factors for mortality. Subjects and Methods: A retrospective review of all patients identified with vascular rings between 1997 and 2010 in the Seoul Nation al University Children’s Hospital. Results: Thirty-five patients were diagnosed with vascular rings (median age at diagnosis, 7 months). The vascular rings of 32 patients were confirmed by cardiac computed tomography or magnetic resonance imaging. The types of vascular rings were: a double aortic arch in ten patients, a right aortic arch with persistent left ligamentum arteriosum in seven, an aberrant subclavian artery in seven, a pulmonary sling in eight, and others types in three patients. Eleven patients were asymptomatic. Gastrointestinal and respiratory symptoms were seen in ten and sixteen patients, respectively. Associated cardiovascular anomalies were present in fifteen patients. Twenty patients with definite symptoms underwent surgical treatment. The median interval between diagnosis and operation was 6 days. Four patients eventu ally died; three deaths were associated with complex heart diseases, and one had pulmonary artery sling with severe tracheal stenosis. Only the presence of a complex heart disease significantly influenced mortality (p=0.002). Conclusion: Vascular rings include several types of anomalies, each with a different prognosis and symptoms. The presence of a complex heart disease was significantly associated with mortality. Early diagnosis and timely surgery in symptomatic patients are essential. (Korean Circ J 2012;42:252-258)

The univentricular heart after the Fontan operation may have a blind pouch formed by the pulmonary stump or rudimentary ventricle according to the anatomy before surgery. Thrombosis in an intracardiac blind pouch of patients with a univentricular heart is a hazardous complication. Because only a few reports have described this complication, the authors evaluated the clinical significance of thrombosis in an intracardiac blind pouch of a univentricular heart. They performed a retrospective review of medical records from August 1986 to December 2007. Four patients were confirmed as having thrombosis in a pulmonary artery stump and one patient as having thrombosis in a rudimentary ventricle shown by cardiac computed tomography (CT). This represents 1.85% (5/271) of patients with ongoing regular follow-up evaluation after the Fontan operation. The median age at diagnosis was 14.2 years. Two of the five patients were taking aspirin and one patient was taking warfarin when they were identified for the development of thrombosis. None of the patients demonstrated thrombosis in the Fontan tract or venous side of the circulation. Brain magnetic resonance imaging (MRI) showed that three patients had cerebral infarction and one patient had suggestive old ischemia. Three patients with thrombus in the pulmonary stump underwent pulmonary artery stump thrombectomy and pulmonary valve obliteration. One patient with thrombus in the rudimentary ventricle underwent ventricular septal defect (VSD) closure with thrombectomy. Thrombus in a blind pouch could cause systemic thromboembolism despite little blood communication. Therefore, surgical modification of the pulmonary stump and VSD closure of the rudimentary ventricle are required to reduce the risk of later thrombus formation. Clinicians should not overlook the possibility of thrombus in a ligated pulmonary artery stump or a rudimentary ventricle after the Fontan operation, which may increase the risk of embolic stroke for patients with single-ventricle physiology.

Background. Despite potential benefits of histidinetryptophan-ketoglutarate (HTK) solution as a cardioplegic solution, it can cause hyponatremia, especially in pediatric patients. Fluctuations in the sodium concentration during cardiopulmonary bypass (CPB) can adversely affect the central nervous system. We evaluated the relationship between the cardioplegic solution, the fluctuation of sodium concentration, and the incidence of postoperative seizure in pediatric cardiac patients. Methods. The medical records of 628 patients were reviewed for the occurrence of a postoperative seizure, type of cardioplegic solution (HTK or del Nido solution), and intraoperative data. A change of sodium concentration exceeding 15 mmol/L (Na>15) during CPB was defined as a significant fluctuation of sodium concentration. Results. Postoperative seizures were detected in 18 patients (2.9%). The Na>15 was detected in 63 of 189 patients (33.3%) who received the HTK solution and in 14 of 439 patients (3.2%) who received the del Nido solution (p 15 was strongly associated with postoperative seizure (odds ratio, 6.3; 95% confidence interval, 2.4 to 16.4, p 0.001). After adjusting for potential confounders, the Na>15 remained significantly associated with postoperative seizure (odds ratio, 3.9; 95% confidence interval, 1.3 to 12.3, p 0.018). Conclusions. Histidine-tryptophan-ketoglutarate solution during CPB frequently causes fluctuations of sodium concentration, usually combined with hyponatremia, which is associated with postoperative seizure. Special attention to sodium concentration is required, particularly when HTK solution is used in pediatric cardiac patients.

