Sacubitril/Valsartan in Pediatric Heart Failure (PANORAMA-HF): A Randomized, Multicenter, Double-Blind Trial.

Background: Sacubitril/valsartan, an angiotensin receptor-neprilysin inhibitor (ARNI), is an established treatment for heart failure (HF) with reduced left ventricular ejection fraction. It has not been rigorously compared with angiotensin-converting enzyme inhibitors in children. PANORAMA-HF (Prospective Trial to Assess the Angiotensin Receptor Blocker Neprilysin Inhibitor LCZ696 Versus Angiotensin-Converting Enzyme Inhibitor for the Medical Treatment of Pediatric HF) is a randomized, double-blind trial that evaluated the pharmacokinetics and pharmacodynamics (PK/PD), safety, and efficacy of sacubitril/valsartan versus enalapril in children 1 month to <18 years of age with HF attributable to systemic left ventricular systolic dysfunction (LVSD).

Sex-Specific Susceptibility Loci Associated With Coronary Artery Aneurysms in Patients With Kawasaki Disease.

Background And Objectives: Kawasaki disease (KD) is an acute vasculitis that primarily affects children under age 5 years. Approximately 20-25% of untreated children with KD and 3-5% of those treated with intravenous immunoglobulin therapy develop coronary artery aneurysms (CAAs). The prevalence of CAAs is much higher in male than in female patients with KD, but the underlying factors contributing to susceptibility to CAAs in patients with KD remain unclear.

The impact of inappropriate steroid exposure before the diagnosis of Kawasaki disease.

Background: Kawasaki disease (KD) is a systemic inflammatory disease characterized by vasculitis. In South Korea, some pediatric doctors empirically prescribe steroids to control febrile pediatric patients. This study aimed to evaluate the clinical characteristics of patients with KD after steroid exposure.

Identification of B-cell-related HSPG2 and CDSN as susceptibility loci for Kawasaki disease.

Kawasaki disease (KD) is an acute pediatric vasculitis that predominantly affects children under the age of 5 years. To date, genome-wide association studies (GWAS) have identified several KD susceptibility genes (e.g.

Outcomes of surgery for coarctation of the aorta based on a new classification system.

Objectives: To evaluate early- and long-term outcomes of the surgical treatment for coarctation of the aorta based on a new classification system.

Methods: A retrospective clinical review of 111 patients with coarctation of the aorta who underwent surgery (March 2011 to August 2020) was performed. We categorised coarctation of the aorta into type I, with all three head vessels tightly packed; type II, with the left subclavian artery separated from the two other head vessels; and type III, with all three head vessels separated from one another.

Coronary artery spasm due to acute myocarditis in an adolescent: a case report.

Background: Myocarditis refers to the inflammation of the myocardium caused by infection or autoimmune disease that may or may not present with clinical manifestations, such as gastrointestinal symptoms, dyspnea, chest pain, or sudden death. Although myocarditis and coronary artery vasospasm may mimic ST-segment elevation myocardial infarction (STEMI) with normal coronary arteries on angiography, acute myocarditis rarely causes coronary artery spasm. Here, we report a case of coronary artery spasm with reversible electrocardiographic changes mimicking STEMI in an adolescent with acute myocarditis.

Right Pulmonary Artery Originating from Ascending Aorta (Hemitruncus Arteriosus) with VACTERL Association in a Neonate: A Case Report.

Vertebral, anal, cardiac, tracheo-esophageal fistula, renal and limb (VACTERL) association is defined as a condition including at least three of the above-mentioned anomalies in the same infant. Several cardiac defects that have been reported as a part of the VACTERL association are ventricular and atrial septal defects, hypoplastic left heart syndrome, transposition of the great arteries and tetralogy of Fallot. Anomalous origin of pulmonary artery (AOPA) from the ascending aorta is an unusual and critical cardiovascular anomaly, which frequently involves the right pulmonary artery (RPA).

Identification of rare coding variants associated with Kawasaki disease by whole exome sequencing.

Kawasaki disease (KD) is an acute pediatric vasculitis that affects genetically susceptible infants and children. To identify coding variants that influence susceptibility to KD, we conducted whole exome sequencing of 159 patients with KD and 902 controls, and performed a replication study in an independent 586 cases and 732 controls. We identified five rare coding variants in five genes (FCRLA, PTGER4, IL17F, CARD11, and SIGLEC10) associated with KD (odds ratio [OR], 1.

Effectiveness of posterior aortopexy for the left pulmonary vein obstruction between the left atrium and the descending aorta.

Background: Left pulmonary vein (PV) obstruction can occur due to compression between the left atrium (LA) and the descending aorta (DA). One of the effective solutions for this problem is posterior aortopexy. In this study, we have reported five cases of posterior aortopexy to relieve left PV obstruction between the LA and the DA.

A Case of Prenatally Diagnosed Uhl's Anomaly with Absent Pulmonary Valve Leaflets and Dysplastic Tricuspid Valve.

