Evaluation of the Efficacy and Accuracy of Super-Flexible Three-Dimensional Heart Models of Congenital Heart Disease Made via Stereolithography Printing and Vacuum Casting: A Multicenter Clinical Trial.

Three-dimensional (3D) printing is an advanced technology for accurately understanding anatomy and supporting the successful surgical management of complex congenital heart disease (CHD). We aimed to evaluate whether our super-flexible 3D heart models could facilitate preoperative decision-making and surgical simulation for complex CHD. The super-flexible heart models were fabricated by stereolithography 3D printing of the internal and external contours of the heart from cardiac computed tomography (CT) data, followed by vacuum casting with a polyurethane material similar in elasticity to a child's heart.

Sarcomere gene variants did not improve cardiac function in pediatric patients with dilated cardiomyopathy from Japanese cohorts.

Dilated cardiomyopathy (DCM) is a progressive myocardial disorder characterized by impaired cardiac contraction and ventricular dilation. However, some patients with DCM improve when experiencing left ventricular reverse remodeling (LVRR). Currently, the detailed association between genotypes and clinical outcomes, including LVRR, particularly among children, remains uncertain.

Decreased smooth muscle cells and fibrous thickening of the tunica media in peripheral pulmonary artery stenosis in Alagille syndrome.

Alagille syndrome is caused by mutations in genes involved in NOTCH signaling, specifically JAG1 and NOTCH2, and is associated with a high rate of peripheral pulmonary artery stenosis. In this study, we report the case of an infant with Alagille syndrome caused by a JAG1 mutation, who succumbed to acute exacerbation of right heart failure due to severe peripheral pulmonary artery stenosis. The autopsy revealed that the peripheral pulmonary arteries were significantly stenosed, exhibiting hypoplasia and thickened vessel walls.

Monitoring Berlin Heart EXCOR by computer vision: A preliminary implementation and evaluation.

Background: EXCOR Pediatric is one of the most commonly used ventricular assist devices (VAD) for small children; it requires visual inspection of the diaphragm movement to assess its operating status. Although this visual inspection can only be performed by trained medical professionals, it can also be attempted by the recent advances in computer vision technology.

Methods: Movement of the diaphragm in the operating EXCOR VAD was recorded as movies and annotated frame-by-frame in three classes according to the state of the diaphragm: "fill," "mid," and "empty.

Prediction of adverse cardiovascular events in children using artificial intelligence-based electrocardiogram.

Background: Convolutional neural networks (CNNs) have emerged as a novel method for evaluating heart failure (HF) in adult electrocardiograms (ECGs). However, such CNNs are not applicable to pediatric HF, where abnormal anatomy of congenital heart defects plays an important role. ECG-based CNNs reflecting neurohormonal activation (NHA) may be a useful marker of pediatric HF.

Removal of central venous catheter using Evolution Rotating Dilator.

We experienced the first case of a difficult-to-extract central venous catheter removed with a pacemaker lead removal system: a 14-year-old boy with Hirschsprung's disease who had repeated catheter infections that could not be removed by traction. Because the catheter lumen was occluded, a suture was tied around the end of the catheter and the catheter was removed with a rotating dilator.

Hemodynamic simulation of complete transposition of the great arteries for optimal treatment strategies based on its circulatory physiology.

Our study aimed to elucidate the role of different shunts and provide novel insights into optimal treatment approaches for complete transposition of the great arteries (TGA), which is characterized by unique and complicated circulatory dynamics. We constructed a computational cardiovascular TGA model and manipulated cardiovascular parameters, such as atrial septal defect (ASD) and patent ductus arteriosus (PDA) sizes, to quantify their effects on oxygenation and hemodynamics. In addition, ASD flow patterns were investigated as innovative indications for balloon atrial septostomy (BAS).

A novel pathogenic variant located just upstream of the C-terminal Ser423-X-Ser425 phosphorylation motif in SMAD3 causing Loeys-Dietz syndrome.

