Objective: Research on fetal mortality due to congenital anomalies is insufficient, particularly that utilizing data specific to South Korea. Thus, we aimed to investigate the characteristics and risk factors for fetal death due to congenital anomalies in Korea. Methods: Fetal deaths registered from 2009-2020 with Statistics Korea were assessed. Fetal charac teristics included gestational age, body weight, sex, and multiple fetuses, while maternal characteri stics included age, educational level, nationality, and place of residence. Risk factors for fetal death were analyzed using simple comparison and logistic regression. Changes in fetal mortality by year were examined using Poisson regression analysis. Results: A total of 37,928 fetal deaths occurred, among which 3,758 were classified as congenital anomaly, 710 as non-congenital anomaly, and 33,460 as unknown cause. Fetal mortality for gesta tional weeks 20 to 27 and ≥28 were 75.3% and 24.7%, respectively. The proportion of congenital anomalies among fetal deaths during these gestational age periods is 11.3% and 5.8%. Multiple fetuses, maternal age of <20 years or ≥40 years were identified as risk factors for fetal death due to congenital anomalies. Among the top 30 causes, covering 97.5% of all deaths, unspecified causes were 88.2%, congenital malformations 8.2%, and other causes 2.0%, respectively. Fetal mortality and deaths from congenital anomalies exhibited downward trends. Conclusion Fetal deaths due to congenital anomalies showed a decreasing trend, but the risks, such as multiple fetuses and advanced maternal age are increasing in Korea. Therefore, careful monitoring of fetal deaths due to congenital anomalies are essential.

Purpose: This study aimed to investigate whether the serum extracellular newly identified receptor for advanced glycation end products binding protein (EN-RAGE) and the soluble form of RAGE (sRAGE) measured at diagnosis are associated with all-cause mortality in patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). Materials and Methods: Serum EN-RAGE and sRAGE were measured in 75 immunosuppressive drug-naïve MPA and GPA patients using an immunoassay, with their clinical and laboratory data reviewed. The optimal cut-off point of EN-RAGE and sRAGE was calculated by finding the threshold with the maximum sum of sensitivity and specificity. In addition, the least absolute shrinkage and selection operator regression was adopted to select variables included in the multivariable Cox proportional hazards (PH) regression model. Results: The median age of the patients was 67.0 years, and 34% were male. Neither serum EN-RAGE nor sRAGE at diagnosis was correlated with the Birmingham Vasculitis Activity Score. Furthermore, no correlation was observed between serum EN-RAGE and sRAGE. Deceased patients had significantly lower serum EN-RAGE and higher serum sRAGE at diagnosis compared to surviving patients. Patients with serum EN-RAGE at diagnosis ≤84.37 ng/mL and serum sRAGE at diagnosis ≥1.82 ng/mL showed significantly lower survival probabilities compared to those without. In multivariable Cox PH regression model, only serum sRAGE at diagnosis ≥1.82 ng/mL, rather than serum EN-RAGE at diagnosis ≤84.37 ng/mL, was independently associated with all-cause mortality (hazard ratio 7.094). Conclusion This study is the first to demonstrate that serum sRAGE at diagnosis may independently predict all-cause mortality during follow-up in patients with MPA and GPA.

Background and Objectives: This study aimed to analyze the outcomes of Fontan surgery in the Republic of Korea, as there were only a few studies from Asian countries. Methods: The medical records of 1,732 patients who underwent Fontan surgery in 10 cardiac centers were reviewed. Results: Among them, 1,040 (58.8%) were men. The mean age at Fontan surgery was 4.3±4.2 years, and 395 (22.8%) patients presented with heterotaxy syndrome. According to the types of Fontan surgery, 157 patients underwent atriopulmonary (AP) type; 303, lateral tunnel (LT) type; and 1,266, extracardiac conduit (ECC) type. The overall survival rates were 91.7%, 87.1%, and 74.4% at 10, 20, and 30 years, respectively. The risk factors of early mortality were male, heterotaxy syndrome, AP-type Fontan surgery, high mean pulmonary artery pressure (mPAP) in pre-Fontan cardiac catheterization, and early Fontan surgery year. The risk factors of late mortality were heterotaxy syndrome, genetic disorder, significant atrioventricular valve regurgitation (AVVR) before Fontan surgery, high mPAP in pre-Fontan cardiac catheterization, and no fenestration. Conclusions In Asian population with a high incidence of heterotaxy syndrome, the heterotaxy syndrome was identified as the poor prognostic factors for Fontan surgery. The preoperative low mPAP and less AVVR are associated with better early and long-term outcomes of Fontan surgery.

