The Long-term Outcomes and Risk Factors of Complications After Fontan Surgery: From the Korean Fontan Registry (KFR).

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.

Safety and efficacy of selumetinib in pediatric and adult patients with neurofibromatosis type 1 and plexiform neurofibroma.

Background: The MEK inhibitor, selumetinib, reduces plexiform neurofibroma (PN) in pediatric patients with neurofibromatosis type 1 (NF1). Its safety and efficacy in adults with PN and effectiveness in other NF1 manifestations (eg, neurocognitive function, growth reduction, and café-au-lait spots) are unknown.

Methods: This open-label, phase II trial enrolled 90 pediatric or adult NF1 patients with inoperable, symptomatic, or potentially morbid, measurable PN (≥3 cm).

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).

Pacemaker-Related Factors and Outcomes of Fontan Patients - Impact of Paced QRS Duration.

Background: Permanent pacemaker (PPM) implantation has been identified as a risk factor for morbidity and mortality after Fontan operation. This study investigated the factors associated with outcomes in patients with Fontan physiology who underwent PPM implantation.

Methods and results: We retrospectively reviewed 508 patients who underwent Fontan surgery at Asan Medical Center between September 1992 and August 2022.

Hybrid Right Ventricular Outflow Stent Insertion in a Small Neonate with Muscular Pulmonary Atresia with Intact Ventricular Septum: A Case Report.

Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital heart disease that often needs a critical decision on whether to open the right ventricular outflow tract (RVOT). Significant morbidity and considerable mortality might preclude the safe use of percutaneous or surgical right ventricular decompression in patients with muscular PAIVS. We report the case of a 21-day-old neonate weighing less than 3 kg who underwent hybrid RVOT stent insertion as initial palliation for muscular PAIVS and subsequent anatomical correction at 5 months of age, with 6 years of follow-up.

Outcomes of Arterial Switch Operation With Aortic Arch Reconstruction.

Background: This study investigated the outcomes and factors associated with reintervention or development of significant pulmonary stenosis (PS) after repair of transposition of the great arteries (TGA) or Taussig-Bing anomaly (TBA) with aortic arch obstruction.

Methods: A total of 51 patients with TGA or TBA who underwent an arterial switch operation and aortic arch reconstruction between 2004 and 2020 were included. The outcomes of interest were all-cause death, including heart transplantation, all-cause reintervention, right-sided reintervention, and development of significant PS.

Outcomes of pulmonary artery sling repair without tracheoplasty.

Objective: This study aimed to investigate surgical outcomes of pulmonary artery (PA) sling without tracheoplasty.

Methods: From 2001 through 2020, among 22 patients who underwent PA sling repair, all but 1 patient who underwent concomitant tracheal surgery were analyzed. The outcomes of interest were all-cause death, PA reintervention, tracheal intervention, and readmission for respiratory symptoms.

Spontaneous defervescence and its prediction in the acute phase of Kawasaki disease.

Background: In Kawasaki disease (KD), fever occasionally resolves spontaneously before 10 days from the onset, right after diagnosing. However, there is not enough evidence of intravenous immunoglobulin (IVIG) treatment in this case. The aim of this study was to investigate the relationship between spontaneous defervescence and coronary artery aneurysm and to develop a scoring model for its prediction in acute KD.

Contemporary Outcomes After Pulmonary Artery Banding in Complete Atrioventricular Septal Defect.

Background: This study investigated the clinical outcomes and the effect of band tightness on outcome after pulmonary artery banding (PAB) in patients with complete atrioventricular septal defect (AVSD).

Methods: From 2000 through 2019, among 133 patients with isolated complete AVSD pursuing biventricular repair, 34 patients (25.6%) who underwent PAB were included in this study.

The impact of a bicuspid pulmonary valve in the aortic position after arterial switch for transposition of the great arteries on neoaortic root dimension and function: a propensity score matched analysis.

Objectives: This study investigated the effect of a bicuspid pulmonary valve on neoaortic root morphology, function, and the clinical outcomes of early survivors after the arterial switch operation using propensity score matching.

Methods: From 1997 to 2018, a total of 442 patients underwent the arterial switch operation for transposition of the great arteries. After exclusion of patients who underwent a staged repair, were repaired beyond 1 year of age, died before discharge and who lacked echocardiographic data at discharge, propensity score matching was used for analysis.

Paediatric heart transplantation recipients ≥7 years of age receiving donors with pre-existing coronary atherosclerosis showed progressive coronary artery disease.

Background: This study aimed to determine the effect of donor-transmitted atherosclerosis on the late aggravation of cardiac allograft vasculopathy in paediatric heart recipients aged ≥7 years.

Methods: In total, 48 patients were included and 23 had donor-transmitted atherosclerosis (baseline maximal intimal thickness of >0.5 mm on intravascular ultrasonography).

Development of Cardiac Events and Functional Recovery Prediction Models for Pediatric Dilated Cardiomyopathy.

Since both the risk of death and the probability of spontaneous functional recovery (FR) coexist in association with pediatric dilated cardiomyopathy (DCMP), management should be based on individualized outcome predictions. A single-center retrospective review of 105 pediatric patients (age at presentation ≤ 18 years) with DCMP, managed between 1994 and 2017, was performed. Logistic regression was conducted to identify variables associated with FR and cardiac events (CEs), i.

