A comparative study using gastric ultrasound to evaluate the safety of shortening the fasting time before pediatric echocardiography: a randomized controlled non-inferiority study.

PURPOSE: The objective of this study was to demonstrate that the gastric cross-sectional area (CSA) in the right lateral decubitus position (RLDP) during a 2-h fasting period is not larger than that during a conventional 4-h fasting period prior to pediatric echocardiography. METHODS: 93 patients aged under 3 years scheduled for echocardiography under sedation were enrolled and randomly allocated into two groups; 2-h fasting vs 4-h fasting. For group 4 h (n = 46), the patients were asked to be fasted for all types of liquid for more than 4 h, while group 2 h (n = 47) were asked to be fasted for all types of liquid for 2 h before echocardiography. Gastric ultrasound was performed before echocardiography, and CSARLDP was measured. We compared CSARLDP, incidence of at-risk stomach, fasting duration, and the incidence of major (pulmonary aspiration, aspiration pneumonia) and minor complications (nausea, retching, and vomiting, apnea, and bradycardia) between two groups. RESULTS: The mean difference of CSARLDP (group 2 h-group 4 h) was 0.49 (- 0.18 to 1.17) cm2, and it was within the non-inferiority margin (Δ = 2.1 cm2). There was no difference in the incidence of at-risk stomach (P = 0.514). There was no significant difference in the incidence of major and minor complications between the two groups. CONCLUSION: Two-hour fasting in pediatric patients who need an echocardiography did not increase major and minor complications and CSA significantly.

Single-cell and spatially resolved interactomics of tooth-associated keratinocytes in periodontitis.

Periodontitis affects billions of people worldwide. To address relationships of periodontal niche cell types and microbes in periodontitis, we generated an integrated single-cell RNA sequencing (scRNAseq) atlas of human periodontium (34-sample, 105918-cell), including sulcular and junctional keratinocytes (SK/JKs). SK/JKs displayed altered differentiation states and were enriched for effector cytokines in periodontitis. Single-cell metagenomics revealed 37 bacterial species with cell-specific tropism. Fluorescence in situ hybridization detected intracellular 16 S and mRNA signals of multiple species and correlated with SK/JK proinflammatory phenotypes in situ. Cell-cell communication analysis predicted keratinocyte-specific innate and adaptive immune interactions. Highly multiplexed immunofluorescence (33-antibody) revealed peri-epithelial immune foci, with innate cells often spatially constrained around JKs. Spatial phenotyping revealed immunosuppressed JK-microniches and SK-localized tertiary lymphoid structures in periodontitis. Here, we demonstrate impacts on and predicted interactomics of SK and JK cells in health and periodontitis, which requires further investigation to support precision periodontal interventions in states of chronic inflammation.

Treatment of Primary Cardiac Tumors in Children: A Single Center 18-Year Experience.

This study aimed to compare the clinical characteristics and courses of pediatric patients with cardiac tumors in nonoperative and operative groups to help guide treatment decisions. We reviewed the medical records of patients diagnosed with primary pediatric cardiac tumors at our institution between 2003 and 2020. Demographic data, clinical characteristics, and follow-up data between the operation and nonoperation groups were compared. A total of 56 patients were included in the study. Thirteen patients underwent surgery. The median age was 1.4 months (range, 1 to 18 years). The patients in the operation group had more frequent symptoms or signs, such as desaturation, respiratory difficulty, murmur, a higher mass area/chamber area (MC) ratio, decreased ventricular contractility, and significant ventricular outflow tract obstruction (VOTO). An MC ratio of 0.568 was the cutoff value for differentiating patients with symptoms or signs of heart failure and decreased ventricular contractility. At the last follow-up, all patients had good ventricular contractility except one patient in the operative group with fibroma. In the non-operative group, rhabdomyomas often regressed spontaneously, while fibromas often increased in size. Two patients in the nonoperative group died. In the operative group, there was no early or late mortality or tumor recurrence. In this study, patients had good outcomes with or without surgery, even when the tumor was large, or surgery was performed in early infancy.

The long-term effects of the fenestration in patients with extracardiac Fontan circulation-a multicenter Korean cohort study based on national Fontan registry.

