As fluoroscopy-guided interventional procedures gain popularity, the associated health threats from radiation exposure to interventionalists during these procedures are increasing. Therefore, an understanding of the potential risks of radiation and careful consideration on minimizing exposure to radiation during the procedures are of paramount importance. The Korean Pancreatobiliary Association has developed a clinical practice guideline to minimize radiation exposure during fluoroscopy-guided interventional procedures. This guideline provides recommendations to deal with the risk of radiation exposure to interventionalists who perform fluoroscopy-guided procedures, and emphasizes the importance of proper and practical approaches to avoid unnecessary radiation exposure.

As fluoroscopy-guided interventional procedures gain popularity, the associated health threats from radiation exposure to interventionalists during these procedures are increasing. Therefore, an understanding of the potential risks of radiation and careful consideration on minimizing exposure to radiation during the procedures are of paramount importance. The Korean Pancreatobiliary Association has developed a clinical practice guideline to minimize radiation exposure during fluoroscopy-guided interventional procedures. This guideline provides recommendations to deal with the risk of radiation exposure to interventionalists who perform fluoroscopy-guided procedures, and emphasizes the importance of proper and practical approaches to avoid unnecessary radiation exposure.

Background and Objectives: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a life-threatening inherited arrhythmogenic disorder. Recently, RYR2, the major CPVTcausative gene, was associated with neuropsychiatric manifestations. We aimed to analyze the clinical presentations, neuropsychiatric manifestations, and treatment outcomes of children with CPVT. Methods: We retrospectively reviewed 23 patients diagnosed with CPVT before 19 years of age. Genetic analysis, history of neuropsychiatric manifestations, changes in ventricular arrhythmia burden before and after treatment, occurrence of cardiac events, and overall survival (OS) were investigated. Results: RYR2 variants were identified in 17 patients, and 14 were classified as pathogenic or likely pathogenic. Neuropsychiatric manifestations, including intellectual disability and attention deficit hyperactivity disorder, were identified in 10 patients (43.5%). The 5-year cardiac event-free survival rate was 31.2%, and the 10-year OS rate was 73.1%. Patients diagnosed since 2009 had a higher cardiac event-free survival rate than those diagnosed before 2009 (p=0.0028).Combined beta-blocker and flecainide therapy demonstrated a lower risk of cardiac events than beta-blocker monotherapy (hazard ratio [HR], 0.08; 95% confidence interval [CI], 0.02–0.38;p=0.002). Left cardiac sympathetic denervation (LCSD) reduced the ventricular arrhythmia burden in Holter monitoring. Occurrence of near-fatal cardiac events after diagnosis was an independent predictor of death (HR, 33.40; 95% CI, 6.23–179.95; p<0.001). Conclusions Neuropsychiatric manifestations are common in children with CPVT. Flecainide and/or LCSD, when added to beta-blocker therapy, reduce the ventricular arrhythmia burden and cardiac events, thereby improving treatment outcomes in recent years.

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.

This case report details a 23-year-old female with Down syndrome who suffered from acute respiratory failure due to severe obstructive sleep apnea syndrome and accompanying pulmonary arterial hypertension (PAH). The patient presented with obesity, adenotonsillar hypertrophy, and craniofacial anomalies commonly seen in Down syndrome, predisposing her to sleep-disordered breathing. Upon intensive care unit admission, she exhibited cardiomegaly, bilateral pulmonary edema, and right ventricular dysfunction. Polysomnography revealed severe sleep apnea with an apnea-hypopnea index of 108.7/hr. Treatment modalities included noninvasive positive pressure ventilation, diuretics, antibiotics, and positive airway pressure (PAP) devices to manage hypercapnia, pulmonary edema, and sleep apnea. PAH, a recognized complication of untreated sleep apnea, contributed to right ventricular dysfunction. A multidisciplinary approach was vital, with long-term management centered on continuous PAP therapy and comprehensive obesity management. This case underscores the intricate interplay between Down syndrome, sleep apnea, and PAH, highlighting the significance of early recognition and coordinated intervention in individuals with Down syndrome to enhance overall outcomes and quality of life.

Purpose: Data regarding the association between metabolically healthy obesity (MHO) and preclinical atherosclerosis in childhood are lacking. Carotid intima-media thickness (cIMT) is a noninvasive method used to assess cardiovascular risk. This study examined the relationships among cIMT, metabolic phenotypes, and cardiometabolic risk factors (CMRFs) in overweight and obese adolescents. Methods: Anthropometric, biochemical, and cIMT data were collected. The study participants were categorized as MHO or metabolically unhealthy obesity (MUO) based on insulin resistance. CMRFs were assessed using blood pressure (BP); levels of triglycerides, high-density lipoprotein cholesterol (HDL-C), and fasting plasma glucose; or a diagnosis of diabetes mellitus. Differences in cIMT values were evaluated according to the metabolic phenotype and factors associated with cIMT. Results: Among the 111 participants (80 boys, 72.1%), 23 (20.7%) were classified as MHO and 88 (79.3%) as MUO. The MHO group exhibited lower glycated hemoglobin and triglyceride levels and higher HDL-C levels compared to those exhibited by the MUO group (all P<0.01). The cIMT values did not differ significantly between the MHO and MUO groups. The high cIMT tertile group revealed higher systolic BP compared to that exhibited by the low cIMT tertile group (123.7±2.1 mmHg vs. 116.9±1.6 mmHg, P=0.028). Mean cIMT was positively correlated with age (β=0.009) and body mass index (BMI) (β=0.033) after adjusting for covariates (both P<0.05). Conclusion In overweight and obese Korean adolescents, cIMT was associated with age and BMI but not with metabolic phenotype or CMRFs. Further research is warranted to determine the relationship between cIMT during adolescence and cardiovascular outcomes during adulthood.