Publications by authors named "Fukiko Ichida"
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.
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- Left ventricular noncompaction (LVNC) is a hereditary heart condition marked by unusual heart muscle structure, and this study specifically focused on biventricular noncompaction (BiVNC) in children to understand its clinical characteristics and genetic factors.
- The research involved 234 pediatric patients and revealed that BiVNC often leads to serious complications, including a higher incidence of congenital heart disease and reduced survival rates compared to other heart conditions.
- Findings indicated that patients with BiVNC frequently exhibited left ventricular dysfunction and a notable percentage had genetic variants linked to mitochondrial and developmental issues, emphasizing the need for thorough genetic screening for better patient outcomes.
Background: Left ventricular hypertrophy (LVH) is a well-recognized cardiac dysfunction in infants of mothers with gestational diabetes mellitus (GDM). Left ventricular noncompaction (LVNC) is a cardiomyopathy that is morphologically characterized by numerous prominent trabeculations and deep intertrabecular recesses on cardiovascular imaging. However, there have been no case reports on neonates of mothers with GDM showing LVH and LVNC.
View Article and Find Full Text PDFBackground: Idiopathic bradyarrhythmia is considered to be due to pathological degeneration of the cardiac conduction system (CCS) during aging. There appears to have been no comprehensive genetic investigations in patients with idiopathic bradyarrhythmia.
Methods and results: Ten autopsy cases with advanced bradyarrhythmia (6 men and 4 women; age: 70-94 years, 81.
Left ventricular noncompaction: a disorder with genotypic and phenotypic heterogeneity-a narrative review.
Cardiovasc Diagn Ther
August 2022
Background And Objective: Left ventricular noncompaction (LVNC) is a cardiomyopathy characterized by excessive trabecular formation and deep recesses in the ventricular wall, with a bilaminar structure consisting of an endocardial noncompaction layer and an epicardial compacted layer. Although genetic variants have been reported in patients with LVNC, understanding of LVNC and its pathogenesis has not yet been fully elucidated. We addressed the latest findings on genes reported to be associated with LVNC morphogenesis and possible pathologies to understand the diverse spectrum between genotype and phenotype in LVNC.
View Article and Find Full Text PDFObjective: Left ventricular non-compaction (LVNC) is morphologically characterised by excessive trabeculations and deep recesses in the ventricular wall. The risk of thromboembolic disease in the paediatric patients with LVNC has not been clearly established. We conducted this systematic review to evaluate the prevalence and incidence of thromboembolism (TE) in paediatric and adult patients with LVNC and searched for risk factors for TE to explore management strategies.
View Article and Find Full Text PDFBackground: Isolated right ventricular hypoplasia (IRVH), not associated with severe pulmonary or tricuspid valve malformation, is a rare congenital myocardial disease. This study aims to evaluate the clinical status and outcome of IRVH.
Methods: A systematic search of keywords on IRVH was conducted.
We encountered cases in which the anticoagulant effects of warfarin (CYP2C9 substrate) were reversibly attenuated by the concomitant administration of rifampicin or bosentan, which are potent pregnane X receptor (PXR) ligands. The purpose of the present study is to report the previous case with rifampicin, and to evaluate the changes in the warfarin anticoagulant effects when withdrawing or switching bosentan treatment. The former is a case study of a 4-year-old girl undergoing warfarin treatment.
View Article and Find Full Text PDFBackground: Kawasaki disease (KD) is a systemic vasculitis that is currently the most common cause of acquired heart disease in children. However, its etiology remains unknown. Long non-coding RNAs (lncRNAs) contribute to the pathophysiology of various diseases.
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- Neonates and infants with coronary artery fistulas (CAFs) are rare, and this study examines their outcomes post-treatment through a retrospective review.
- Out of 48 patients analyzed, most had proximal CAFs, with high procedural success rates for both transcatheter (92%) and surgical closure (94%).
- Follow-up results showed that while a majority had optimal coronary remodeling, those with distal CAFs experienced more complications, including symptomatic and asymptomatic thrombosis.
Background: The usefulness of electrocardiographic (ECG) voltage criteria for diagnosing hypertrophic cardiomyopathy (HCM) in pediatric patients is poorly defined.
Methods and results: ECGs at the 1st grade (mean [±SD] age 6.6±0.