Objective We evaluated the efficacy of the early rehabilitation of remnant pulmonary artery in unilateral absent intrapericardial pulmonary artery and the factors affecting pulmonary artery growth. Methods We retrospectively reviewed the medical records and imaging modalities of 15 patients with unilateral absent intrapericardial pulmonary artery (7 left and 8 right; median age at diagnosis, 5 months) from 1991 to 2008. Results The remnant pulmonary artery was found in 12 patients (mean diameter, 2.6 ± 0.7 mm) at the hilum. Eleven patients underwent operation (main pulmonary artery flap angioplasty in 5 patients; tube graft interposition in 6 patients), and 4 patients were inoperable. Transcatheter balloon angioplasty or stent implantation was required for the remaining pulmonary artery stenosis in 6 patients (55%). The last ipsilateral lung perfusion proportion at lung perfusion scan was 39% (range, 15%–51%), and the Z value of the last ipsilateral pulmonary artery diameter was −0.5 (range, −4.2 to 2). The patients with a smaller initial remnant pulmonary artery required more interventions (P = .003). The final perfusion proportion of affected lung was higher in the patients treated early (≤6 months, n = 7) than in those treated late (>6 months, n = 4) (41.9% ± 8.5% vs 24.9% ± 10.7%, respectively, P = .024). The patients with graft interposition showed a lower perfusion proportion of affected lung than those with main pulmonary artery flap angioplasty (P = .017). Conclusions In patients with unilateral absent intrapericardial pulmonary artery, early and aggressive management of combined surgical reconstruction and transcatheter intervention improved pulmonary artery growth and lung perfusion.

Background and aim of the study: Debate continues over the optimal timing for total repair of tetralogy of Fallot. Here, we report our experience with early one-stage total repair of tetralogy of Fallot. Methods: Between January 2000 and March 2008, surgical treatment was required in 24 symptomatic patients who were diagnosed with tetralogy of Fallot at less than three months of age. Among them, total repair was performed in 13 patients (group 1) and initial palliation was done in the others (group 2). The median follow-up duration was 20 months (5 to 40 months) and 67 months (1 to 100 months) in each group. Results: There was one operative death in group 2. The duration of mechanical ventilatory support, and the lengths of intensive care unit stay and hospital stay were not statistically different between both groups. The ventricular septal defects were repaired through the right atrium in 12 patients (12/13, 92.3%) of group 1 and nine patients (9/11, 81.8%) of group 2 (p = 0.576). Transannular repair was performed in six patients (6/13, 46.2%) of group 1 and 10 patients (10/11, 90.9%) of group 2 (p = 0.036). Reintervention or reoperation was required in two patients of group 1 and three of group 2 (p = 0.630). During follow-up, the pulmonary arterial growth was more prominent in group 1. Conclusions: Early one-stage total repair of tetralogy of Fallot can be performed safely without increasing the risk for reoperation or reintervention, in the short term. The pulmonary annulus is likely to be preserved when it is repaired at an early age. Early total repair may be better for the pulmonary arterial growth than staged repair. (J Card Surg 2010;25:394-399)

