Search

Pulmonary atresia with an intact ventricular septum (PAIVS) is a rare congenital cardiac disease with various structural abnormalities. For patients with moderate right ventricular (RV) hypoplasia for whom the decision has not been made between biventricular and single ventricular repair, if possible, the ideal treatment choice is biventricular repair, by promoting the growth of the underdeveloped RV. Since RV sinus myectomy has been proposed, various surgical strategies have been introduced to promote RV growth. Herein, we described instances of RV sinus myectomy and the accompanying techniques for “RV overhaul.” Furthermore, we introduced a current approach for small patients with PAIVS within an uncertain area.

We encountered siblings with familial Majewski osteodysplastic primordial dwarfism type II (MOPD II) with acute myocardial infarction in adolescence and in their early 20s. We successfully performed percutaneous and surgical coronary interventions. From these cases, we were able to better understand coronary artery disease of MOPD II and provide better management. (Level of Difficulty: Intermediate.), Central Illustration

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

Cardiac resynchronization therapy (CRT) is an effective treatment for heart failure. However, in pediatric and congenital heart disease (CHD) patients, current adult indications cannot be directly applied because of heterogeneity in anatomy and diagnosis. Therefore, CRT responses and clinical outcomes in these patients were investigated to derive possible candidates for CRT.This study retrospectively analyzed 16 pediatric and CHD patients who underwent CRT implantation at a single center in early (0.7±0.2 year) and late (4.7±0.3 years) follow-up period after CRT.The median age at CRT implantation was 2.5 (0.3-37.2) years, and median follow-up duration was 6.3 (0.1-13.6) years. Thirteen had non-transvenous CRT. Two had congenital complete atrioventricular (AV) block with previous right ventricular pacing, 5 had dilated cardiomyopathy (DCM) with left bundle branch block, and 9 had CHD. The mean ejection fraction of the systemic ventricle increased from 28.1±10.0% to 44.3±21.0% (p=0.003) in early and 51.8±16.3% (p=0.012) in late outcome. The mean functional class improved from 3.1±0.9 to 1.8±1.1 after CRT (p=0.003). Twelve patients (75%) showed improvement in ventricular function or functional class after CRT. Proportion of responders differed between patients without CHD (2/2 patients with complete AV block and 5/5 with DCM, 100%) and those with CHD (5/9, 56%), although statistical significance was not reached (p=0.088).CRT improved ventricular function and functional status according to the underlying condition in pediatric and CHD patients. However, further large and longer-term studies are needed to establish the guideline for the patient selection of CRT in these patients.

Background IPNB is very rare disease and most previous studies on IPNB were case series with a small number due to low incidence. The aim of this study is to validate previously known clinicopathologic features of intraductal papillary neoplasm of bile duct (IPNB) based on the first largest multicenter cohort. Methods Among 587 patients previously diagnosed with IPNB and similar diseases from each center in Korea, 387 were included in this study after central pathologic review. We also reviewed all preoperative image data. Results Of 387 patients, 176 (45.5%) had invasive carcinoma and 21 (6.0%) lymph node metastasis. The 5-year overall survival was 80.9% for all patients, 88.8% for IPNB with mucosal dysplasia, and 70.5% for IPNB with invasive carcinoma. According to the “Jang & Kim's modified anatomical classification,” 265 (68.5%) were intrahepatic, 103 (26.6%) extrahepatic, and 16 (4.1%) diffuse type. Multivariate analysis revealed that tumor invasiveness was a unique predictor for survival analysis. (p = 0.047 [hazard ratio = 2.116, 95% confidence interval 1.010–4.433]). Conclusions This is the first Korean multicenter study on IPNB through central pathologic and radiologic review process. Although IPNB showed good long-term prognosis, relatively aggressive features were also found in invasive carcinoma and extrahepatic/diffuse type.

Backgrounds/aims The comparative effectiveness of pylorus-resecting pancreaticoduodenectomy (PRPD) and pylorus- preserving pancreaticoduodenectomy (PPPD) in pancreatic head cancer is still disputed. The aim of this study was to analyze the data obtained from a large, single center with PPPD compared with PRPD in terms of postoperative outcomes, including blood glucose levels and survival in patients with pancreatic head cancer. Methods Between January 2007 and December 2016, a total of 556 patients with pancreatic head cancer underwent either PPPD or PRPD. We analyzed the clinicopathologic data to assess short- and long-term outcomes retrospectively. Results For underlying disease, patients with DM in PPPD were fewer than in PRPD (33.0% vs. 46.2%, p=0.002). The median value of CA19-9 was significantly higher in PRPD than in PPPD (129.36 vs. 86.47, p=0.037). The incidence of Clavien-Dindo grade III to V major complications in PPPD was significantly higher than in PRPD (20.4% vs. 13.4%, p=0.032). Resection of pylorus was shown to reduce complications in univariate and multivariate analyses (p=0.032 and = 0.021, respectively). The 5-year survival rates were 27.6% in the PPPD group and 22.4% in the PRPD group (p=0.015). Conclusions The results of PPPD and PRPD showed no significant differences from those reported conventionally in previous studies. Although further well-designed studies are needed, it is more important to select the range of surgical resection for the patient's disease regardless of resection of pylorus.

Background Elevated levels of serum indoxyl sulfate (IS) have been linked to cardiovascular complications in patients with chronic kidney disease (CKD). Oral sorbent therapy using spherical carbons selectively attenuates IS accumulation in CKD patients. This study aimed to investigate whether oral administration of a new oral spherical carbon adsorbent (OSCA), reduces serum IS levels in moderate to severe CKD patients. Methods This prospective, multicenter, open-label study enrolled patients with CKD stages 3–5. Patients were prescribed OSCA for 8 weeks (6 g daily in 3 doses) in addition to standard management. Serum IS levels were measured at baseline and 4 and 8 weeks of treatment with OSCA. Results A total of 118 patients were enrolled and 87 eligible patients completed 8 weeks of study. The mean age of the study subjects was 62.8 ± 13.7 years, and 80.5% were male. Baseline levels of serum IS were negatively correlated with estimated glomerular filtration rate (eGFR) (r = − 0.406, P P for trend = 0.001). The patients showed significant reduction in serum total IS levels as early as 4 weeks after OSCA treatment (22.5 ± 13.9% reduction from baseline, P P Conclusions OSCA effectively reduced serum IS levels in moderate to severe CKD patients. Gastrointestinal symptoms were the most commonly reported complications, but no treatment-related severe adverse effects were reported. Trial registration Clinical Research Information Service (KCT0001875. 14 December 2015.)