Objective: Loeys-Dietz syndrome (LDS) is a heritable disorder of connective tissue closely related to Marfan syndrome (MFS). LDS is caused by loss-of-function variants of genes that encode components of transforming growth factor-β (TGF-β) signaling; nevertheless, LDS type 1/2 caused by TGFBR1/2 pathogenic variants is frequently found to have paradoxical increases in TGF-β signaling in the aneurysmal aortic wall. Here, we present a Japanese LDS family having a novel SMAD3 variant.

Reduced volume and altered composition of paraspinal muscles in Marfan syndrome: A retrospective cohort study.

Retrospective cohort study. Spinal deformities in patients with Marfan syndrome (MFS) are distinct from those in patients with idiopathic scoliosis (IS). It is more prone to progression and more likely to present with sagittal malalignment than IS.

Factors affecting in-hospital mortality among pediatric patients with myocarditis treated with mechanical circulatory support.

Background: Mechanical circulatory support (MCS) is a common treatment modality for circulatory failure caused by pediatric myocarditis. Despite improvements in treatment strategy, the mortality rate of pediatric patients with myocarditis treated with MCS is still high. Identifying the factors associated with mortality among pediatric patients with myocarditis treated with MCS may help reduce the mortality rate.

Cardiovascular Complications of Down Syndrome: Scoping Review and Expert Consensus.

Cardiovascular disease is a leading cause of morbidity and mortality in individuals with Down syndrome. Congenital heart disease is the most common cardiovascular condition in this group, present in up to 50% of people with Down syndrome and contributing to poor outcomes. Additional factors contributing to cardiovascular outcomes include pulmonary hypertension; coexistent pulmonary, endocrine, and metabolic diseases; and risk factors for atherosclerotic disease.

Aortic and bronchial perforation caused by a vascular clip 3 years after its placement.

Vascular clips are currently acknowledged as a safe and efficient tool for vessel ligation in every surgical field. Here, we describe a case of massive haemoptysis due to perforation of both the aorta and bronchus, caused by a titanium clip that was placed between them 3 years ago. The present case highlights the rare but life-threatening complications that clips can cause in the mediastinum, especially when placed between a fixed structure and a large blood vessel or respiratory tract.

Practice and Safety of Static Balloon Atrial Septostomy Based on a Nationwide Registry Data.

Background: Balloon atrial septostomy (BAS) is an essential catheterization procedure for congenital heart lesions. Recently, a balloon catheter for static BAS was approved for the first time in Japan as an alternative to the conventional pull-through BAS. Despite the expected increase in the use of static BAS, reports on its safety are scarce worldwide.

Status of adult outpatients with congenital heart disease in Japan: The Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease Registry.

Background: The Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease (JNCVD-ACHD) was founded in 2011 for the lifelong care of adult patients with congenital heart disease (ACHD patients). This network maintains the first Japanese ACHD registry.

Methods And Results: From 2011 to 2019, the JNCVD-ACHD registered 54 institutions providing specialized care for ACHD patients in 32 of the 47 prefectures in Japan.

Effect of procedural volume on the outcomes of congenital heart surgery in Japan.

Objectives: The present study developed a new risk model for congenital heart surgery in Japan and determined the relationship between hospital procedural volume and mortality using the developed model.

Methods: We analyzed 47,164 operations performed between 2013 and 2018 registered in the Japan Cardiovascular Surgery Database-Congenital and created a new risk model to predict the 90-day/in-hospital mortality using the Japanese congenital heart surgery mortality categories and patient characteristics. The observed/expected ratios of mortality were compared among 4 groups based on annual hospital procedural volume (group A [5539 procedures performed in 90 hospitals]: ≤50, group B [9322 procedures in 24 hospitals]: 51-100, group C [13,331 procedures in 21 hospitals]: 101-150, group D [18,972 procedures in 15 hospitals]: ≥151).

Successful use of ivabradine in a 10-year-old patient with graft failure after heart transplantation.

We encountered a paediatric case of graft failure due to antibody-mediated rejection after heart transplantation in which ivabradine was effective. Inappropriate sinus tachycardia in denervated transplanted hearts is a good indication for ivabradine administration as beta-blockers have a limited efficacy. To our knowledge, this is the first report on the effectiveness of ivabradine in a paediatric heart transplant rejection case.