Cerebral and Somatic Oxygen Saturation in Neonates with Congenital Heart Disease before Surgery.

Background: We investigated preoperative cerebral (ScO) and abdominal (StO) regional oxygen saturations according to cardiac diagnosis in neonates with critical CHD, their time trends, and the clinical and biochemical parameters associated with them.

Methods: Thirty-seven neonates with a prenatal diagnosis of CHD were included. ScO and StO values were continuously evaluated using near-infrared spectroscopy.

Clinical Outcomes of Implantable Cardioverter-Defibrillator in Pediatric Patients - A Korean Multicenter Study.

Background: Implantable cardioverter-defibrillator (ICD) therapy is important for the prevention of sudden cardiac death, but data on clinical outcomes of ICD therapy in Asian pediatric patients are scarce. The aim of this Korean multicenter study was to evaluate the current state and elucidate the clinical outcomes of ICD therapy in children.

Methods and results: Data from 5 pediatric cardiology centers were retrospectively collected from 2007 to 2019.

Procedural Predictors and Outcomes of Percutaneous Secundum Atrial Septal Defect Closure in Children Aged <6 Years.

Background: Percutaneous atrial septal defect (ASD) closure is the treatment of choice for patients with a suitable ASD anatomy; however, the procedural characteristics and outcomes in children aged <6 years are unclear. The feasibility and safety of percutaneous ASD closure in children aged <6 years was evaluated and the predictors of procedural failure and challenging cases were identified.

Methods and results: Patients from a single center between 2006 and 2018 (n=407) were retrospectively evaluated.

Pulmonary valve replacement following repair of tetralogy of Fallot: comparison of outcomes between bio- and mechanical prostheses.

Objectives: The aims of this study were to evaluate and compare the outcomes after pulmonary valve replacement (PVR) with a mechanical prosthesis (MP) and a bioprosthesis (BP).

Methods: From 2004 through 2017, a total of 131 patients, who had already been repaired for tetralogy or Fallot or its variants, underwent their first PVR with an MP or a BP. Outcomes of interests were prosthesis failure (stenosis >3.

The impact of additional antegrade pulmonary blood flow at bidirectional Glenn shunt on long-term outcomes.

Objectives: We investigated the impact of additional antegrade pulmonary blood flow on the long-term outcomes after bidirectional Glenn shunt.

Methods: From 2001 to 2015, 279 patients underwent bidirectional Glenn shunt as an interim palliation for a functionally single ventricle. After excluding patients with a previous Kawashima or Norwood operation, 202 patients with preexisting antegrade pulmonary blood flow before bidirectional Glenn shunt were included in this study.

Predictive Parameters of Decreased Left Ventricular Global Longitudinal Strain at 1 Month After Pediatric Heart Transplantation.

Previous reports indicate that the decreased left ventricular global longitudinal strain (LVGLS) seen in the early postoperative period of pediatric heart transplant patients generally recovers over the course of 1-2 years. In this study, we investigate the predictive capacity of preoperative parameters on the LVGLS decline seen at 1 month post transplant. Forty-six transplant subjects with 2D echocardiographic images sufficient for speckle tracking echocardiography were enrolled.

Growth Pattern of the Neo-Aorta after Arterial Switch Operation during Childhood.

Background And Objectives: Neo-aortic root dilatation (ARD) and annular dilatation (AAD) tend to develop after arterial switch operation (ASO). However, the trend of neo-aortic growth has not been well established. This paper aims to identify this trend, its associated factors, and predictors of neo-aortic dilatation after ASO.

Identification of high-risk Fontan candidates by intraoperative pulmonary flow study.

Background: Pre-Fontan risk stratification is still less than optimal. We aimed to evaluate the effectiveness of intraoperative pulmonary flow study (IOFS), in combination with conventional preoperative evaluation, in the identification of high-risk candidates for the Fontan operation.

Method: Data from 37 patients (age: 38.

B-Type Natriuretic Peptide Levels and Cardiac Dysfunction in Children on Peritoneal Dialysis.

Abnormalities in left ventricular (LV) structure and function are prevalent in patients on peritoneal dialysis (PD). The risk of cardiovascular mortality is also 10 - 20 times higher in PD patients than in age- and gender-matched healthy control subjects.Echocardiography was performed annually or every 6 months for children on PD; the frequency was increased if the patient had hypertension or any heart problem.

Coronary artery status of patients with transient fever 24-36 h after first IVIG infusion did not differ from that seen in responsive patients.

Background: Current management guidelines for patients with Kawasaki disease (KD) differ in their recommendations for fever observation times when determining resistance to initial intravenous immunoglobulin (IVIG). This retrospective study assessed coronary artery status in patients with transient fever 24-36 h after the completion of a first IVIG infusion.

Methods: Children with KD treated with IVIG between January 2006 and February 2017 were included.

Revisitation of Double-Inlet Left Ventricle or Tricuspid Atresia With Transposed Great Arteries.

Background: Patients with double-inlet left ventricle or tricuspid atresia with transposition of the great arteries are predisposed to systemic outflow tract obstruction necessitating systemic outflow relief operations (SORO).

Methods: Between January 2000 and February 2018, 30 patients with double-inlet left ventricle (n = 20) or tricuspid atresia (n = 10) with transposition of the great arteries underwent single-ventricle palliation. Arch obstruction was observed in 14 patients.