In gallbladder cancer, stage T2 is subdivided by tumour location into lesions on the peritoneal side (T2a) or hepatic side (T2b). For tumours on the peritoneal side (T2a), it has been suggested that liver resection may be omitted without compromising the prognosis. However, data to validate this argument are lacking. This study aimed to investigate the prognostic value of tumour location in T2 gallbladder cancer, and to clarify the adequate extent of surgical resection.Clinical data from patients who underwent surgery for gallbladder cancer were collected from 14 hospitals in Korea, Japan, Chile and the USA. Survival and risk factor analyses were conducted.Data from 937 patients were available for evaluation. The overall 5-year disease-free survival rate was 70·6 per cent, 74·5 per cent for those with T2a and 65·5 per cent among those with T2b tumours (P = 0·028). Regarding liver resection, extended cholecystectomy was associated with a better 5-year disease-free survival rate than simple cholecystectomy (73·0 versus 61·5 per cent; P = 0·012). The 5-year disease-free survival rate was marginally better for extended than simple cholecystectomy in both T2a (76·5 versus 66·1 per cent; P = 0·094) and T2b (68·2 versus 56·2 per cent; P = 0·084) disease. Five-year disease-free survival rates were similar for extended cholecystectomies including liver wedge resection versus segment IVb/V segmentectomy (74·1 versus 71·5 per cent; P = 0·720). In multivariable analysis, independent risk factors for recurrence were presence of symptoms (hazard ratio (HR) 1·52; P = 0·002), R1 resection (HR 1·96; P = 0·004) and N1/N2 status (N1: HR 3·40, P 0·001; N2: HR 9·56, P 0·001). Among recurrences, 70·8 per cent were metastatic.Tumour location was not an independent prognostic factor in T2 gallbladder cancer. Extended cholecystectomy was marginally superior to simple cholecystectomy. A radical operation should include liver resection and adequate node dissection.En el cáncer de vesícula biliar, la ubicación del tumor subdivide el estadio T2 en tumores con invasión del lado peritoneal y del lado del hígado (T2a y T2b). Para los tumores que invaden el lado peritoneal (T2a) se sugiere que se puede obviar la resección hepática sin que ello comprometa el pronóstico. Sin embargo, este argumento no ha sido validado. El estudio tuvo como objetivo investigar el valor pronóstico de la localización del tumor en el cáncer de vesícula biliar T2 y establecer la extensión adecuada de la resección quirúrgica. MÉTODOS: Se recogieron los datos clínicos de pacientes que se sometieron a cirugía por cáncer de vesícula biliar en 14 hospitales de Corea, Japón, Chile y Estados Unidos. Se realizaron análisis de la supervivencia y de los factores de riesgo.Se dispuso de datos de 937 pacientes para ser evaluados. La tasa de supervivencia global libre de enfermedad a los 5 años fue del 70,6%, y las de T2a y T2b del 74,5% y 65,5% (P = 0,028). Con respecto a la resección hepática, la colecistectomía extendida presentó una tasa mejor de supervivencia libre de enfermedad a los 5 años que la colecistectomía simple (73,0% versus 61,5%, P = 0,012). La tasa de supervivencia libre de enfermedad a los 5 años fue marginalmente mejor para la colecistectomía extendida que para la colecistectomía simple tanto en T2a (76,5% versus 66,1%, P = 0,094) como en T2b (68,2% versus 56,2%, P = 0,084). Las tasas de supervivencia libre de enfermedad a los 5 años no fueron diferentes entre la resección hepática en cuña y la segmentectomía S4b+S5 (74,1% versus 71,5%, P = 0,720). En el análisis multivariable, los factores de riesgo independientes para la recidiva fueron la presencia de síntomas (cociente de riesgos instantáneos, hazard ratio, HR 1,52, P = 0,002), la resección R1 (HR 1,96, P = 0,004) y el estadio N1/N2 (N1 HR 3,40, P0,001; N2 HR 9,56, P0,001). El 70,8% de las recidivas eran metastásicas. CONCLUSIÓN: La localización del tumor no fue un factor pronóstico independiente en el cáncer de vesícula biliar T2. La colecistectomía extendida fue marginalmente superior que la colecistectomía simple. La cirugía radical debe incluir una resección hepática y una linfadenectomía adecuada.

Protein-losing enteropathy (PLE) is a devastating complication after the Fontan operation. This study aimed to investigate the clinical characteristics, treatment response, and outcomes of Fontan-associated PLE.We reviewed the medical records of 38 patients with Fontan-associated PLE from 1992 to 2018 in 2 institutions in Korea.PLE occurred in 4.6% of the total 832 patients after the Fontan operation. After a mean period of 7.7 years after Fontan operation, PLE was diagnosed at a mean age of 11.6 years. The mean follow-up period was 8.9 years. The survival rates were 81.6% at 5 years and 76.5% at 10 years. In the multivariate analysis, New York Heart Association Functional classification III or IV (p=0.002), low aortic oxygen saturation (90%) (p=0.003), and ventricular dysfunction (p=0.032) at the time of PLE diagnosis were found as predictors of mortality. PLE was resolved in 10 of the 38 patients after treatment. Among medical managements, an initial heparin response was associated with survival (p=0.043). Heparin treatment resulted in resolution in 4 patients. We found no evidence on pulmonary vasodilator therapy alone. PLE was also resolved after surgical Fontan fenestration (2/6), aortopulmonary collateral ligation (1/1), and transplantation (1/1).The survival rate of patients with Fontan-associated PLE has improved with the advancement of conservative care. Although there is no definitive method, some treatments led to the resolution of PLE in one-fourth of the patients. Further investigations are needed to develop the best prevention and therapeutic strategies for PLE.

Backgrounds/aims Although intraductal papillary mucinous neoplasm (IPMN) has showed a favorable prognosis compared to pancreatic ductal adenocarcinoma, its recurrence patterns have somewhat questionable in detail. After partial pancreatectomy for IPMN, the evaluation for risk of metachronous occurrence of high-risk lesions (HRL) in the residual pancreas is important to establish a postoperative surveillance modality and duration of follow-up. This study aimed to evaluate the factors that may predict the metachronous occurrence of HRL in the remnant pancreas after surgery of the IPMN. Methods From 2005 to 2016, clinicopathologic and surveillance data for 346 consecutive patients who underwent surgical resection for IPMN were reviewed retrospectively. Histologic subtype was classified as gastric, intestinal, pancreato-biliary, or oncocytic type. Results All of IPMN were classified as main duct (n=64, 18.5%), branch duct (n=171, 49.4%), and mixed type (n=111, 32.1%). Forty-eight patients (13.9%) experienced recurrence during follow-up. Among these, 9 patients (2.6%) were identified to metachronous development of HRL in the remnant pancreas. After multivariate analysis, high-grade dysplasia (HGD) or invasive carcinoma (IC) compared to low- or intermediate dysplasia was only independent risk factor for recurrence (HR 3.688, 95% CI 2.124- 12.524, p=0.009). The independent risk factors for metachronous development were HGD/IC (HR 8.414, 95% CI 4.310- 16.426, p=0.001), and intestinal/pancreato-biliary subtype compared to gastric subtype (HR 7.874, 95% CI 3.650- 27.027, p=0.010). Conclusions Patients with high-grade dysplasia or invasive carcinoma, and with intestinal or pancreatobiliary subtype should undergo close, long-term surveillance of the remnant pancreas after initial resection.