Background As the number of cases with artificial pulmonary valve implantation increases for congenital heart disease, the number of young adults with artificial pulmonary valves has also increased. Methods From 2000 to 2007, 146 artificial valves, such as the Carpentier-Edward Perimount, Hancock II, Biocor, homograft and hand-made valves were implanted for pulmonary valve in 132 patients with various forms of congenital heart disease. Among them, the outcomes of the Carpentier-Edward Perimount (n = 63) and the Hancock II (n = 40) valves were reviewed retrospectively. The mean age at initial implantation was 12.8 ± 6.6 years. The overall duration of follow up was 36.0 ± 24.2 months. Results There was an early death due to right ventricular failure with intractable ventricular arrhythmia and 3 late deaths due to progressive right ventricular failure, dilated cardiomyopathy and infective endocarditis. The overall survival and re-operation free rate was 96.3% and 89.8% respectively. Eight out of 63 Carpentier-Edward patients (12.6%) underwent re-replacement at 49.2 ± 25.2 months. The re-operation free rates were 97.7%, 87.7% and 50% at 1, 3 and 5 years respectively. There was no re-operation required for the 40 Hancock II patients over 18.0 ± 10.8 months. There was no statistical significance in the re-operation free rates between these 2 valves (p-value = 0.51). Conclusions The overall survival rate associated with pulmonary valve bioprosthetic valve implantation was acceptable. However, the re-operation freedom rate was not satisfactory at mid-term for the Carpentier-Edward.

Background This study was undertaken to evaluate long-term results of biventricular repairs for congenitally corrected transposition of the great arteries, and to analyze the risk factors that affect mortality and morbidity. Methods Between 1983 and 2009, 167 patients with congenitally corrected transposition of the great arteries underwent biventricular repairs. The physiologic repairs were performed in 123 patients, and anatomic repairs in 44. Average follow-up was 9.3 ± 6.6 years. Results Kaplan-Meier estimated survival was 83.3% ± 0.5% at 25 years in biventricular repair. In anatomic repair, left ventricular training and right ventricular dysfunction had negative impact on survival, but bidirectional cavopulmonary shunt had positive impact on survival. The reoperation-free ratio was 10.1% ± 7.8% at 22 years after physiologic repair, and 46.2% ± 12.4% at 15 years after anatomic repair ( p = 0.885). Freedom from any arrhythmia was 49.6% ± 7.5% at 22 years after physiologic repair, and 60.8% ± 14.8% at 18 years after anatomic repair ( p = 0.458). Freedom from systemic atrioventricular valve and ventricular dysfunction as well as tricuspid valve and right ventricular dysfunction was significantly higher in anatomic repair than in physiologic repair. Conclusions Long-term results of biventricular repair were satisfactory. Patients presenting with right ventricular dysfunction or need for left ventricular training represent a high-risk group of anatomic repair for which selection criteria are particularly important. Late functional outcomes of anatomic repair were excellent compared with physiologic repair. Anatomic repair is the procedure of choice for those patients if both ventricles are adequate or if surgical technique is modified with the help of additional a bidirectional cavopulmonary shunt.

