Gaku Izumi

Gaku Izumi
Hokkaido University Graduate School of Medicine · Pediatrics

MD PhD

About

43
Publications
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320
Citations

Publications

Publications (43)
Article
Full-text available
Background: Asymptomatic premature ventricular complex (PVC) in childhood often disappears over time. However, predictive factors for persistent PVC are unknown. We examined predictive factors for persistent PVCs on initial Holter electrocardiogram (ECG) in pediatric patients with asymptomatic PVC. Methods and Results: The initial Holter ECG findin...
Article
Left main coronary artery ostial atresia (LMCAOA) is an extremely rare condition. Here, we report the case of a 14-year-old boy with Noonan syndrome-like disorder in whom LMCAOA was detected following cardiopulmonary arrest. The patient had been diagnosed with Noonan syndrome-like disorder with a pathogenic splice site variant of CBL c.1228-2 A > G...
Article
Full-text available
Background: Cardiac calmodulinopathy, characterized by a life-threatening arrhythmia and sudden death in the young, is extremely rare and caused by genes encoding calmodulin, namely calmodulin 1 (CALM1), CALM2, and CALM3. Methods and Results: We screened 195 symptomatic children (age 0–12 years) who were suspected of inherited arrhythmias for 48...
Article
Full-text available
Barth syndrome (BTHS) is an X-linked disorder characterized by cardiomyopathy, skeletal myopathy, and 3-methylglutaconic aciduria. The causative pathogenic variants for BTHS are in TAZ, which encodes a putative acyltransferase named tafazzin and is involved in the remodeling of cardiolipin in the inner mitochondrial membranes. Pathogenic variants i...
Article
Atrial tachyarrhythmias (ATAs), which may occur after tetralogy of Fallot (TOF) surgery, can cause sudden cardiac death. However, ATAs may also develop in response to electrical substrates. This study aims to examine the predictive factors for ATAs by identifying electrical substrates in the atrium obtained from 12-lead electrocardiogram in patient...
Article
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Idiopathic pulmonary arterial hypertension (PAH) is a rare, progressive disease affecting the pulmonary arteries. Epoprostenol, a synthetic prostaglandin analog, is the most potent pharmacological treatment modality used in patients with PAH. However, it requires continuous intravenous infusion, which negatively impacts the patient’s quality of lif...
Article
Full-text available
We report the case of an adult who had a cardiac arrest in the setting of pulmonary hypertension and a previously repaired intermediate atrioventricular septal defect, with left main coronary trunk stenosis due to dilatation of the main pulmonary artery. In patients with pulmonary hypertension exhibiting anginal symptoms, it is advisable to perform...
Article
The risk factors and the appropriate interventions for perioperative junctional ectopic tachycardia (JET) in congenital heart disease (CHD) surgery have not been sufficiently investigated despite the severity of this complication. This study aimed to examine the risk factors and interventions for perioperative JET. From 2013 to 2020, 1062 surgeries...
Article
Biplane Area-Length (AL) method by left ventriculography (LVG) has been widely adopted as a standard method to estimate left ventricular volume. However, we have experienced difficulties in adopting the value by AL method for the children with Tetralogy of Fallot (TOF) due to the discrepancy among volumetric modalities. This study validated some li...
Article
Twin atrioventricular nodes (AVNs) associated with complex tachycardias has been described in a heart with discordant atrioventricular (AV) connection. The present case had twin AVNs (approximately 3 o'clock and 8 o'clock position) of sole AV annulus with absent right AV connection. It is a first report demonstrated the twin AVNs identified at atyp...
Article
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Respiratory syncytial virus (RSV) is a common pathogen that causes extremely severe respiratory symptoms in the first few weeks and months of life. In infants with cardiopulmonary diseases, RSV infections have a significant clinical impact. Palivizumab, a humanised monoclonal antibody for RSV, has been shown to significantly reduce the rate of hosp...
Article