Because Fontan circulation does not have a subpulmonary ventricle, the preload is limited. In Fontan circulation with extracardiac conduit, the size of conduit could be an important factor in determining the preload. We compared exercise capacity with each conduit size and tried to search for optimal conduit size in Fontan circulation. We reviewed the medical record of 677 patients with Fontan circulation. Patients who had other type Fontan circulation (Kawashima, atriopulmonary, lateral tunnel), SpO2

Background In 2013, the fistula risk score (FRS) was developed to assess the risk of clinically relevant postoperative pancreatic fistula (CR‐POPF). In 2017, the alternative FRS (a‐FRS) was proposed. The purpose of this study was to validate the original FRS (o‐FRS) and a‐FRS for CR‐POPF in pancreaticoduodenectomy (PD). Methods From January 2007 to December 2016, 1,771 patients underwent PD for periampullary cancers. POPF was defined and classified according to the 2016 International Study Group for Pancreatic Fistula. All data were reviewed retrospectively. Results Pathologic diagnosis other than ductal adenocarcinoma (P < 0.001), pancreas duct diameter (P < 0.001), and body mass index (P < 0.001) were independent risk factors for CR‐POPF. Pancreatic texture (P = 0.534) and estimated blood loss (P = 0.827) were not associated with CR‐POPF. The CR‐POPF incidence increased with increasing o‐FRS score (P < 0.001), and also increased statistically significantly with increasing a‐FRS in the higher risk group (P < 0.001). However, the correlations differed. The area under the curve was 0.629 for o‐FRS and 0.622 for a‐FRS. Conclusions Both o‐FRS and a‐FRS might reflect CR‐POPF incidence, but some risk factors had no or low statistical significance. Further research is needed to revise the FRS.

Pulmonary valve replacement (PVR) is often performed in patients with repaired tetralogy of Fallot (TOF). Concomitant tricuspid valvuloplasty (TVP) in those with tricuspid regurgitation (TR) at the time of PVR is still controversial. We retrospectively reviewed clinical records of patients who underwent PVR between 2001 and 2012. We analyzed the impact of concomitant TVP on the tricuspid valve function and right ventricle function and size in mid-term. 119 patients with mild to moderate TR at the time of PVR were enrolled. 33 patients underwent concomitant TVP (TVP group) and 86 patients underwent PVR alone (no-TVP group). There was a significant reduction of TR (p

BACKGROUND/PURPOSE The current study aimed to develop a prediction model using a multi-marker panel as a diagnostic screening tool for pancreatic ductal adenocarcinoma. METHODS Multi-center cohort of 1991 blood samples were collected from January 2011 to September 2019, of which 609 were normal, 145 were other cancer (colorectal, thyroid, and breast cancer), 314 were pancreatic benign disease, and 923 were pancreatic ductal adenocarcinoma. The automated multi-biomarker Enzyme-Linked Immunosorbent Assay kit was developed using three potential biomarkers: LRG1, TTR, and CA 19-9. Using a logistic regression model on a training data set, the predicted values for pancreatic ductal adenocarcinoma were obtained, and the result was classification into one of the three risk groups: low, intermediate, and high. The five covariates used to create the model were sex, age, and three biomarkers. RESULTS Participants were categorized into four groups as normal (n = 609), other cancer (n = 145), pancreatic benign disease (n = 314), and pancreatic ductal adenocarcinoma (n = 923). The normal, other cancer, and pancreatic benign disease groups were clubbed into the non-pancreatic ductal adenocarcinoma group (n = 1068). The positive and negative predictive value, sensitivity, and specificity were 94.12, 90.40, 93.81, and 90.86, respectively. CONCLUSIONS This study demonstrates a significant diagnostic performance of the multi-marker panel in distinguishing pancreatic ductal adenocarcinoma from normal and benign pancreatic disease states, as well as patients with other cancers.

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

Background Early diagnosis is paramount in increasing the survival rate of pancreatic ductal adenocarcinoma (PDAC). However, effective early diagnostic tools are lacking at present. The current study aimed to develop a prediction model using a multi-marker panel (LRG1, TTR, and CA19-9) as a diagnostic screening tool for PDAC. Methods A large multi-center cohort of 1,991 samples were collected from January 2011 to September 2019, of which 609 are normal (NL), 145 are other cancer (OC; colorectal, thyroid, and breast cancer), 314 are pancreatic benign disease (PB), and 923 are PDAC. The automated multi-biomarker Enzyme-Linked Immunosorbent Assay kit was developed using three potential biomarkers, LRG1, TTR, and CA 19 − 9. Using a logistic regression (LR) model trained on training data set, the predicted values for PDACs were obtained, and the result was classified into one of the three risk groups: low, intermediate, and high. The five covariates used to create the model were sex, age, and biomarkers TTR, CA 19 − 9, and LRG1. Results Participants were categorized into four groups as NL (n = 609, 30.6%), OC (n = 145, 7.3%), PB (n = 314, 15.7%), and PDAC (n = 923, 46.4%). The NL, OC, and PB groups were clubbed into the non-PDAC group (n = 1068, 53.6%). The positive and negative predictive value, sensitivity, and specificity were 94.12, 90.40, 93.81, and 90.86, respectively. Conclusions This study demonstrates a significant diagnostic performance of the multi-marker panel in distinguishing PDAC from normal and benign pancreatic disease states, as well as patients with other cancers. The study indicates that the introduced multi-marker panel prediction model for PDAC diagnosis can help guide medical decisions for patients, including patients with early stage PDAC or with normal levels of CA 19 − 9.