No previous study has investigated the association between early trauma and suicidal behavior in medical students. We evaluated the types of early trauma which are the most strongly associated with a lifetime history of suicidal behavior in medical students.A total of 6986 medical students completed a self-administered questionnaire (response rates: 49.6% of the entire medical student body in Korea) which included lifetime suicidal behavior, stressors, and the Early Trauma Inventory Self Report-Short Form (ETISR-SF). This was used to evaluate the most serious forms of trauma experienced before the age of 18, including general trauma, physical, emotional and sexual abuse.Among medical students, lifetime prevalence of suicidal behavior was 34.0% for those who experienced early trauma and 18.1% in those without a history of trauma (chi(2)=215.7, p0.0001). Emotional abuse exhibited a higher odds ratio for lifetime suicidal behavior (OR=3.6, 95%CI=2.9-4.4) than other traumas including general trauma (OR=2.1, 95%CI=1.8-2.4), sexual (OR=2.0, 95%CI=1.5-2.8) or physical (OR=1.8, 95%CI=1.5-2.1) abuse, and current stressors including heavy stress (OR=1.5, 95%CI=1.4-1.8), poor physical health (OR=1.3, 95%CI=1.2-1.5), and poor economic status (OR=1.2, 95%CI=1.0-1.3). Emotional abuse also showed a higher odds ratio for lifetime suicidal ideation (OR=3.5, 95%CI=2.8-4.4), plan (OR=3.9, 95%CI=2.4-6.2), and attempt (OR=4.1, 95%CI=2.4-6.8) than other early traumas or stressors. In emotional abuse, a continuously cold or uncaring parental attitude exhibited a stronger association with lifetime suicidal behavior (OR=4.5, 95%CI=2.7-7.7) than other emotional abuse.Emotional abuse, especially continuous parental emotional abuse in childhood, is significantly associated with lifetime suicidal behavior in Korean medical students.

Background Sertoli cells (SC) have immunomodulative properties, and chemokine receptor 7 (CCR7) can optimize the systemic immunomodulatory effect by guiding SC from the periphery to the secondary lymphoid organs. Methods The effect of immortalized neonatal porcine SC (NPSCi) was evaluated by analysis of cytokine levels. Hyporesponsiveness to donor cells was determined by MLC and analysis of splenocyte phenotypes using a murine allogeneic skin graft model. The effect of CCR7-expressing NPSCi (NPSCi-CCR7) combined with cobra venom factor (CVF) was evaluated using a heterotopically transplanted murine allogeneic heart model. Results Expression of immune cytokines was markedly modulated by NPSCi. The lymphocyte proliferation and splenocyte phenotypes were significantly suppressed by NPSCi-CCR7. Although pre-transplantation of NPSCi or NPSCi-CCR7 did not prolong graft survival of allogeneic cardiac grafts, CVF treatment facilitated pre-transplantation of NPSCi-CCR7 to prolong survival of allogeneic cardiac grafts (25.5 ± 7.05 vs 9.5 ± 0.58 days, p Conclusions NPSCi may be used as a powerful immunomodulatory tool, and our strategy to traffic NPSCi to lymphoid organs using CCR7 optimizes the systemic immunomodulatory effect in vivo. With the help of initial immunosuppression for humoral mechanisms using CVF, the host immune response against allogeneic cardiac grafts can be effectively ameliorated by immunomodulation of the cellular mechanism with NPSCi-CCR7.

Background We implanted Shelhigh porcine pulmonic valve conduits because of the limited availability of homografts in our country. The aim of this study was to evaluate the short-term results of SPVC. Methods From November 2002 to July 2005, the Shelhigh porcine pulmonic valve conduit was implanted in 73 patients (81 procedures) in the right ventricular outflow tract to correct congenital heart diseases. Operative procedures were Rastelli operation in 65, anatomic correction of atrioventricular discordance in 5, and Ross operation in 3. Age at operation was 6.8 ± 7.5 years, including 11 patients under 1 year. The median conduit size was 18 mm (range, 12 to 24 mm). Results There was no operative mortality and 1 nonconduit-related late death (mean follow-up, 11.3 ± 10.7 months). Ten conduits (12.3%, 7 patients) were removed at a median of 9.6 months (range, 2.5 to 25.4) owing to obstruction in 9 and pseudoaneurysm in 1. In the explanted conduits, we found a prominent intimal peel at the distal anastomosis without leaflet calcification. Freedom from reoperation at 24 months was 87% ± 11.7% in large-sized conduits (≥18 mm) and 62.8% ± 10.6% in small-sized conduits (≤16 mm). Especially, 12-mm sized conduit showed 33.3% freedom from reoperation during the first 12 months of follow-up. Conclusions On the basis of our short-term results, Shelhigh porcine pulmonic valve conduits are not satisfactory. Small-sized conduits (≤16 mm) fail earlier; large-sized conduits (≥18 mm) fail after 2 years of implantation due to intimal peel formation at the distal segment.