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Background We report a rare case of left ventricular inflow obstruction from a branch of the left circumflex coronary artery to the right atrium caused by a coronary arteriovenous fistula (CAVF) in a young Japanese male child. Case presentation The patient was diagnosed with CAVF following a heart murmur shortly after birth. The left-to-right shun...
Article
A case of hypertrophic cardiomyopathy in the transition from childhood to adulthood, which was low risk by the conventional risk assessment model, medium risk by the adult risk prediction model, and high risk by the paediatric risk prediction model, was inserted an implantable cardioverter-defibrillator. Three years post-implantation, the patient w...
Article
Full-text available
Background: Total anomalous pulmonary venous connection (TAPVC) is a critical congenital heart disease for which emergency surgery is required after birth. In cases of no intervention, TAPVC is associated with a high mortality rate in the first year of life. Although foetal echocardiographic techniques for diagnosing TAPVC have improved, TAPVC rem...
Article
We describe a 15-year-old girl who underwent intraoperative catheter ablation for the ventricular tachycardia associated with Ebstein’s anomaly with functional pulmonary atresia and a small right ventricle (RV) after Fontan surgery. The computed tomography showed the dilated right atrium and RV due to the failure of RV plication. The activation map...
Article
We describe a 15-year-old girl who underwent intraoperative catheter ablation for the ventricular tachycardia associated with Ebstein’s anomaly with functional pulmonary atresia and a small right ventricle (RV) after Fontan surgery. The computed tomography showed the dilated right atrium and RV due to the failure of RV plication. The activation map...
Article
Full-text available
Mitochondrial cardiomyopathy (MCM) is usually recognized as one of the phenotypes of systemic mitochondrial disease. However if there are no cardiac symptoms, it is difficult to make a definite diagnosis because of various cardiovascular phenotypes and no diagnostic criteria in pathological examination. To add myocardial pathology to the diagnostic...
Article
We described a 15‐year‐old boy who underwent the catheter ablation for the nodoventricular (NV) tachycardia that had difficulty in differentiation from atrioventricular nodal reentrant tachycardia with upper common pathway. The modification of the fast pathway revealed an anterograde conduction of the NV fiber. We successfully performed the cathete...
Article
Childhood obstructive sleep apnoea syndrome (OSAS) secondary to adenoid hyperplasia is known to give rise to pulmonary hypertension. However, we present a case of a toddler with pulmonary hypertension (PH) and right heart failure due to OSAS, the cause of which is difficult to identify. After the patient underwent an adenotonsillectomy, OSAS disapp...
Article
A 2-year-old male with right isomerism was referred for supraventricular tachycardias. Atrial pacing study revealed that anterograde conduction was only through the posterior atrioventricular node. During the mapping of ventriculoatrial conduction, we identified a sharp potential resembling a His-bundle electrogram with a decremental property at th...
Article
Full-text available
Aims Mitochondrial cardiomyopathy (MCM) is difficult to make a definite diagnosis because of various cardiovascular phenotypes and no diagnostic criteria in the pathology examination. We aim to add myocardial pathology to the diagnostic criteria for mitochondrial respiratory chain disorders. Methods Quantitative analysis of mitochondria using elec...
Article
Congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) is one of the major complications in patients with CHD. A timely closure of the left-to-right shunt will generally result in the normalization of the pulmonary hemodynamics, but a few patients have severe prognosis in their early childhood. We hypothesized that wide-rangi...
Article
Recent reports suggested that cardiopulmonary bypass (CPB) time is one of the risk factors for postoperative complications after Fontan conversion. Although Fontan conversion may be performed for the patients with hepatic fibrosis after initial Fontan procedure, there is no predictive indicator regarding the liver function associated with hemostasi...
Article
Background: Genetic studies have indicated possible involvement of the upregulated calcium-nuclear factor of activated T cells pathway in the pathogenesis of Kawasaki disease. We aimed to assess safety and efficacy of ciclosporin, an immunosuppressant targeting this pathway, for protection of patients with Kawasaki disease against coronary artery...