Objective: Research on fetal mortality due to congenital anomalies is insufficient, particularly that utilizing data specific to South Korea. Thus, we aimed to investigate the characteristics and risk factors for fetal death due to congenital anomalies in Korea. Methods: Fetal deaths registered from 2009-2020 with Statistics Korea were assessed. Fetal charac teristics included gestational age, body weight, sex, and multiple fetuses, while maternal characteri stics included age, educational level, nationality, and place of residence. Risk factors for fetal death were analyzed using simple comparison and logistic regression. Changes in fetal mortality by year were examined using Poisson regression analysis. Results: A total of 37,928 fetal deaths occurred, among which 3,758 were classified as congenital anomaly, 710 as non-congenital anomaly, and 33,460 as unknown cause. Fetal mortality for gesta tional weeks 20 to 27 and ≥28 were 75.3% and 24.7%, respectively. The proportion of congenital anomalies among fetal deaths during these gestational age periods is 11.3% and 5.8%. Multiple fetuses, maternal age of <20 years or ≥40 years were identified as risk factors for fetal death due to congenital anomalies. Among the top 30 causes, covering 97.5% of all deaths, unspecified causes were 88.2%, congenital malformations 8.2%, and other causes 2.0%, respectively. Fetal mortality and deaths from congenital anomalies exhibited downward trends. Conclusion Fetal deaths due to congenital anomalies showed a decreasing trend, but the risks, such as multiple fetuses and advanced maternal age are increasing in Korea. Therefore, careful monitoring of fetal deaths due to congenital anomalies are essential.

Purpose: This study aimed to investigate whether the serum extracellular newly identified receptor for advanced glycation end products binding protein (EN-RAGE) and the soluble form of RAGE (sRAGE) measured at diagnosis are associated with all-cause mortality in patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). Materials and Methods: Serum EN-RAGE and sRAGE were measured in 75 immunosuppressive drug-naïve MPA and GPA patients using an immunoassay, with their clinical and laboratory data reviewed. The optimal cut-off point of EN-RAGE and sRAGE was calculated by finding the threshold with the maximum sum of sensitivity and specificity. In addition, the least absolute shrinkage and selection operator regression was adopted to select variables included in the multivariable Cox proportional hazards (PH) regression model. Results: The median age of the patients was 67.0 years, and 34% were male. Neither serum EN-RAGE nor sRAGE at diagnosis was correlated with the Birmingham Vasculitis Activity Score. Furthermore, no correlation was observed between serum EN-RAGE and sRAGE. Deceased patients had significantly lower serum EN-RAGE and higher serum sRAGE at diagnosis compared to surviving patients. Patients with serum EN-RAGE at diagnosis ≤84.37 ng/mL and serum sRAGE at diagnosis ≥1.82 ng/mL showed significantly lower survival probabilities compared to those without. In multivariable Cox PH regression model, only serum sRAGE at diagnosis ≥1.82 ng/mL, rather than serum EN-RAGE at diagnosis ≤84.37 ng/mL, was independently associated with all-cause mortality (hazard ratio 7.094). Conclusion This study is the first to demonstrate that serum sRAGE at diagnosis may independently predict all-cause mortality during follow-up in patients with MPA and GPA.

Objective: Research on fetal mortality due to congenital anomalies is insufficient, particularly that utilizing data specific to South Korea. Thus, we aimed to investigate the characteristics and risk factors for fetal death due to congenital anomalies in Korea. Methods: Fetal deaths registered from 2009-2020 with Statistics Korea were assessed. Fetal charac teristics included gestational age, body weight, sex, and multiple fetuses, while maternal characteri stics included age, educational level, nationality, and place of residence. Risk factors for fetal death were analyzed using simple comparison and logistic regression. Changes in fetal mortality by year were examined using Poisson regression analysis. Results: A total of 37,928 fetal deaths occurred, among which 3,758 were classified as congenital anomaly, 710 as non-congenital anomaly, and 33,460 as unknown cause. Fetal mortality for gesta tional weeks 20 to 27 and ≥28 were 75.3% and 24.7%, respectively. The proportion of congenital anomalies among fetal deaths during these gestational age periods is 11.3% and 5.8%. Multiple fetuses, maternal age of <20 years or ≥40 years were identified as risk factors for fetal death due to congenital anomalies. Among the top 30 causes, covering 97.5% of all deaths, unspecified causes were 88.2%, congenital malformations 8.2%, and other causes 2.0%, respectively. Fetal mortality and deaths from congenital anomalies exhibited downward trends. Conclusion Fetal deaths due to congenital anomalies showed a decreasing trend, but the risks, such as multiple fetuses and advanced maternal age are increasing in Korea. Therefore, careful monitoring of fetal deaths due to congenital anomalies are essential.