Background: Effective intervention achieved by manipulating cell-mediated xenogeneic immune responses would critically increase the clinical feasibility of xenotransplantation as immediate hyperacute rejections become controllable through genetic modulations of donor organs. Endogenous negative regulatory signals like the programmed death 1 (PD-1)-programmed death ligand 1 (PD-L1) system are candidate targets for the control of cell-mediated xenogeneic immune response. Methods: A porcine PD-L1 molecule was cloned using RACE (rapid amplification of cDNA ends) technology based on the human PD-L1 sequence. The functional effects of cloned porcine PD-L1 were tested on human CD4+ T cell activation using porcine PD-L1-transfected bystander cells. Cellular proliferation was monitored by [3H] thymidine incorporation, and human T cell apoptosis was measured by flow cytometry. Results: Porcine PD-L1 (GenBank accession number AY837780) was found to have 73.8% sequence homology with human PD-L1 and to contain two immunoglobulin domains in its extracellular region. Moreover, porcine PD-L1 expressed on Chinese hamster ovary (CHO) cells inhibited human CD4+ T cell proliferation stimulated with anti-CD3 only or anti-CD3 plus anti-CD28. Percentages of apoptotic activated human T cells increased by over 30% in the presence of porcine PD-L1/CHO cells, and the addition of recombinant human PD-1-Fc fusion proteins during human T cell activation reversed the inhibitory effects of porcine PD-L1. Conclusions: Cloned porcine PD-L1 showed high sequence homology with human PD-L1 and a similar molecular structure. Moreover, porcine PD-L1 inhibited human CD4+ T cell activation in human PD-1-dependent manner, and this involved activated T cell apoptosis. The authors suggest that PD-1-PD-L1 might play an important endogenous immune regulatory role during xenogeneic transplantation, and that the effective application of this system would improve transplanted xenogeneic organ survival.

Sertoli cells (SC) are known to contain immunoprotective properties, which allow them to survive as allografts without the use of immunosuppressive drugs. Experiments were designed to determine which factors are related to prolonged survival of allogeneic SC. Balb/c derived Sertoli (TM4) and colon cancer (CT-26) cell lines were implanted beneath the kidney capsule of non-immunosuppressed C57BL/6 mice and compared their survival as allografts. Compared to TM4 graft, which survived more than 7 days after transplantation, CT-26 showed massive infiltration of polymorphonuclear cells, necrosis and enlargement of draining lymph nodes. Cultured cell lines showed no differences in their expression patterns of FasL, TGF beta1, clusterin and two complement regulatory proteins (CRP, i.e., membrane cofactor protein, MCP; decay accelerating factor, DAF), but protectin (CD59), another member of CRP was expressed only on TM4. These results suggest that CD59 and unknown factors may contribute to the prolonged survival of SC in non-immunoprivileged sites.

Although dendritic cells (DCs) are unrivaled for initiation of immune responses, the immunomodulatory capacity of chemically fixed DC has not been thoroughly evaluated. We monitored the tolerogenic capacity of chemically fixed DCs using allogeneic heart transplantations. Bone marrow progenitors were differentiated into immature DCs which were then chemically fixed and injected intravenously into recipient mice at 14 days before allogeneic heart transplantation. Chemically fixed DCs markedly prolonged graft survival in the major histocompatibility complex (MHC) I/II mismatch cardiac transplantation (B6 --> B10.A; median survival time [MST] 12.5 days vs >70 days). T cells that encountered chemically fixed DCs showed attenuated apoptotic cell death and inactivated phenotypes after allogeneic heterotropic heart transplantation. Furthermore, when DCs from interleukin (IL)-10-/- mice were treated, the in vitro T-cell response was greater than that from IL-12-/- mice. We have suggested that the chemically fixed DCs may mediate peripheral T-cell tolerance, with therapeutic potential for allogeneic transplantation.