Article
Background: Long QT syndrome (LQTS) can cause syncope, ventricular tachycardia/fibrillation, and/or sudden death. Several disease-causing gene mutations of ion channels have been identified. Occasionally, double gene mutations in one or two genes have been detected. However, it remains unclear whether children who are double mutation carriers exhib...
Article
Background: Elevated venous pressure is thought to play a pivotal role in the development of liver fibrosis/dysfunction, an important complication after Fontan surgery, particularly that for the atriopulmonary connection (APC). We hypothesized that right atrial volume (RAv) rather than RA pressure (RAp) is important for the progression of liver fib...
Article
Concomitant with advances in genetic diagnosis of congenital long QT syndrome (LQTS), results of genetic analysis in patients with LQTS are becoming increasingly important in clinical practice to design appropriate treatment strategies. The present study reports an LQTS family. The proband was a female patient who exhibited repetitive exercise-indu...
Article
Catheter ablation of ventricular tachycardia is often difficult in patients after surgery for CHD. In patients with a ventricular septal defect patch, it is necessary to decide which ventricular side is appropriate for catheter ablation. In this article, we report a case of successful point catheter ablation of re-entrant ventricular tachycardia. I...
Article
Elevated right atrial (RA) pressure and progressive RA dilation are thought to play pivotal roles in the development of late complications after atriopulmonary connection (APC) Fontan surgery. However, no clear cut-off value for RA pressure or RA volume has been determined for stratifying the risk of developing Fontan complications. We hypothesized...
Article
Objective: It has been recently reported that vitamin D deficiency may contribute to systemic illnesses that accompany chronic heart failure. These reports also suggest the serum levels of parathormone, which activates vitamin D in the liver, can be a useful marker of heart failure. This study was designed to evaluate the clinical implications of...
Article
Full-text available
Long QT syndrome (LQTS) can cause syncope, ventricular fibrillation, and death. Recently, several disease-causing mutations in ion channel genes have been identified, and compound mutations have also been detected. It is unclear whether children who are carriers of compound mutations exhibit a more severe phenotype than those with single mutations....
Article
The substrate for atrial fibrillation (AF) in young patients with congenital heart disease could be heterogeneous. We report a 24-year old patient with persistent AF having the dilated right atrium following surgical repair of congenital heart disease demonstrating sustained pulmonary vein tachycardia (PVT) after pulmonary vein isolation. The susta...
Article
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Liver cirrhosis (LC), which may result in hepatic failure or cancer, has been reported in patients after Fontan procedure. The purpose of this study was to clarify the frequency and histological characteristics of LC, and to evaluate the risk factors and serological markers of LC with Fontan circulation. Retrospective review of contrast-enhanced CT...
Article
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Background: Incidence of supraventricular tachycardia (SVT) is less in patients following total cavo-pulmonary connection (TCPC) than in classical Fontan (atrio-pulmonary connection; APC). However, in case SVT occurs following TCPC, catheter ablation (CA) is extremely difficult because of no transvenous entry to the heart. The purpose of this study...
Article
Full-text available
Barth syndrome (OMIM #302060) (BTHS) is an X-linked disorder of lipid metabolism characterized by skeletal myopathy, neutropenia, growth delay, and cardiomyopathy. It is caused by mutations in the tafazzin gene (TAZ), which lead to decreased production of an enzyme required to produce cardiolipin, a component of the inner mitochondrial membrane nec...
Article
Full-text available
Barth syndrome (BTHS) is an X-linked disorder characterized by skeletal myopathy, neutropenia, growth delay, and cardiomyopathy. It is caused by mutations in the tafazzin gene (TAZ). Although early diagnosis is critical to prevent the progression of heart failure, this disease remains unrecognized when heart failure is not clinically significant. H...

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