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

Nephrogenic systemic fibrosis (NSF) is a progressive systemic fibrosing disease that may occur after gadolinium contrast exposure. It can lead to severe complications and even death. NSF is highly prevalent among patients with advanced chronic kidney disease (CKD). In this report, however, we describe the case of a patient with NSF that occurred during early CKD. A 65-year-old man with stage 3a CKD was transferred to our hospital because of lower extremity edema. The medical history revealed that he was exposed to gadolinium 185 days earlier, and the result of his tibial skin biopsy was consistent with NSF. The patient underwent a combined therapy with ultraviolet-A1 phototherapy and methotrexate and steroid therapy for 6 months. The combined therapy stopped the systemic progression of NSF., Graphical Abstract

Background and Aims Both high salt diet and abnormal lipid metabolism are critical risks of vascular endothelial dysfunction. This study investigated the interactive effects of increased sodium intake and elevated serum triglyceride (TG) on elevated blood pressure (BP). Method We conducted nation-wide, population-based interaction analysis using Kawasaki method for estimating 24-h urinary sodium excretion (e24hUNaEKawasaki) as a candidate indicator of dietary sodium intake. Eligible as cases were all native Koreans aged 20 years or older without significant medical illness. Results: A total of 16936 participants were divided into quintiles according to their e24hUNaEKawasaki results. Participants in the highest quintile were more obese and hypertensive and had increased white blood cell count, decreased hemoglobin, greater glycemic exposure, and poor lipid profiles as compared to lower quintiles. Linear regression showed that e24UNaEKawasaki was closely related with dietary sodium intake, systolic BP, diastolic BP, and TG. Multiple logistic regression, adjusted for dietary sodium intake and various conventional risk factors of hypertension, demonstrated that both e24UNaEKawasaki and TG were significant predictors of hypertension. Our interaction analysis revealed that high sodium intake had a bigger effect on the risk of hypertension in participants with elevated TG than those without (adjusted RERI = 0.022, 95% CI = 0.017-0.027; adjusted AP = 0.017, 95% CI = 0.006-0.028; adjusted SI = 1.010, 95% CI = 1.007-1.014). Conclusion Our findings indicate that the interaction of high salt diet and abnormal lipid metabolism may exert synergistic biologic effect on the increased arterial BP.

The 8th American Joint Committee on Cancer (AJCC) staging system for distal cholangiocarcinoma (DCC) included a positive lymph node count (PLNC), but a comparison of the prognostic predictive power of PLNC and lymph node ratio (LNR) is still under debate. This study aimed to compare various staging models made by combining the abovementioned factors, identify the model with the best predictive power, and propose a modified staging system. We retrospectively reviewed 251 patients who underwent surgery for DCC at four centers. To determine the superiority of various staging models for predicting overall OSR, Akaike information criterion (AIC), Bayesian information criterion (BIC), AIC correction (AICc), and Harrell&rsquo, s C-statistic were calculated. In multivariate analysis, age (p = 0.003), total lymph node count (p = 0.033), and revised T(LNR)M staging (p &lt, 0.001) were identified as independent factors for overall survival rate. The predictive performance of revised T (LNR) M staging (AIC: 1288.925, BIC: 1303.377, AICc: 1291.52, and Harrell&rsquo, s C statics: 0.667) was superior to other staging system. A modified staging system consisting of revised T category and LNR predicted better overall survival of DCC than AJCC 7th and AJCC 8th editions. In the future, external validation of the proposed new system using a larger cohort will be required.

Background: The aim of this study is to compare the prognostic impact of 2 precursor lesions of ampullary adenocarcinoma, intra-ampullary papillary-tubular neoplasm (IAPN) and flat dysplasia (FD). Methods: From December 1994 to December 2012, a total of 359 patients underwent curative surgery for ampullary adenocarcinoma. Results: The precursor lesions were IAPNs in 134 (37.3%) patients and FD in the other 225 (62.7%) patients. The FD group had more aggressive tumor biology with advanced T stage (p = 0.002), nodal involvement (p < 0.001), poor differentiation (p < 0.001), perineural and lymphovascular invasion (p < 0.001), and pancreatobiliary or mixed subtype (p < 0.001). Five-year overall survival rates were 71.1% in the IAPN group and 51.4% in the FD group (p = 0.002), respectively. Five-year disease-free survival rates were 69.7% in the IAPN group and 49.6% in the FD group (p < 0.001), respectively. The recurrence rate was also higher in the FD group (49.8 vs. 30.6%; p < 0.001). On multivariate analysis, higher levels of tumor markers including CEA and CA19-9, lymph node metastasis, poorly differentiated histology, and perineural invasion were negative predictive factors for survival. Higher levels of CEA and CA19-9, lymphovascular invasion, and FD were independent prognostic factors for recurrence. Conclusion: FD was significantly associated with worse prognosis and a greater tendency toward advanced disease. Further studies are needed to clarify the impacts of these precursor lesions.

Purpose Postcholecystectomy syndrome (PCS) is characterized by abdominal symptoms following gallbladder removal. However, there is no consensus for the definition or treatment for PCS. The purpose of this study was to define PCS among various symptoms after laparoscopic cholecystectomy, and to identify risk factors affecting PCS. Methods This study was conducted at Dongguk University Ilsan Hospital and Chung-Ang University Hospital (2012-2013). Outcomes were assessed using European Organization for Research and Treatment of Cancer QLQ-C30 questionnaire. Symptom cluster for determining PCS was made by factor analysis. Cluster analysis evaluating risk factors of PCS was made by Ward methods and Dentogram. Results Factor analysis revealed three distinct symptom clusters, those are 'insomnia and financial difficulties (eigenvalue, 1.707; Cronbach α, 0.190),' 'appetite loss and constipation (eigenvalue, 1.413; Cronbach α, 0.480),' and 'right upper quadrant (RUQ) pain and diarrhea (eigenvalue, 1.245; Cronbach α, 0.315).' Among these symptom clusters, the cluster of 'RUQ pain and diarrhea' was determined as PCS. However, we could not find any risk factors between high symptomatic group and low symptomatic group. Conclusion PCS could consist of RUQ pain and diarrhea. Well-designed prospective trials are needed to determine risk factors of PCS.

