Search

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.

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.

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 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.

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.

Due to their unique capacity to self-renew and for multiple differentiation, stem cells are considered promising candidates for cell replacement therapy in many devastating diseases. However, studies on immune rejection, which is a major problem facing successful stem cell therapy, are rare. In this study, we examined MHC expression in the M13SV1 cell line, which has previously been shown to have stem cell properties and to be non-tumorigenic, in order to determine whether human adult stem cells might be rejected after transplantation. Our results show low expression levels of MHC class I molecules on the surface of these cells. An induction of MHC class I expression was observed when the cells were treated with IFN-gamma. Maximal induction of MHC class protein expression was observed at 48 h after treatment with concentrations above 5 ng/ml of IFN-gamma. Elevated MHC class I levels were sustained for 72 h after withdrawing IFN-gamma. Therefore, we introduced human cytomegalovirus (hCMV) US genes, which are known to be able to reduce MHC class I expression on the cell surface after infection, into M13SV1 cells. Cells transfected with the hCMV US2, US3, US6 or US11 genes exhibited a reduction (40-60%) of MHC class I expression compared with mock-transfected cells. These results suggest that human adult stem cells are capable of expressing high levels of MHC class I proteins, and thus may be rejected on transplantation unless they are modified. In addition, viral stealth mechanisms can be exploited for stem cell transplantation.

Background . The Lecompte procedure for correcting transposition of the great arteries has an advantage because it obviates the need for an extracardiac conduit for the reconstruction of the pulmonary outflow tract. In this study, we evaluated the effectiveness and the application of the Lecompte procedure based on our experiences. Methods . A retrospective review was conducted of the records of 45 patients who underwent the Lecompte procedure during the past 11 years to achieve direct right ventricle to pulmonary artery continuity. Mean age at operation was 2.4 ± 1.7 years (range 3.5 months to 6.9 years). The diagnoses involved anomalies of the ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction, such as transposition of the great arteries, double-outlet right ventricle, and double-outlet left ventricle. Results . Early mortality was 4.4% (2 of 45 patients) and late mortality was 4.7% (2 of 43). The mean follow-up was 4.9 ± 3.1 years. Fourteen patients (34.1% of survivors, n=41) had pulmonary stenosis (pressure gradient above 30 mm Hg), the main reason for which was a calcified monocusp valve (n = 10, 71.4%). Eight of 45 patients (17.8%) underwent reoperation: 2 for residual ventricular septal defect, 1 for recurrent septic vegetation, and 5 for pulmonary stenosis. The cumulative survival rates were 91.1% ± 4.2% at 10 years. The actuarial probabilities of freedom from reoperation for pulmonary stenosis were 93.8% ± 4.3% and 71.4% ± 11.8% at 5 and 10 years, respectively. Conclusions . Our review suggests that the Lecompte procedure is an effective treatment modality for anomalies of the ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction. Repair in early age is possible with acceptable morbidity and mortality, but recurrent right ventricular outflow tract obstruction caused by degeneration of the monocusp valve is a problem that needs resolution.

Anterior translocation of the pulmonary root was used as a new approach to the staged repair of Taussig-Bing anomaly with an interrupted aortic arch. It was performed to construct the right ventricle outflow tract with intraventricular baffling of the left ventricle to the aorta as the second stage operation after repair of the interrupted arch and pulmonary artery banding. This technique allows minimization of pulmonary regurgitation and has the major theoretical advantage for growth potential, which could diminish the need for reoperation.

We describe an alternative technique to the extracardiac Fontan procedure in a patient with a univentricular heart, in which the inferior caval vein and the hepatic vein drained separately into the common atrium and the intraatrial orifice of the hepatic vein was adjacent to the opening of the left lower pulmonary vein.