Objective: The aim of this study was to determine the outcome of the neoaortic valve after the arterial switch operation for transposition of the great arteries. Methods: A retrospective review of arterial switch operations that were performed during the period from 1991 to 2003 was conducted. We followed patients with echocardiography. When regurgitation of the neoaortic valve was observed we analyzed the risk factors. Results: One hundred and three patients underwent a successful arterial switch operation. Eighty-one males and 22 females participated in the study. Follow-up period was 77 ± 42 months. The age and body weight at the time of the arterial switch operation were 1.4 ± 2.8 months and 3.8 ± 1.0 kg, respectively. Preoperative pulmonary valve regurgitation was found in six patients (two patients had grade I and four patients had grade II). In the postoperative echocardiography, 52 patients demonstrated neoaortic valve regurgitation (26 patients had grade I 25 patients had grade II, and 1 patient had grade III). At the last follow-up visit, 61 patients demonstrated neoaortic regurgitation (18 patients had grade I, 37 patients had grade II, 5 patients had grade III, and 1 patient had grade IV). Neoaortic valve regurgitation increased progressively with follow-up (p-value < 0.01). The size discrepancy between the aorta and the pulmonary artery was correlated with neoaortic valve regurgitation (p-value = 0.02). The age and body surface area, relationship of the great arteries, coronary arterial pattern, pulmonary artery banding, use of trapdoor technique, myocardial ischemic time, use of total circulatory arrest, and existence of ventricular septal defect were not significant risk factors. Conclusions: Neoaortic valve regurgitation progressed after the arterial switch operation. The degree of regurgitation was more severe in patients with a size discrepancy between the aorta and the pulmonary artery preoperatively.

Background: The immunoprotective nature of the testes has prompted numerous investigations into their supportive roles during allogeneic or xenogeneic cellular grafts. However, the optimal developmental stage of these cells in terms of maximum efficacy for cellular grafts has not been elucidated. In this study, the time-dependent expressions of immune-privilege- and proliferation-related molecules in Sertoli cells were determined. Methods: To investigate the time course of the expression of proteins related to immune privilege and proliferation, immunohistochemistry and reverse transcriptase-polymerase chain reaction (RT-PCR) were performed using testes of 18 Yorkshire pigs from birth to 20 weeks of age. We included fas ligand (FasL), transforming growth factor β1 (TGF-β1) and clusterin as the immune-protective molecules, and follicle stimulating hormone receptor (FSHR), p27Kip1, GATA-4 and Wilm's tumor antigen (WT1) as Sertoli cell proliferation markers. RT-PCR was used to complement histological assessment. Results: The expression of FasL in Sertoli cells gradually increased with age, whereas TGF-β1 showed the reverse pattern, and clusterin expression showed no age-related difference. p27Kip1 showed a gradual increase in its expression from week 12, but GATA-4 showed earlier postnatal expression from birth to week 12. WT1 expression gradually increased from week 16. Conclusion: We confirmed the age-dependent expression of immune-privilege- and proliferation-related molecules in porcine Sertoli cells. These data may be useful for determining the optimal time for harvesting Sertoli cells with respect to maximal immunoprotection and proliferative activity.