Introduction: The long-term effects of fenestration in patients with Fontan circulation remain unclear. We aim to evaluate the fenestration impact on early and late outcomes in patients with extracardiac Fontan (ECF) using a propensity score matching analysis. Methods: We performed an extensive retrospective multicenter clinical data review of the Korean Fontan registry and included 1,233 patients with surgical ECF (779 fenestrated, 454 non-fenestrated). Demographics, baseline, and follow-up data were collected and comprehensively analyzed. Patients were divided into two groups according to the baseline presence or absence of surgical fenestration. Subsequently, patients were sub-divided according to the fenestration status at the last follow-up. Propensity-score matching was performed to account for collected data between the 2 groups using a multistep approach. The primary outcomes were survival and freedom from Fontan failure (FFF). We also looked at postoperative hemodynamics, cardiopulmonary exercise test results, oxygen saturations, and functional status. Results: After propensity-score matching (454 matched pairs), there was no difference in survival or FFF between the 2 groups. However, ECF patients with baseline fenestration had significantly lower oxygen saturation (p = 0.001) and lower functional status (p < 0.001). Patients with fenestration had significantly longer bypass times, higher postoperative central venous pressure, higher postoperative left atrial pressure, and less prolonged pleural effusion in the early postoperative period. The propensity score matching according to the fenestration status at the last follow-up (148 matched pairs) showed that patients with a persistent fenestration had significantly lower oxygen saturation levels (p < 0.001). However there were no intergroup differences in the functional status, survival and FFF. Conclusions: Our results showed no long-term benefits of the Fenestration in terms of survival and FFF. Patients with persistent fenestration showed oxygen desaturation but no difference in exercise intolerance was shown between the 2 groups.

Should I speak up? How trust in leaders and leader-leader exchanges influence nurses' voice behaviour.

AIM: Discussing the nurses' voice behaviour could support the managers in making the right decisions and solve problems. DESIGN: This was a discursive paper. METHODS: The discursive was based on reviewing the literature. RESULTS: Nurses play a critical role in offering useful constructive advice, which leads to management figuring out and solving problems immediately for the purpose of bettering the working environment. Therefore, we assert that trust in leadership and the leader-leader exchange system also plays a critical role in enforcing voice behaviour. Trust is a crucial aspect of voice behaviour, and integrated trust in leadership and leader-leader exchange as a possible practical suggestion for the fostering of voice behaviour are proposed. Nurse managers must maintain a sense of reciprocal moral obligation in order to nurture value-driven voice behaviour. It is important that open dialogue, active listening and trust in leadership exist. Nurse managers must consider ways to foster mutual trust, and support and enable nurses to use voice behaviour in everyday practice.

Long-Term Outcomes of Modified Cone Reconstruction for Ebstein's Anomaly in Pediatric Patients in a Single Center.

BACKGROUND AND OBJECTIVE: We aimed to investigate long-term clinical and echocardiographic outcomes, including tricuspid valve durability, annular growth, and left ventricular reverse remodeling, after modified cone reconstruction in patients with Ebstein's anomaly. METHODS: This was a retrospective analysis of all pediatric patients who underwent modified cone reconstruction for Ebstein's anomaly at a single tertiary center between January 2005 and June 2021. RESULTS: A total of 14 pediatric patients underwent modified cone reconstruction for Ebstein's anomaly; the median age was 5.8 years (range, 0.01-16.6). There were three patients (21.4%) with Carpentier type B, ten patients with Carpentier type C (71.4%), and one patient with Carpentier type D (7.1%). There was no early or late mortality, arrhythmia, or readmission for heart failure at a 10-year follow-up. There were no cases of more than mild tricuspid stenosis or more than moderate tricuspid regurgitation during the study period, except for one patient with severe tricuspid regurgitation who underwent reoperation. The z value for tricuspid valve annular size significantly decreased immediately after the operation (2.46 vs. -1.15, p<0.001). However, from 1 year to 7 years after surgery, the z values were maintained between -1 and +1. Left ventricular end-systolic volume, end-diastolic volume, and stroke volume increased after surgery and remained elevated until seven years postoperatively. CONCLUSIONS: Ebstein's anomaly in children can be repaired by modified cone reconstruction with low mortality and morbidity, good tricuspid valve durability, and annular growth relative to somatic growth.

Reepithelialization of Diabetic Skin and Mucosal Wounds Is Rescued by Treatment With Epigenetic Inhibitors.

