Role of the Right Ventricle Overhaul Procedure in Comprehensive Management for Pulmonary Atresia With Intact Ventricular Septum - A Magnetic Resonance Imaging-Based Study.

Background: We evaluated the surgical outcomes of modified right ventricle (RV) overhaul (mRVOh), implemented as part of comprehensive management for pulmonary atresia with intact ventricular septum (PA-IVS).

Methods And Results: Twenty-five mRVOh procedures were performed in 23 patients with PA-IVS without RV-dependent coronary circulation. The procedure involved RV sinus myectomy, infundibular muscle resection, and tricuspid valve (TV) and pulmonary valve (PV) repair.

Clinical Implications of Leaflet Thrombosis After Percutaneous Pulmonary Valve Implantation With the Pulsta Valve: Life-Long Aspirin Is Necessary?

Background: Hypo-attenuated leaflet thickening (HALT) is a subclinical finding on cardiac computed tomography (CT) associated with leaflet thrombosis in bioprosthetic valves. However, its incidence and risk factors following percutaneous pulmonary valve implantation (PPVI) remain poorly characterized.

Aims: This study aimed to determine the incidence of HALT after PPVI and to identify clinical and procedural factors associated with its occurrence.

Arrhythmic Risk and Clinical Features in Catecholaminergic Polymorphic Ventricular Tachycardia: Results From a Multicenter Study in Korea.

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare but life-threatening inherited arrhythmia in children, often associated with sudden cardiac death (SCD). Risk stratification remains challenging. This study aimed to assess clinical characteristics, genetic profiles, and risk factors for adverse outcomes in Korean pediatric CPVT patients.

ANALGESIC EFFECTS OF PREEMPTIVE CELECOXIB OR TRAMADOL/ACETAMINOPHEN ADDED TO STANDARD POSTOPERATIVE PAIN MANAGEMENT IN IMPACTED MANDIBULAR THIRD MOLAR EXTRACTION: A MULTICENTER, RANDOMIZED, DOUBLE-BLIND, PLACEBO-CONTROLLED TRIAL.

Objectives: This study aimed to assess the efficacy and safety of adding single-dose preemptive celecoxib 200 mg (Cc) or tramadol 37.5 mg/acetaminophen 325.0 mg (T/A) to standard postoperative analgesia after impacted mandibular third molar extraction, a design not previously explored.

Pregnancy Outcomes in Women With Fontan Circulation: Single Tertiary Center Experience.

Background And Objectives: The Fontan operation is a significant advancement in the palliative treatment of patients with single ventricular physiology. However, pregnancy in women with Fontan circulation presents increased risks for both the mother and fetus, with limited data available on maternal and fetal outcomes. This study reports maternal and fetal outcomes of pregnancies in women with Fontan circulation from a single tertiary center.

Treatment Outcomes in Children With Catecholaminergic Polymorphic Ventricular Tachycardia: A Single Institutional Experience.

Background And Objectives: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a life-threatening inherited arrhythmogenic disorder. Recently, , the major CPVT-causative gene, was associated with neuropsychiatric manifestations. We aimed to analyze the clinical presentations, neuropsychiatric manifestations, and treatment outcomes of children with CPVT.

Long-term outcomes of extracardiac Gore-Tex conduits in Fontan patients.

Background: Extracardiac conduit Fontan procedure (ECFP) employing a Gore-Tex conduit has been widely used for patients with single ventricle physiology; however, the long-term status of the conduit is unknown. We investigated the changes in a Gore-Tex conduit after ECFP and the factors associated with its narrowing.

Methods: We conducted a retrospective analysis of 86 patients who underwent ECFP between January 1995 and December 2008 and had cardiac computed tomography (CT) during the follow-up period.

Tbx5 maintains atrial identity in post-natal cardiomyocytes by regulating an atrial-specific enhancer network.

Understanding how the atrial and ventricular heart chambers maintain distinct identities is a prerequisite for treating chamber-specific diseases. Here, we selectively knocked out (KO) the transcription factor in the atrial working myocardium to evaluate its requirement for atrial identity. Atrial inactivation downregulated atrial cardiomyocyte (aCM) selective gene expression.

Risk Factors for Coronary Artery Complications After Prosthetic Pulmonary Valve Implantation in Patients With Congenital Heart Disease.

Background: Coronary artery complications (CACs) in patients who undergoing prosthetic pulmonary valve implantation for congenital heart disease can lead to fetal outcomes. However, the incidence of and risk factors for CACs in these patients remain unknown.

Methods and results: A retrospective cohort study was conducted on patients who underwent cardiac computed tomography or invasive coronary angiography after prosthetic pulmonary valve implantation at Seoul National University Hospital from June 1986 to May 2021.

Computed Tomography Measurement of the Aorta in Midaortic Syndrome in Children and Adolescents and Their Clinical Manifestations and Outcomes.

Midaortic syndrome (MAS) presents challenges in diagnosis due to the absence of well-defined diagnostic criteria in pediatric patients. This retrospective study aimed to aid in the diagnosis of MAS by employing computed tomography (CT) to measure the z-score of the aorta as well as to identify and understand its clinical features. CT images, echocardiography findings, and medical records of 17 patients diagnosed with MAS between 1997 and 2023 were reviewed, and z-scores were calculated.

Late development of intra-atrial reentrant tachycardia in lateral tunnel Fontan patients and the preventive role of prophylactic cryoablation.