BACKGROUND The toll-like receptor (TLR) is stimulated by not only pathogen-associated molecular patterns but also endogenous TLR ligands provided by injured cells. The influence of cyclosporine A (CsA)-induced renal injury on TLR expression and subsequent signaling pathway was evaluated. METHODS Induction of chronic CsA nephropathy was made by administering CsA (15 mg/kg/day) for 28 days in rats. The TLR2 and TLR4 mRNA and protein expression, TLR-signaling pathway (MYD88, NF-kappaB and AP-1), putative TLR ligand (heat shock protein 70 [HSP70]), and maturation of dendritic cells were evaluated in CsA-treated rat kidneys. RESULTS Long-term CsA treatment upregulated TLR2 and TLR4 mRNA and protein expression on renal tubular cells, and these were accompanied by increased MYD88, NF-kappaB and AP-1 expression. Putative TLR ligand (HSP70) was also significantly increased in CsA-treated rat kidney compared with vehicle-treated rat kidney. CsA-treatment increased expression of TNF-alpha mRNA, the number of dendritic cells, and expression of MHC class II antigen. Double-labeling of markers of dendritic cells and MHC class II antigen revealed that matured dendritic cells increased in CsA-treated rat kidney. CONCLUSIONS CsA-induced renal injury stimulates components of innate immunity, and this finding suggests close association between CsA-induced renal injury and activation of innate immunity.

Although supraventricular tachycardia in complex congenital heart disease (CHD) has been reported after surgical repair, its exact electrophysiologic identification has been limited to intraatrial reentrant tachycardia (IART). Moreover, junctional tachycardia (JT) has not previously been described as a cause of late postoperative arrhythmia.Since 1993, a total of 12 patients with congenital heart disease presented with paroxysmal focal JT. The patients with only typical immediate postoperative junctional ectopic tachycardia were excluded. Medical records, standard electrocardiography and Holter monitoring were reviewed. An intracardiac electrophysiologic (EP) study was performed in 11 patients. Ten patients were in post-Fontan status (5.7% of total Fontan survivors). Focal JT occurred more frequently in heterotaxy syndrome among the Fontan survivors (7/52 vs. 3/124; P0.05). The commonest anatomy of the atrioventricular (AV) junction was complete AV canal in 8 patients. EP characteristics of focal JT were as follows: (1) various tachycardia mechanisms were identified (increased automaticity or a triggered mechanism in 6/11, and focal reentry in 5/11, including one concealed nodofascicular pathway) (2) ventriculoatrial conduction during tachycardia was either dissociation (7/12) or variable (5/12) (3) All JTs were terminated by adenosine. Class III antiarrhythmic agent was effective in 5/6. His bundle ablation was performed in one Fontan patient, who already had pacemaker because of accompanying intractable IART and sinus node dysfunction.Focal JT may be a source of late term supraventricular tachycardia in patients with complex CHD. The tachycardia mechanism was either automatic/triggered or reentrant. In all patients, JT was effectively terminated by adenosine.

The coexistence of two distinct atrioventricular (AV) nodes has been described in congenital heart disease requiring Fontan type palliation. The purpose of this study was to evaluate the occurrence of twin AV node according to anatomical subgroups, and to determine its relation to tachycardia.From 2001 to 2003, we performed an electrophysiologic (EP) study upon 52 consecutive patients who had undergone cardiac catheterization after Fontan completion. Atrial pacing was performed at three or more different atrial sites.In 10/52 patients, two different QRS complexes were recorded at different pacing sites, suggesting twin AV node (9/20 in right isomerism, 1/8 discordance AV connections, 0/24 other complex anomalies). AV reciprocating tachycardia (AVRT), presumably involving two AV nodes and a connecting sling, was induced in 6 of 10 patients who had twin AV node (4/6 used posterior AV node as an antegrade limb, 2/6 used an anterior AV node as an antegrade limb). Heterotaxy syndrome (P0.001) and complete AV septal defect (P = 0.002) were found to be risk factors for twin AV node. Junctional tachycardia (JT; HR150/min) with either VA dissociation (7/9) or second degree VA block (2/9) were induced by pacing or isoproterenol infusion in 9/52 patients.JT induction was associated with a twin AV node (P = 0.04), or a history of early postoperative junctional ectopic tachycardia (P = 0.02). A complicated AV node conduction system such as twin AV node was frequent in heterotaxy syndrome. Both AVRT and JT with VA block may be important causes of tachyarrhythmia in this patient group.