Wound healing is a complex, highly regulated process and is substantially disrupted by diabetes. We show here that human wound healing induces specific epigenetic changes that are exacerbated by diabetes in an animal model. We identified epigenetic changes and gene expression alterations that significantly reduce reepithelialization of skin and mucosal wounds in an in vivo model of diabetes, which were dramatically rescued in vivo by blocking these changes. We demonstrate that high glucose altered FOXO1-matrix metallopeptidase 9 (MMP9) promoter interactions through increased demethylation and reduced methylation of DNA at FOXO1 binding sites and also by promoting permissive histone-3 methylation. Mechanistically, high glucose promotes interaction between FOXO1 and RNA polymerase-II (Pol-II) to produce high expression of MMP9 that limits keratinocyte migration. The negative impact of diabetes on reepithelialization in vivo was blocked by specific DNA demethylase inhibitors in vivo and by blocking permissive histone-3 methylation, which rescues FOXO1-impaired keratinocyte migration. These studies point to novel treatment strategies for delayed wound healing in individuals with diabetes. They also indicate that FOXO1 activity can be altered by diabetes through epigenetic changes that may explain other diabetic complications linked to changes in diabetes-altered FOXO1-DNA interactions. ARTICLE HIGHLIGHTS: FOXO1 expression in keratinocytes is needed for normal wound healing. In contrast, FOXO1 expression interferes with the closure of diabetic wounds. Using matrix metallopeptidase 9 as a model system, we found that high glucose significantly increased FOXO1-matrix metallopeptidase 9 interactions via increased DNA demethylation, reduced DNA methylation, and increased permissive histone-3 methylation in vitro. Inhibitors of DNA demethylation and permissive histone-3 methylation improved the migration of keratinocytes exposed to high glucose in vitro and the closure of diabetic skin and mucosal wounds in vivo. Inhibition of epigenetic enzymes that alter FOXO1-induced gene expression dramatically improves diabetic healing and may apply to other conditions where FOXO1 has a detrimental role in diabetic complications.

Prognosis of Chronic Kidney Disease and Metabolic Syndrome in Adults With Congenital Heart Disease.

BACKGROUND: Few studies have examined the incidence of chronic kidney disease (CKD) and metabolic syndrome (MS) and their combined prognostic effects in adult congenital heart disease (ACHD). Our aims were to identify the incidence and prognostic implications of CKD and MS in ACHD. METHODS: This is retrospective cohort study. We included 2,462 ACHD ≥ 20 years of age who were treated at a tertiary hospital in Korea from 2006 to 2018. CKD was defined as an estimated glomerular filtration rate < 60 mL/min/1.73m². MS was diagnosed based on the presence of abnormal metabolic parameters: blood sugar level, obesity, dyslipidemia, and hypertension. The primary outcome was all-cause mortality from 2006 through 2019 using data from the Ministry of the Interior and Safety in Korea. RESULTS: The incidence of CKD and MS in ACHD was 7.6% and 35.9%, respectively. The coexistence rate of CKD and MS was 4.6%. Although MS was not independently associated with mortality in the multiple analysis (adjusted hazard ratio [aHR], 1.07; 95% confidence interval [CI], 0.79-1.46), it was closely related to the presence of CKD (adjusted odds ratio, 2.62; 95% CI, 1.89-3.63). ACHD patients with CKD had a significantly increased risk of mortality compared with those without CKD (aHR, 2.84; 95% CI, 2.00-4.04). CONCLUSIONS: In patients with ACHD, the distribution of MS is higher, and both MS and its components were associated with CKD. Given the CKD was independently associated with mortality, close monitoring and management of renal dysfunction and metabolic parameters in ACHD patients is needed.

Primed inflammatory response by fibroblast subset is necessary for proper oral and cutaneous wound healing.

Fibroblasts are ubiquitous mesenchymal cells that exhibit considerable molecular and functional heterogeneity. Besides maintaining stromal integrity, oral fibroblast subsets are thought to play an important role in host-microbe interaction during injury repair, which is not well explored in vivo. Here, we characterize a subset of fibroblast lineage labeled by paired-related homeobox-1 promoter activity (Prx1Cre+ ) in oral mucosa and skin and demonstrate these fibroblasts readily respond to microbial products to facilitate the normal wound healing process. Using a reporter mouse model, we determined that Prx1Cre+ fibroblasts had significantly higher expression of toll-like receptors 2 and 4 compared to other fibroblast populations. In addition, Prx1 immunopositive cells exhibited heightened activation of inflammatory transcription factor NF-κB during the early wound healing process. At the cytokine level, CXCL1 and CCL2 were significantly upregulated by Prx1Cre+ fibroblasts at baseline and upon LPS stimulation. Importantly, lineage-specific knockout to prevent NF-κB activation in Prx1Cre+ fibroblasts drastically impaired both oral and skin wound healing processes, which was linked to reduced macrophage infiltration, failure to resolve inflammation, and clearance of bacteria. Together, our data implicate a pro-healing role of Prx1-lineage fibroblasts by facilitating early macrophage recruitment and bacterial clearance.