Uhl's anomaly is a very rare malformation of unknown cause, characterized by complete or partial absence of the right ventricular myocardium. The cardiac malformation causes progressive right heart failure, increased right-sided cardiac pressure, massive peripheral edema, and ascites. Patients usually present in infancy and rarely survive to adulthood.

IgA Levels Are Associated with Coronary Artery Lesions in Kawasaki Disease.

Background And Objectives: Kawasaki disease (KD) is an acute systemic vasculitis that affects the coronary arteries. Abnormal immune reactions are thought to contribute to disease pathogenesis. The effect of immunoglobulin (Ig) isotype (IgG, IgA, IgM, and IgE) on inflammatory data and clinical outcomes of patients with KD was examined.

Erratum: Application of Three Dimensional Printed Models in Congenital Heart Surgery: Surgeon's Perspective.

[This corrects the article on p. 310 in vol. 81.

[Application of Three-Dimensional Printed Models in Congenital Heart Surgery: Surgeon's Perspective].

To treat congenital heart disease, it is important to understand the anatomical structure correctly. Three-dimensional (3D) printed models of the heart effectively demonstrate the structural features of congenital heart disease. Occasionally, the exact characteristics of complex cardiac malformations are difficult to identify on conventional computed tomography, magnetic resonance imaging, and echocardiography, and the use of 3D printed models can help overcome their limitations.

Association of the IL16 Asn1147Lys polymorphism with intravenous immunoglobulin resistance in Kawasaki disease.

Kawasaki disease (KD) is an acute, self-limited vasculitis, mainly affecting children younger than 5 years old, with accompanying fever and signs of mucocutaneous inflammation. Intravenous immunoglobulin (IVIG) is the standard treatment for KD; however, ~15% of patients are resistant to IVIG treatment. To identify protein coding genetic variants influencing IVIG resistance, we re-analyzed our previous genome-wide association study (GWAS) data from 296 patients with KD, including 101 IVIG non-responders and 195 IVIG responders.

The coronary reimplantation after neoaortic reconstruction technique can make a difference in arterial switch operation.

Background: The aim of this study was to determine if there was a difference between coronary reimplantation after neoaortic reconstruction and open coronary reimplantation technique in arterial switch operation (ASO).

Methods: A total of 236 patients who underwent ASO from March 1994 to August 2018 were enrolled in this study. Multivariate analysis was performed for postoperative early mortality.

Risk Factors for Prolonged Pleural Effusion After Extracardiac Fontan Operation.

Prolonged pleural effusion after Fontan operation is a significant morbidity that leads to long hospital stays. We investigated the association of multiple risk factors, including clinical characteristics, hemodynamic parameters, and preoperative, operative, and postoperative factors, with prolonged pleural effusion after Fontan operation. Eighty-five patients who underwent a Fontan operation between January 2005 and June 2018 in our center were included in this retrospective study.

Simplified Tricuspid Polytetrafluoroethylene Valved Conduit: Midterm Results of Multicenter Study.

Background: Conduit survival without significant dysfunction is important when selecting the right ventricular outflow tract conduit. We made an expanded polytetrafluoroethylene tricuspid valved conduit using a simplified technique. We aimed to investigate the midterm functional results and longevity of this conduit.

Relief of Anteriorly Translocated Pulmonary Artery Compression With a Nuss Bar.

A concomitant anterior translocation of the right pulmonary artery (RPA) can be used in patients with airway compression by a dilated RPA associated with congenital heart disease having a large left-to-right shunt or aortic arch anomaly. However a chest wall deformity and mechanical compression of the anteriorly translocated RPA could develop after the operation. In this situation simple RPA angioplasty is not sufficient.

Identification of SAMD9L as a susceptibility locus for intravenous immunoglobulin resistance in Kawasaki disease by genome-wide association analysis.

Kawasaki disease (KD) is a systemic vasculitis affecting infants and children; it manifests as fever and signs of mucocutaneous inflammation. Intravenous immunoglobulin (IVIG) treatment effectively attenuates the fever and systemic inflammation. However, 10-20% patients are unresponsive to IVIG.

Does Coronary Reimplantation After Neoaortic Reconstruction Increase Aortic Regurgitation?

Coronary reimplantation after neoaortic reconstruction (CRANR) in the arterial switch operation (ASO) allows easy selection of accurate coronary transfer sites in the distended neoaorta. However, neoaortic valve injury may occur during coronary reimplantation. We determined whether the CRANR procedure increased the incidence of aortic valve regurgitation (AR) after ASO.

Infliximab Treatment for Intravenous Immunoglobulin-resistant Kawasaki Disease: a Multicenter Study in Korea.

Background And Objectives: We investigated the status of infliximab use in intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) patients and the incidence of coronary artery aneurysms (CAAs) according to treatment regimens.

Methods: Between March 2010 and February 2017, 16 hospitals participated in this study. A total of 102 (32.