Background/aims This study was designed to investigate the roles of aristolochic acid I (AA-I) and hypokalemia in acute aristolochic acid nephropathy (AAN). Methods After an adaptation period (1 week), a total of 40 C57BL/6 mice (male, 8 weeks old) were divided into four groups: I (control group), II (low potassium [K] diet), III (normal K diet with administration of AA-I [10 mg/kg weight]), and IV (low K diet with AA-I). After collecting 24 hours of urine at 2 weeks, the mice were sacrificed, and their blood and kidneys were obtained to perform immunochemical staining and/or Western blot analysis. Results Proteinuria, glycosuria, and increased fractional excretion of sodium and K were prominent in groups III and IV (p l 0.05). Diffuse swelling and poor staining of collecting duct epithelial cells were evident in the medullas of group II. Typical lesions of toxic acute tubular injury were prominent in the cortices of groups III and IV. Α-Smooth muscle actin (α-SMA) was higher in the cortices of the mice in groups III and IV versus group II (p l 0.05), and higher in the medullas of group IV than groups I and III (p l 0.05). E-cadherin was higher in the cortices of groups III and IV compared to group I (p l 0.05). The F4/80 value was higher in the cortices and medullas of groups II, III, and IV compared to group I (p l 0.05), particularly in the case of group II. Conclusions AA-I can induce acquired Fanconi syndrome in the acute stage of AAN. Macrophages appear to play a key role in the pathogenesis of AAN and hypokalemic nephropathy. It remains uncertain whether hypokalemia plays any role in AAN and hypokalemia.

Simple Summary Pancreatic acinar cell carcinoma accounts for less than 1% of primary pancreatic neoplasms. Because of its rarity, its characteristics and clinical outcomes remain unclear. Treatment strategies for pancreatic acinar cell carcinoma have relied on those of pancreatic ductal adenocarcinoma. In previous studies, it has been difficult to identify its characteristics due to the lack of cohort numbers in single institutional studies with detailed data and the lack of detailed data in large cohort multi-institutional studies. This retrospective multicenter cohort used a database founded in 2015 by the Korean Association of Hepato-Biliary Pancreatic Surgery. This database has collected nationwide patient data with details. In the present study, we aimed to better understand clinical outcomes of resected pancreatic acinar cell carcinoma and to lay the groundwork for establishing proper treatment strategies. Abstract Given the rare incidence of pancreatic acinar cell carcinoma (PACC), its post-resection clinical outcomes remain unclear. Treatment strategies for PACC have relied on those of pancreatic ductal adenocarcinoma (PDAC). The present study retrospectively investigated clinicopathologic characteristics of resected PACC registered in the Korea Tumor Registry System Biliary Pancreas database. Among 59 patients with a mean age of 59.2 years and a male predominance (83.1%), 43, 5, 7, and 4 had pure PACC, ductal differentiations, mixed neuroendocrine carcinomas, and intraductal and papillary variants, respectively. The mean tumor size was 4.6 cm, consisting of eight at T1, 26 at T2, and 25 at T3 stages. Metastasis to regional lymph node was identified in 15 (25.4%) patients. Thirty-one (52.5%) patients received adjuvant therapy. Five-year survival rate was 57.4%. The median survival was 78.8 months. In survival comparison according to the stage with AJCC system, N stage (lymph node metastasis), but not T stage, showed significant differences (p = 0.027). Resected PACC appeared to have clinical outcomes distinct from those of PDAC in this nationwide study. Therefore, large-scale multinational studies are needed to overcome the rarity of PACC and to establish an appropriate treatment strategies and staging system.

OBJECTIVES This study aimed to compare the effects of hypothermic ventricular fibrillation and beating-heart techniques during cardiopulmonary bypass (CPB) on postoperative outcomes after simple pulmonary valve replacement in patients with repaired tetralogy of Fallot (TOF). METHODS We retrospectively reviewed the data of 47 patients with repaired tetralogy of Fallot at a single institution, who received pulmonary valve replacement under the ventricular fibrillation or beating-heart technique without cardioplegic cardiac arrest during CPB between January 2005 and April 2015. RESULTS The patients were divided into fibrillation (n = 32) and beating-heart (n = 15) groups. On comparing these groups, the fibrillation group had a larger sinotubular junction (27.1 ± 4.6 vs 22.1 ± 2.4 mm), had a longer operation duration (396 ± 108 vs 345 ± 57 min), required more postoperative transfusions (2.1 ± 2.6 vs 5.0 ± 6.3 units) and had a higher vasoactive-inotropic score at intensive care unit admission (8.0 vs 10, all P

Background The prognosis for patients with pancreatic cancer is extremely poor. The diagnosis of pancreatic ductal adenocarcinoma at an early stage is uncommon. The purpose of this study was to analyze the clinicopathological characteristics of patients with pathologically proven pancreatic ductal adenocarcinoma following surgical resection and their actual 5 year survival rates, especially for those with T1 and T2 early stage cancer. Methods Retrospective analysis was performed for 433 patients with pancreatic ductal adenocarcinoma who underwent resection at Samsung medical center between May 1995 and December 2010. The actual 5 year survival rates and prognostic factors were analyzed. Results Multivariate analysis showed that positive resection margin, poor differentiation, large tumor size, large amount of blood loss, and T3/T4 were independent prognostic factors on overall survival. The median survival for T1/T2 stage was 71.7 months compared to 16.1 months for those with T3/T4 stage. The actual 5 year survival rates for T1/T2 and T3/T4 stages were 66.7% and 18.4%, respectively. Conclusions T stage is one of the strongest independent prognostic factor for overall survival of patients with pancreatic cancer. T1/T2 pancreatic ductal adenocarcinoma showed good survival outcome. Therefore, additional efforts are needed to improve the screening for early detection.

Background/Introduction Lowering blood pressure is very beneficial in reducing the risk of cardiovascular morbidity and mortality. But, the extent of optimal target blood pressure in hypertensive patients is still controversial. Purpose The objectives of this study were to assess the level of proper systolic and diastolic blood pressure to prevent cardiovascular events in older and younger patients. Methods We used the National Sample Cohort from the National Health Insurance Service of 2007 to 2013 in Korea and analyzed data from 44,462 hypertensive patients aged from 20 to 84, treated with one or more antihypertensive agents and participated at least one general health examination. Achieved systolic and diastolic blood pressure (SBP/DBP) were categorized by exclusive average achieved SBP ( Results Of 44,462 patients, 5,478 (12.3%), 13,410 (30.2%), 15,021 (33.8%), 7,051 (15.9%), and 3,502 (7.9%) patients achieved SBP Conclusion In conclusion, an intensive lowering of blood pressure is more likely to increase the risk rather than to prevent major cardiovascular events and all-cause death, particularly in older than younger. Therefore, an intensive blood pressure lowering of SBP/DBP below 120/70 mm Hg in the elderly should be avoided. Acknowledgement/Funding The Korea National Institute of Health research grant 2017-NI63001-00