Valve-sparing root replacement in children with connective tissue disease: Long-term risk of aortic events.

BACKGROUND: Cardiovascular involvement in pediatric patients with connective tissue disease (CTD) is life-threatening, with aortic root dilatation being the most prevalent cardiovascular abnormality. We attempted to determine long-term outcomes of valve-sparing root replacement (VSRR) in this group, including cardiovascular reoperations for aortic aneurysm and dissection. METHODS: We conducted a retrospective analysis of pediatric patients with CTD who received VSRR in a single center from 2002 to 2021. The primary end point was a composite event of all-cause death and cardiovascular reoperations. The median follow-up duration was 8.3 years, with a maximum of 20.7 years. RESULTS: The median age of 24 pediatric patients who had VSRR was 14.4 years. Marfan syndrome and Loeys-Dietz syndrome affected 19 (79.2%) and 5 (20.8%) patients, respectively. There was no early death. The 15-year survival rate was 91.7%. At 10 years after VSRR, the cumulative incidence of reoperation for aortic regurgitation was 15.6%, and for aortic aneurysm or dissection, it was 29.1%. The 10-year rate of freedom from the primary end point was 53.1%. The Cox multivariable analysis revealed younger age at surgery (hazard ratio, 1.279; 95% confidence interval, 1.086-1.505; P = .003) and VSRR before 13 years of age (hazard ratio, 5.005; 95% confidence interval, 1.146-21.850; P = .032) as independent prognostic factors for the primary endpoint. CONCLUSIONS: VSRR for aortic root dilatation in pediatric patients with CTD demonstrated good long-term survival and low reoperation rates for aortic regurgitation. However, several patients developed later aortic aneurysm or dissection, and careful surveillance may be required, particularly in those who received VSRR at younger age.

Subzero saline chilling with or without prechilling in icy water improved chilling efficiency and meat tenderness of broiler carcasses.

Freshly slaughtered carcasses need to be chilled to improve product quality, meat safety, and processing efficiency. This research investigated the effect of subzero saline chilling (SSC) on broiler carcasses with or without prechilling in icy water. Water immersion chilling at 0.5°C (WIC) or SSC at 4% NaCl/-2.41°C (SSC) was a major chilling step. For the combination of pre- and postchilling, the warm water immersion chilling (WWIC) at 10°C was used as prechilling and the WIC as postchilling (WWIC-WIC), and WIC was used as prechilling and the SSC as postchilling (WIC-SSC). The internal temperature of breast fillets was monitored during chilling. Carcasses in a prechiller were transported to a postchiller when their internal temperature reached 15°C. Chilling was completed when the carcass temperature reached 4.4°C or below, and breast fillets were harvested at 3-h postmortem to measure the pH and sarcomere length. Color (L*, a*, and b*) values were evaluated on both breast skin and skinless breast surfaces. Meat tenderness was evaluated using the breast fillets after overnight storage and cooking to an internal temperature of 76°C. The carcasses in the SSC and WIC-SSC showed shorter chilling times (85-91 min) than those (100-144 min) of WIC and WWIC-WIC. A higher chilling yield was observed for the carcasses in WIC-SSC, and a lower cooking yield was seen for the carcasses in WWIC-WIC than other chilling methods (P < 0.05). The breast fillets of broilers in the SSC and WIC-SSC showed lower shear forces and longer sarcomere length than the WIC and WWIC-WIC. No difference was found for L* and a* values, while lower b* value was observed in the SSC than the other chilling methods (P < 0.05). Based on these results, chilling of broiler carcasses in the SSC (4% NaCl/-2.41°C) with or without prechilling in WIC at 0.5°C significantly improved chilling efficiency and meat tenderness, with minor color changes on carcasses.