Objective: Intra-atrial reentrant tachycardia is an important late-onset complication in patients undergoing the Fontan procedure. However, the protective effects of prophylactic cryoablation against late-onset intra-atrial reentrant tachycardia are unclear. This study investigated the late development of intra-atrial reentrant tachycardia in patients undergoing the lateral tunnel Fontan procedure and the role of prophylactic cryoablation.

Shallow suture at ventricular septal defect may safely reduce right bundle branch block.

Background: To avoid rhythm disturbance, sutures for ventricular septal defect closure have been traditionally placed 2∼5 mm or more away from the edge of the ventricular septal defect. However, the traditional suturing method appears to induce right bundle branch block and tricuspid valve regurgitation after ventricular septal defect closure more than our alternative technique, shallow suturing just at the edge of the ventricular septal defect (shallower bites at the postero-inferior margin). We aimed to verify our clinical experience of perimembranous ventricular septal defect repair.

Trend of fetal echocardiography use and comparison of medical costs for congenital heart disease treatment based on fetal echocardiography use in a Korean single center.

Background: The rate of the prenatal diagnosis of congenital heart disease is increasing along with advances in fetal echocardiography techniques. Here, we aimed to investigate the trend of the use of fetal echocardiography over time and to compare the medical costs of congenital heart disease treatment according to whether fetal echocardiography was performed.

Methods: We reviewed our hospital's database, and patients who underwent the first surgery for congenital heart disease within 30 days of birth during 2005-2007, 2011-2013, and 2017-2019 were included.

maintains atrial identity by regulating an atrial enhancer network.

Understanding how the atrial and ventricular chambers of the heart maintain their distinct identity is a prerequisite for treating chamber-specific diseases. Here, we selectively inactivated the transcription factor in the atrial working myocardium of the neonatal mouse heart to show that it is required to maintain atrial identity. Atrial inactivation downregulated highly chamber specific genes such as and , and conversely, increased the expression of ventricular identity genes including .

Arrhythmia and COVID-19 in children.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection caused worldwide health problems, and coronavirus disease 2019 (COVID-19) was declared a pandemic by the World Health Organization in March 2020. Cardiovascular complications of COVID-19 are not uncommon; among them, arrhythmia is considered a significant risk factor for poor health outcomes in adults. However, data are scarce on the arrhythmia of pediatric patients with SARS-CoV-2 infection, possibly due to their mild symptoms and low incidence of cardiovascular involvement.

Cardiovascular Characteristics and Progressions of Hypertrophic Cardiomyopathy and Pulmonary Stenosis in RASopathy Syndrome in the Genomic Era.

Introduction: To investigate cardiovascular characteristics and progressions of hypertrophic cardiomyopathy (HCM) and pulmonary stenosis (PS) and determine whether any genotype-phenotype correlations exist in patients with gene-confirmed RASopathy syndrome.

Study Design: Eighty patients (male, 55%) confirmed as having RASopathy syndrome by genetic testing at a single tertiary center were enrolled. Subjects' medical and echocardiography records were reviewed and the changes in the z scores of left ventricular wall thickness (LVWT) and the degree of PS over time were examined during follow-up of 5.

Application of sequential multimodal analgesia before and after impacted mandibular third molar extraction: Protocol for a randomized controlled trial.

Background: Several analgesics have been applied under various protocols to control the moderate-to-severe postoperative pain caused by the surgical extraction of an impacted mandibular third molar. However, a consensus on optimal pain management while minimizing side effects is yet to be reached.

Methods: This multi-center, prospective, double-blind, randomized controlled trial aims to evaluate the efficacy and safety of sequential multimodal analgesia combined with postoperative zaltoprofen along with multiple preemptive analgesics.

Late Outcomes of Pediatric and Congenital Heart Disease Patients Following Cardiac Resynchronization Therapy.

Background And Objectives: 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.

The Outcome of Long QT Syndrome, a Korean Single Center Study.

Background And Objectives: Although long QT syndrome (LQTS) is a potentially life-threatening inherited cardiac channelopathy, studies documenting the long-term clinical data of Korean patients with LQTS are scarce.

Methods: This retrospective cohort study included 105 patients with LQTS (48 women; 45.7%) from a single tertiary center.

Valve-Sparing Root Replacement in a Turner Syndrome Patient with Bicuspid Aortic Valve and Juxtacommissural Origin of the Right Coronary Artery: A Case Report.

A 32-year-old woman diagnosed with Turner syndrome presented to the hospital for an evaluation of cardiovascular complications. Preoperative computed tomography (CT) and echocardiography showed progression of aortic root and ascending aorta dilatation, as well as a bicuspid aortic valve. There was no evidence of aortic regurgitation.

Long-term Outcome of Fontan-Associated Protein-Losing Enteropathy: Treatment Modality and Predictive Factor of Mortality.

Background And Objectives: 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.

Methods: We reviewed the medical records of 38 patients with Fontan-associated PLE from 1992 to 2018 in 2 institutions in Korea.

Acute perimyocarditis mimicking acute myocardial infarction in a 12-year-old boy with duchenne muscular dystrophy.

Differential diagnosis of chest pain in the pediatric population is important but can be challenging. A 12-year-old boy with Duchenne muscular dystrophy presented with chest pain, cardiac enzyme elevation, and convex ST elevations in the inferior leads with reciprocal ST depression in the anterior leads on electrocardiogram. Echocardiography on admission revealed normal left ventricular function.