We studied the effects of pancreatic enzyme replacement therapy (PERT) on body weight, nutritional status, and quality of life (QoL) in patients with pancreatic exocrine insufficiency after pancreatoduodenectomy.We performed a randomized, double-blind trial of 304 patients who underwent pancreatoduodenectomy at 7 tertiary referral hospitals in South Korea. Patients with fecal levels of elastase of 200 μg/g or less, before and after surgery, were assigned randomly to groups that received PERT (a single capsule of 40,000 IU pancreatin, Norzyme (40,000 IU, Pankreatan; Nordmark Arzneimittel GmbHCo, Uetersen, Germany), 3 times each day during meals for 3 months; n = 151) or placebo (n = 153). Protocol completion was defined as taking more than two thirds of the total dose without taking other digestive enzymes; the protocol was completed by 71 patients in the PERT group and 93 patients in the placebo group. Patients underwent a physical examination, oral glucose tolerance tests, and blood tests at baseline and at month 3 of the study period. The primary end point was change in body weight. Secondary end points were changes in bowel habits, nutritional parameters, and QoL.In the per-protocol analysis, 3 months after the study began, patients in the PERT group gained a mean of 1.09 kg in weight and patients in the placebo group lost a mean of 2.28 kg (difference between groups, 3.37 kg; P.001). However, no difference in body weight was observed between groups in the intent-to-treat analysis. Three months after the study began, the mean serum levels of prealbumin increased by 10.9 mg/dL in the PERT group and increased by 7.8 mg/dL in the placebo group (P = .002). Poor compliance to PERT was a significant risk factor for weight loss (P.001). There was no significant difference in QoL scores between groups.In the intent-to-treat analysis of data from a randomized trial, we found no significant effect of PERT on mean body weights of patients with pancreatic exocrine insufficiency after pancreatoduodenectomy. However, with active education and monitoring, PERT could increase body weight and nutritional parameters. ClinicalTrials.gov no: NCT02127021.

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.

OBJECTIVE Cardiovascular lesions are the leading cause of morbidity and mortality in patients with Williams syndrome. Recent studies have rebutted conventional reports about the natural course of cardiovascular anomalies in Williams syndrome. DESIGN Retrospective study. SETTING Single tertiary center. PATIENTS Eighty patients with Williams syndrome followed up for more than 5 years. INTERVENTIONS Not applicable. OUTCOME MEASURES Long-term outcome of cardiovascular lesions, peak velocity change in obstructive cardiovascular lesions over time, post-interventional courses of disease-specific intervention, and intervention-free survival of obstructive cardiovascular lesions. RESULTS The median follow-up duration was 11.0 (5.1-28.3) years. Among 80 patients, supravalvular aortic stenosis (87.5%) was the most common cardiovascular lesion, followed by branch pulmonary stenosis (53.8%), mitral valve prolapse (22.5%), and aortic arch hypoplasia/coarctation (5.0%). During the follow-up period, the peak flow velocity of supravalvular aortic stenosis did not change on peak Doppler echocardiography. Initially, severe supravalvular aortic stenosis was aggravated (P

In Ebstein's anomaly, maximal expansion in the atrialized right ventricle (RV) occurs during early diastole, whereas that of the functional RV occurs in late diastole, resulting in diastolic dyssynchronous expansion (DSE). We quantitatively assessed DSE and identified preoperative factors correlated with persistent DSE after surgery. Seventeen patients diagnosed with Ebstein's anomaly in whom transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR) images available were retrospectively analyzed for quantitative DSE assessment and 10 patients who underwent surgery and postoperative TTE available were additionally analyzed for postoperative DSE. Severity of DSE was assessed by the time difference of maximal expansion between the atrialized and functional RV divided by the cardiac cycle length × 100 ("DSE index"). Relations between DSE and, clinical, electrophysiologic parameters and the severity of tricuspid valve (TV) tethering (the RV length / tethering height during diastole: "Tethering index") were assessed. In total patients, median DSE index and tethering index were 30.3 and 2.1 respectively, and the DSE index was correlated with tethering index (rs = 0.664, P = 0.004). In 10 patients who underwent surgery, this association remained after surgery and at 2-year follow up. Tethering index ≥2.5 separated patients with and without persistent DSE. In conclusion, DSE exists in Ebstein's anomaly. DSE index is related to the tethering index and DSE persists postoperatively if tethering index ≥ 2.5. As the persistent DSE might possibly impede the optimal recovery of RV function after surgery, severity of TV tethering should take into account in considering surgery.

Introduction Postoperative pancreatic fistula (POPF) is one of the most critical complications following pancreatic surgery. The aim of this study was to evaluate selective use of prophylactic octreotide for patients stratified by their individual risks of POPF. Methods From June 2019 to July 2020, a total of 243 patients underwent pancreatoduodenectomy with pancreatojejunostomy in Samsung Medical Center. Individual POPF risk scores were calculated with previously developed nomogram and clinicopathological data of the patients were retrospectively reviewed. Results There were 81 patients in low-risk group and 182 patients in high-risk group. No statistically significant differences were found in clinically relevant POPF (CR-POPF) rates between the control group and the octreotide group in both low- (3.0% vs. 7.1%, p = 0.439) and high- (23.6% vs. 16.1%, p = 0.206) risk groups. In risk factor analyses, postoperative use of octreotide was not an independent risk factor for CR-POPF in all (p = 0.897) patients, low- (p = 0.436), and high- (p = 0.614) risk group. Drain fluid amylase on the first postoperative day was significantly correlated with CR-POPF, regardless of individual risks. Conclusions Selective use of octreotide, even in high risk patients, showed no protective effect against POPF. Routine use of postoperative octreotide would not be recommended.

Introduction Given the rare incidence of the pancreatic acinar cell carcinoma (PACC), the post-resection clinical outcomes remains still unclear, and treatment strategies have relied on that of pancreatic ductal adenocarcinoma (PDAC). Methods The present study retrospectively investigated the clinicopathologic characteristics of resected PACC of 54 patients registered in the Korea Tumor Registry System Biliary Pancreas (KOTUS-BP) database between March 2003 and March 2020. Results Among 54 patients, with a mean age of 59 years and a male predominance (81.5%), there were 38 pure PACC (70.4%), five ductal differentiations (9.3%), seven mixed neuroendocrine carcinomas (13.0%), and four intraductal and papillary variants (7.4%). A mean tumor size was 4.6 cm consisting of seven at T1, 25 at T2, and 22 at T3 stages. Metastasis to regional lymph node was identified in 13 patients (24.1%). Twenty-eight patients (51.9%) received adjuvant chemotherapy (5-fluorouracil-based [n = 15]; gemcitabine-based [n = 13]). The 5-year survival rate was 54.6% with a median survival of 78.8 months. In survival comparison according to the stages of American Joint Committee on Cancer system, N stage (lymph node metastasis) showed significant differences (p = 0.008), but T stage did not derive any significant difference. Conclusions The resected PACC appears to have clinical outcomes distinct from the PDAC in this nation-wide study. Therefore, large-scale multinational studies are needed to overcome the rarity of PACC and to establish appropriate treatment strategies and staging system.