Effects of time of bed rest on vascular complications after cardiac catheterization in pediatric patients with congenital heart disease: A randomized controlled trial.

BACKGROUND: Pediatric cardiac catheterization, which is performed by accessing the femoral vessel, requires immobilization and bed rest for 4-6 h to prevent vascular complications. Studies in adults suggest that the immobilization time for the same access can be safely reduced to approximately 2 h after catheterization. However, it is unclear whether bed-rest time can be safely decreased after catheterization in children. OBJECTIVE: To assess the effects of bed-rest duration on bleeding, vascular complications, pain level, and the use of additional sedatives after transfemoral cardiac catheterization in children with congenital heart disease. METHODS: This study was an open-label, randomized, controlled, posttest-only design, including 86 children who underwent cardiac catheterization. Children were allocated to receive either 2 h of bed rest (n = 42) in the experimental group or 4 h of bed rest (n = 42) in the control group following catheterization. RESULTS: The mean age of children was 3.93 (±3.82) years in the experimental group and 5.63 (±3.97) years in the control group. There was no difference in site bleeding incidence (P = 0.214), vascular complication score (P = 0.082), pain level (P = 0.445), or additional sedation use (P = 1.000) between the two groups. CONCLUSIONS: There were no significant hemostatic complications after 2 h of bed rest following pediatric catheterization; therefore, 2 h of bed rest was as safe as 4 h of bed rest. (Trial registration: KCT0007737).

The effects of music intervention on anxiety and stress responses in adults with CHD undergoing cardiac catheterisation.

INTRODUCTION: This study evaluated the effect of music intervention on the anxiety and stress responses of patients who underwent an interventional cardiac catheterisation. METHODS: The study design was a pre- and post-test randomised controlled trial that included 94 patients who underwent a transcatheter atrial septal defect closure. Patients were allocated to receive either music intervention (n = 47) or usual care (n = 47) during the interventional cardiac catheterisation. Music intervention effectiveness was examined in terms of anxiety, salivary cortisol level, and heart rate variability. RESULTS: The average age of participants was 45.40 years (±16.04) in the experimental group and 47.26 years (±13.83) in the control group. Two-thirds (66.0%) of the participants in each group were women. State anxiety (F = 31.42, p < 0.001), anxiety-numerical rating scale (F = 20.08, p < 0.001), salivary cortisol levels (F = 4.98, p = 0.021), and low-frequency component/high-frequency component ratio (F = 17.31, p < 0.001) in the experimental group were significantly reduced compared with those in the control group at the end of the music intervention. CONCLUSION: This study provides practical evidence of a reduction in anxiety and stress response from music intervention preceding an interventional cardiac catheterisation, indicating that this intervention should be considered in clinical management.

Distinct fibroblast progenitor subpopulation expedites regenerative mucosal healing by immunomodulation.

Injuries that heal by fibrosis can compromise organ function and increase patient morbidity. The oral mucosal barrier has a high regenerative capacity with minimal scarring, but the cellular mechanisms remain elusive. Here, we identify distinct postnatal paired-related homeobox-1+ (Prx1+) cells as a critical fibroblast subpopulation that expedites mucosal healing by facilitating early immune response. Using transplantation and genetic ablation model in mice, we show that oral mucosa enriched with Prx1+ cells heals faster than those that lack Prx1+ cells. Lineage tracing and scRNA-seq reveal that Prx1+ fibroblasts exhibit progenitor signatures in physiologic and injured conditions. Mechanistically, Prx1+ progenitors accelerate wound healing by differentiating into immunomodulatory SCA1+ fibroblasts, which prime macrophage recruitment through CCL2 as a key part of pro-wound healing response. Furthermore, human Prx1+ fibroblasts share similar gene and spatial profiles compared to their murine counterpart. Thus, our data suggest that Prx1+ fibroblasts may provide a valuable source in regenerative procedures for the treatment of corneal wounds and enteropathic fibrosis.

MIF is a common genetic determinant of COVID-19 symptomatic infection and severity.