Introduction Pancreatic ductal adenocarcinoma (PDAC) is a notorious disease famous for its high recurrence rate even after surgical resection with curative intent. The characteristics of the post-resection recurrence, which include patterns, location, timing and related factors, have not been clearly identified in previous studies. Methods We retrospectively analyzed medical data of the patients who underwent surgical resection with curative intent for PDAC between January 2007 and December 2016. The recurrence was defined as presence of either radiological or histological evidence, and the postoperative 1 year as a value differentiating between early and late recurrence groups. Results Among 732 patients, 555 patients (75.8%) developed recurrence during the follow-up period. The 5-year overall survival rate and recurrence free survival of all patients was 28.9% and 19%, respectively. Three hundred and fifty-eight patients (64.5%) reccurred within the first year after surgery. Prognostic factors of early recurrence by univariate logistic regression analysis were significantly associated elevated CA-19-9 (p = 0.004), poorly differentiated tumor (p < 0.001), LN metastasis (p = 0.025) and preoperative tumor location (body to tail, p = 0.025). In the early recurrence group, metastasis occurred more in liver (n = 116, 30%) and in the late recurrence group, metastasis occurred more in lung (n = 37, 21.8%) and local recurrence (n = 64, 37.6%). Conclusions We have identified the characteristics of the post-resection recurrence. Further study to predict patients with high probability of early recurrence will help to establish individualized treatment.

Background/Aims: Immunoglobulin A nephropathy (IgAN) is a generally progressive disease, even in patients with favorable prognostic features. In this study, we aimed to investigate the antiproteinuric effect and tolerability of low-dose valsartan (an angiotensin II receptor blocker) therapy in normotensive IgAN patients with minimal proteinuria of less than 0.5 to 1.0 g/day. Methods: Normotensive IgAN patients, who had persistent proteinuria with a spot urine protein-to-creatinine ratio of 0.3 to 1.0 mg/mg creatinine, were recruited from five hospitals and randomly assigned to either 40 mg of valsartan as the low-dose group or 80 mg of valsartan as the regular-dose group. Clinical and laboratory data were collected at baseline, and at 4, 8, 12, and 24 weeks after valsartan therapy. Results: Forty-three patients (low-dose group, n = 23; regular-dose group, n = 20) were enrolled in the study. Proteinuria decreased significantly not only in the regular-dose group but also in the low-dose group. The change in urine protein-to-creatinine ratio at week 24 was −41.3% ± 26.1% (p < 0.001) in the regular-dose group and −21.1% ± 45.1% (p = 0.005) in the low-dose group. In the low-dose group, blood pressure was constant throughout the study period, and there was no symptomatic hypotension. In the regular-dose group, blood pressure decreased at weeks 8 and 12. No significant change in glomerular filtration rate, serum creatinine level, or serum potassium level was observed during the study period. Conclusions: Our results suggest that low-dose valsartan can significantly reduce proteinuria without causing any intolerability in normotensive IgAN patients with minimal proteinuria.

Several studies have reported short-term results for post-cholecystectomy symptoms and quality of life (QoL). However, reports on long-term results are still limited. This study aimed to identify risk factors affecting short- and long-term patient-reported outcome (PRO) following laparoscopic cholecystectomy.From 2016 to 2017, a total of 476 patients from 5 institutions were enrolled. PRO was examined using the Numeric Rating Scale (NRS) pain score and the Gastrointestinal (GI) QoL Index questionnaire at postoperative 1 month and 1 year.Most of patients recovered well at postoperative 1 year compared to postoperative 1 month for the NRS pain score, QoL score, and GI symptoms. A high operative difficulty score (HR 1.740, P = .031) and pathology of acute or complicated cholecystitis (HR 1.524, P = .048) were identified as independent risk factors for high NRS pain scores at postoperative 1 month. Similarly, female sex (HR 1.571, P = .003) at postoperative 1 month and postoperative complications (HR 5.567, P = .001) at postoperative 1 year were independent risk factors for a low QoL. Also, age above 50 (HR 1.842, P = .001), female sex (HR 1.531, P = .006), and preoperative gallbladder drainage (HR 3.086, P = .001) were identified as independent risk factors for GI symptoms at postoperative 1 month.Most patients showed improved long-term PRO measurement in terms of pain, QoL, and GI symptoms. There were no independent risk factors for long-term postoperative pain and GI symptoms. However, postoperative complications were identified to affect QoL adversely at postoperative 1 year. Careful and long-term follow up is thus necessary for patients who experienced postoperative complications.

No studies have yet analyzed the characteristics of recurrence after resection for intraductal papillary neoplasm of bile duct (IPNB) based on tumor location. We analyzed the patterns, timing, and risk factors for recurrence. From 1994 to 2014, data from 103 patients who were diagnosed with IPNB were retrospectively reviewed. Among these, 44 were extrahepatic IPNB (E-IPNB) and 59 were intrahepatic IPNB (I-IPNB). CK20, pancreaticobiliary type, tumor invasion beyond ductal wall, tumor invasion to adjacent organs, and invasive disease were more frequently found in E-IPNB than in I-IPNB (22.7 vs. 8.5%; p = 0.043, 38.6 vs. 23.7%; p = 0.050, 20.5 vs. 11.9%; p

Background There is no consensus on the optimal treatment of T1b gallbladder cancer (GBC) due to the lack of evidence and the difficulty of anatomy and pathological standardization. Methods A total of 272 patients with T1b GBC who underwent surgical resection at 14 centers with specialized hepatobiliary-pancreatic surgeons and pathologists in Korea, Japan, Chile, and the United States were studied. Clinical outcomes including disease-specific survival (DSS) rates according to the types of surgery were analyzed. Results After excluding patients, the 237 qualifying patients consisted of 90 men and 147 women. Simple cholecystectomy (SC) was performed in 116 patients (48.9%) and extended cholecystectomy (EC) in 121 patients (51.1%). The overall 5-year DSS was 94.6%, and it was similar between SC and EC patients (93.7% vs. 95.5%, P = 0.496). The 5-year DSS was similar between SC and EC patients in America (82.3% vs. 100.0%, P = 0.249) as well as in Asia (98.6% vs. 95.2%, P = 0.690). The 5-year DSS also did not differ according to lymph node metastasis (P = 0.688) or tumor location (P = 0.474). Conclusions SC showed similar clinical outcomes (including recurrence) and survival outcomes as EC; therefore, EC is not needed for the treatment of T1b GBC.