BACKGROUND: Genetic predisposition to coronavirus disease 2019 (COVID-19) may contribute to its morbidity and mortality. Because cytokines play an important role in multiple phases of infection, we examined whether commonly occurring, functional polymorphisms in macrophage migration inhibitory factor (MIF) are associated with COVID-19 infection or disease severity. AIM: To determine associations of common functional polymorphisms in MIF with symptomatic COVID-19 or its severity. METHODS: This retrospective case-control study utilized 1171 patients with COVID-19 from three tertiary medical centers in the USA, Hungary and Spain, together with a group of 637 pre-pandemic, healthy control subjects. Functional MIF promoter alleles (-794 CATT5-8,rs5844572), serum MIF and soluble MIF receptor levels, and available clinical characteristics were measured and correlated with COVID-19 diagnosis and hospitalization. Experimental mice genetically engineered to express human high- or low-expression MIF alleles were studied for response to coronavirus infection. RESULTS: In patients with COVID-19, there was a lower frequency of the high-expression MIF CATT7 allele when compared to healthy controls [11% vs. 19%, odds ratio (OR) 0.54 [0.41-0.72], P < 0.0001]. Among inpatients with COVID-19 (n = 805), there was a higher frequency of the MIF CATT7 allele compared to outpatients (n = 187) (12% vs. 5%, OR 2.87 [1.42-5.78], P = 0.002). Inpatients presented with higher serum MIF levels when compared to outpatients or uninfected healthy controls (87 ng/ml vs. 35 ng/ml vs. 29 ng/ml, P < 0.001, respectively). Among inpatients, circulating MIF concentrations correlated with admission ferritin (r = 0.19, P = 0.01) and maximum CRP (r = 0.16, P = 0.03) levels. Mice with a human high-expression MIF allele showed more severe disease than those with a low-expression MIF allele. CONCLUSIONS: In this multinational retrospective study of 1171 subjects with COVID-19, the commonly occurring -794 CATT7MIF allele is associated with reduced susceptibility to symptomatic SARS-CoV-2 infection but increased disease progression as assessed by hospitalization. These findings affirm the importance of the high-expression CATT7MIF allele, which occurs in 19% of the population, in different stages of COVID-19 infection.

Long-Term Observational Outcomes after Total Correction of Congenital Heart Disease in Korean Patients with Down Syndrome: A National Cohort Study.

Background: In the present study, the population prevalence and postoperative morbidity and mortality in Down syndrome patients who underwent total correction for congenital heart disease were investigated using data from a large national cohort. Methods: Retrospective administrative data from 2,395,966 participants born between 2008 and 2012 were acquired from the National Investigation of Birth Cohort in Korea. Among Down syndrome patients, 58.3% had congenital heart disease and 32.3% underwent total correction. Propensity score matching (maximum 1:1) and stabilized inverse probability treatment weighting (IPTW) were performed for each group (153 Down syndrome patients and 4482 non-Down syndrome patients). Results: T late mortality rate was significantly higher in the Down syndrome group than in the non-Down syndrome group (8.1% vs. 3.8%). No differences were observed in postoperative heart failure and arrhythmias, but pulmonary hypertension was significantly greater in the Down syndrome group than in the non-Down syndrome group (26.9% vs. 7.0%). The length of hospitalization was longer in the Down syndrome group than in the non-Down syndrome group (14 days vs. 11 days; interquartile range (IQR): 10−25 vs. 6−19; p < 0.0001). After total correction, readmission frequency for any reason was minimally but statistically significantly higher in the Down syndrome group compared to the non-Down syndrome group (5 times vs. 5 times; IQR: 3−8 vs. 4−9; p < 0.0001). However, the number of emergency room visits was minimally but significantly lower in the Down syndrome group compared to the non-Down syndrome group (2 visits vs. 2 visits (IQR): 2−7 vs. 1−4; p = 0.016). Conclusions: Down syndrome patients with congenital heart disease undergoing total correction showed pulmonary hypertension after surgery, longer length of hospitalization, frequent hospitalization after surgery, and a higher rate of late mortality.

Outcomes After Transcatheter Closure of Atrial Septal Defect Without Using a Balloon-Sizing Technique: A Randomized Controlled Comparison with Closures Using a Balloon-Sizing Technique.