Background Open surgical implantation of epicardial leads in neonates and infants remains the first option of treatment. We reviewed the long-term outcomes after epicardial pacemaker implantation in neonates and infants. Methods From 1989 to 2016, 48 patients (16 neonates) underwent pacemaker implantation within the first year of life. Their median age and weight were 66.5 days (range: 0∼319 days), and 4.2 kg (range: 1.9∼9.3 kg), respectively, at the time of first pacemaker implantation. The indications for pacemaker implantation were postoperative or congenital atrioventricular block, sinus node dysfunction, and/or myocarditis-induced atrioventricular block. Forty-six (46) unipolar epicardial leads (non-steroid-eluting: 22; steroid-eluting: 24) and two bipolar leads (steroid-eluting) were inserted using a median sternotomy or subxiphoid approach. Results The mean follow-up duration was 8.5 ± 7.9 years. The most commonly used generator mode at first implantation was VVI (n = 24, 50.0%). Eleven (11) generator mode changes from the initial VVI or VVIR to dual-chamber pacing were made at a mean of 7.0 ± 6.2 years after the first implantation for better inter-chamber synchrony and ventricular function. Freedom from reoperation for generator change after the first implantation was 95.3, 70.6, and 21.9% at 1, 5, and 10 years. Eighteen (18) lead malfunction events (34.1%) were detected. Freedom from reoperation for lead change was 97.8, 76.2, and 46.3% at 1, 5, and 10 years. The lead replacement rate was significantly higher in patients with non-steroid-eluting than steroid-eluting leads (p = 0.045). Conclusions Neonates and infants require more frequent changes in pacemaker generator and leads than the older population. The use of steroid-eluting leads increased lead longevity and reduced the need for surgical re-interventions.

Background The aim of this multicenter study was to evaluate the safety and efficacy of tolvaptan (TLV) in Korean patients with the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Methods Of 51 enrolled patients with SIADH, 39 patients (16 female patients, aged 70.8 ± 11.3 years) were included in an intention to treat analysis. All patients received 15 mg/day as the initial dose, and the dose was then increased up to 60 mg/day (as needed) until day 4. Results Serum sodium increased significantly from baseline during the first 24 hours (126.8 ± 4.3 vs. 133.7 ± 3.8 mmol/L, P < 0.001), rose gradually between days 1 and 4 (133.7 ± 3.8 vs. 135.6 ± 3.6 mmol/L, P < 0.05), and then plateaued until day 11 (136.7 ± 4.5 mmol/L). The correlation between the change in serum sodium for the first 24 hours and initial serum sodium concentration was significant (r = −0.602, P < 0.001). In severe hyponatremia (< 125 mmol/L), the change was significantly higher (11.1 ± 4.8 mmol/L) than in moderate (6.4 ± 2.5 mmol/L, P < 0.05) or mild hyponatremia (4.3 ± 3.3 mmol/L, P < 0.01). In addition, logistic regression analysis showed that body weight (odds ratio [OR], 0.858; 95% confidence interval [CI], 0.775–0.976; P = 0.020) and body mass index (BMI) (OR, 0.692; 95% CI, 0.500–0.956; P = 0.026) were associated with rapid correction. No serious adverse events were reported, but in 13% of patients hyponatremia was overcorrected. Conclusion TLV is effective in correcting hyponatremia and well-tolerated in Korean patients with SIADH. However, those with low body weight, low BMI or severe hyponatremia, could be vulnerable to overcorrection with the initial dose of 15 mg TLV., Graphical Abstract

Twenty-six-year-old Ebstein's anomaly patient, who had failed both biventricular and one-and-a-half repair, underwent right ventricle exclusion and Fontan operation. She completed pregnancy and delivery owing to the excellent long-term clinical course. Although the caesarean section was performed due to symptoms of heart failure on the gestational age of 32+6 weeks, preterm delivery was agreeable with neonatology support. In Korea, there has not yet been a case of pregnancy and delivery of functional single ventricle patient because most patients have been discouraged from getting pregnant. However, functional single ventricle patient can endure pregnancy and delivery, if valve function and ventricular contractility, status of Fontan pathway and absence of arrhythmia predict favorable outcome as presented in this case. Although the patient maintained her pregnancy without anticoagulation owing to laminar flow in the Fontan pathway and absence of thromboembolic event, anticoagulation should be considered, weighing the benefits and risks during the pregnancy.

The Korean Society of Nephrology (KSN) launched a nationwide official survey program about dialysis therapy in 1985. Nowadays, the accumulated data for 30 years by this “Insan Prof. Min Memorial end-stage renal disease (ESRD) Registry” program have been providing the essential information for dialysis clinical practice, academic nephrology research, and health management policy. We reviewed 30 years of data to identify important changes and implications for the future improvement of dialysis therapy in Korea.Hemodialysis patients, especially diabetics and elderly patients have increased in number very rapidly during recent years in Korea. The Korean prevalence rate of ESRD patients was about 70% of the United States and about 50% of Japan according to the international comparisons in the annual data report of United States Renal Data System. The blood pressure control, anemia control, and dialysis adequacy have continuously improved year by year. The importance of calcium and phosphorus control has also been increasing because of the increase in long-term dialysis patients. In addition, chronic dialysis complications should be closely monitored and dialysis modifications, such as hemodiafiltration therapy, might be considered. Because of the increase of private clinics and nursing hospitals in dialysis practice, the role of dialysis specialists and continuing education are thought to be essential. For strict cost-effective dialysis control of increasing elderly, diabetic, and long-term dialysis patients, the KSN ESRD patient registration should be run by the KSN and health ministry in cooperation, in which the dialysis fee reimbursement should be accompanied.

Prolonged hypokalemia from chronic laxative abuse is recognized as the cause of chronic tubulointerstitial disease, known as “hypokalemic nephropathy,” but it is not clear whether it contributes to acute kidney injury (AKI). A 42-year-old woman with a history of chronic kidney disease as a result of chronic laxative abuse from a purging type of anorexia nervosa (AN-P), developed an anuric AKI requiring hemodialysis and a mild AKI 2 months later. Both episodes of AKI involved severe to moderate hypokalemia (1.2 and 2.7mmol/L, respectively), volume depletion, and mild rhabdomyolysis. The histologic findings of the first AKI revealed the remnants of acute tubular necrosis with advanced chronic tubulointerstitial nephritis and ischemic glomerular injury. Along with these observations, the intertwined relationship among precipitants of recurrent AKI in AN-P is discussed, and then we postulate a contributory role of hypokalemia involved in the pathophysiology of the renal ischemia-induced AKI.

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).