BACKGROUND: The transcatheter closure of atrial septal defect could be completed without the balloon-sizing technique, so we evaluated long-term outcomes compared with closure using balloon sizing, which was the conventional method. Even without using the balloon-sizing technique, transcatheter closure of atrial septal defect might be safe and effective. METHODS: We included 124 patients with isolated atrial septal defects who underwent device closure without balloon sizing between 2012 and 2016, and we further included 257 patients as a control group. Patients who received closure with multiple devices or who experienced postoperative residual defects were excluded. Immediate procedural results, as well as long-term outcomes for closure without balloon sizing, were investigated and compared with the control group. RESULTS: The procedural success rate was 96.7%, and there were no mortalities. No embolization or cardiac erosions were observed; however, one patient experienced residual shunt, and another developed progressed mitral regurgitation during the follow-up period (983±682 days). Newly onset persistent atrial fibrillation developed in one patient (1.0%). There were no significant differences in procedures or follow-up between the study and control groups. Despite the shorter procedural time in the study group, fluoro time was not different. Atrial arrhythmias were more frequently observed in the control group, but the difference was not significant. Persistent atrial fibrillation was observed in two patients in the control group (0.8%). CONCLUSIONS: Transcatheter closure of atrial septal defect can be performed safely and effectively without using the balloon-sizing technique. The long-term outcomes were similar to outcomes with balloon sizing.

Development of necrotizing enterocolitis in full-term infants with duct dependent congenital heart disease.

BACKGROUND: Although many studies have described an increased risk of necrotizing enterocolitis in duct dependent congenital heart diseases, very few have investigated its occurrence in full-term infants with duct dependent congenital heart diseases. METHODS: To evaluate the characteristics and risk factors of necrotizing enterocolitis, we performed a retrospective review of 355 full-term infants with duct dependent congenital heart diseases who received prostaglandin E1 therapy from April 2000 to May 2020. RESULTS: Necrotizing enterocolitis was observed in 10 patients (3.0%). Their average gestational age and birth weight were 38.2 weeks and 2783.5 g, respectively. The median age at diagnosis was 8.0 days (2-70 days). One patient was diagnosed with necrotizing enterocolitis stage IIA, five with stage IIB, two with stage IIIA, and two with stage IIIB; two (20%) received surgical treatment. The duct dependent pulmonary circulation group had higher frequencies of necrotizing enterocolitis (4.4%) than the duct dependent systemic circulation (2.0%) and parallel circulation (1.3%) groups. The necrotizing enterocolitis and the other groups had significantly different birth weight (2783.5 g vs 3170.9 g, respectively) and gestational age (38.2 weeks vs 39.1 weeks, respectively). Gestational age under 38 weeks (OR 8.87, p = 0.002), birth weight of < 2500 g (OR 5.1, p = 0.042), need for mechanical ventilation (OR 4.6, p = 0.021), parenteral nutrition (OR 107.7, p < 0.001), and functional single ventricle (OR 5.8, p = 0.009) were significant risk factors. The case-fatality rate was higher in the necrotizing enterocolitis (40.0%) than in the other group (8.3%, p = 0.009). CONCLUSIONS: Three percent of full-term infants with duct dependent congenital heart diseases developed necrotizing enterocolitis. Neonates with low birth weight, gestational age less than 38 weeks, functional single ventricle, or receiving assisted mechanical ventilation or parenteral nutrition are at increased risk.

A Retrospective Population-Based Survival Study of Idiopathic Pulmonary Arterial Hypertension in Korea.

BACKGROUND: Few studies used nationwide data to assess the survival rates (SRs) and death risk for idiopathic pulmonary arterial hypertension (IPAH; ICD-10 I27.0) in Korea. METHODS: IPAH data (N = 9,017; female:male = 6:4) were collected from the National Health Insurance Service in Korea, from 2006 through 2017. The data consisted of primary diagnoses related to IPAH. The Kaplan-Meier method and Cox proportional-hazards analyses were carried out. RESULTS: The mean age was 62.3 (± 19.4) years, 64.2 (± 18.9) years in female and 59.4 (± 19.8) years in male (P < 0.001). The one-, three-, five- and 10-year SRs for IPAH were 89.0%, 79.8%, 72.3% and 57.0%, respectively. The adjusted hazard ratio (HR) of IPAH was 1.81 (95% confidence interval [CI], 1.26-2.59) in 60-69 age group, 3.42 (95% CI, 2.40-4.87) in 70-79, and 7.73 (95% CI, 5.43-11.0) in 80s. Other risk factors were male, low-income status, diabetes, myocardial infarction, atrial fibrillation, ischemic stroke, hemorrhagic stroke, and malignant neoplasm. CONCLUSION: The 10-year SR of IPAH was 57% in Korea. The HR for IPAH was significantly high in patients with older age and other risk factors.