Chronic myocarditis is a prolonged inflammatory condition in the myocardium and its histological manifestation is defined by the presence of an inflammatory infiltrate. Chronic myocarditis has not been well known and its treatment of chronic myocarditis has not been established. Primary outcome of this study was to assess the efficacy of immunomodulatory treatment in addition to conventional treatment, and secondary outcomes were to clarity the prognosis of natural history of chronic myocarditis and incidence of chronic myocarditis in patients with dilated cardiomyopathy (DCM).
View Article and Find Full Text PDFBackground: Left ventricular noncompaction (LVNC) is morphologically characterized by numerous prominent trabeculations and a severely thickened, two-layered myocardium. The fetal onset of LVNC has rarely been described.
Methods and results: We conducted nationwide retrospective surveys on fetal cardiomyopathy (CM) in Japan from 2010 to 2016, from which 38 fetal patients with CM were enrolled, including 16 patients with LVNC.
Background: Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by prominent ventricular trabeculations on cardiovascular imaging. Acquired reversible LVNC has not been reported in pediatrics without a genetic background.
Case Presentation: A 9-year-old girl with a ventriculoperitoneal (VP) shunt for neonatal posthemorrhagic hydrocephalus was referred due to exacerbation of hydrocephalus caused by VP shunt dysfunction.
Article Synopsis
- Restrictive cardiomyopathy (RCM) is a rare heart condition in kids that leads to poor filling of the heart and has a generally bad outlook, often accompanied by abnormal P-waves in ECG.
- This study analyzed 17 ECGs from children diagnosed with idiopathic RCM across Japan, comparing them to a control group of nearly 35,000 students to identify key ECG characteristics.
- The researchers found that the sum of certain amplitudes in lead V1 (P1 + P2 V1) is a strong indicator of RCM, proposing a cutoff value of >200 µV to help detect the disease in asymptomatic or early-stage cases during school screenings.
Background: Left ventricular noncompaction (LVNC) is a hereditary cardiomyopathy, associated with high morbidity and mortality, but the role of genetics in cases of fetal-onset has not been fully evaluated. The goal of this study was to identify the genetic background in LVNC fetal-onset patients using next-generation sequencing (NGS).
Methods: Thirty-three fetal-onset Japanese probands with LVNC (20 males and 13 females) were enrolled.
This study was performed for a better understanding of the pharmacokinetics of sildenafil (SIL) and N-desmethyl sildenafil (DMS) in 13 children treated in the intensive care unit (ICU). Blood samples were taken periodically after the first oral administration of SIL (0.5 mg/kg).
View Article and Find Full Text PDFBackground: T-wave inversion (TWI) is not considered useful for diagnosing pediatric arrhythmogenic right ventricular cardiomyopathy (ARVC), because right precordial TWI in ARVC resembles a normal juvenile pattern.
Objectives: The aims of this study were to clarify the electrocardiographic (ECG) characteristics of pediatric ARVC to distinguish those patients from healthy children.
Methods: Between 1979 and 2017, 11 ARVC patients under 18 years old were registered and compared with school screening ECGs from 48,401 healthy children.
Article Synopsis
- Mutations in nuclear envelope genes linked to Emery-Dreifuss muscular dystrophy can cause cardiac issues, but some mutations result in heart problems without skeletal symptoms.
- A study analyzed families with cardiac conduction diseases and identified three specific mutations associated with progressive atrial arrhythmias and left ventricular noncompaction (LVNC).
- The findings suggest that cardiac emerinopathy presents as a new condition characterized by atrial standstill, LVNC, and a heightened risk of thromboembolism, distinguishing it from traditional Emery-Dreifuss muscular dystrophy.
Background: Left ventricular noncompaction (LVNC) is a hereditary type of cardiomyopathy. Although it is associated with high morbidity and mortality, the related ion channel gene variants in children have not been fully investigated. This study aimed to elucidate the ion channel genetic landscape of LVNC and identify genotype-phenotype correlations in a large Japanese cohort.
View Article and Find Full Text PDFBackground: TBX5 is a transcription factor that has an important role in development of heart. TBX5 variants in the region encoding the T-box domain have been shown to cause cardiac defects, such as atrial septal defect or ventricular septal defect, while TBX5 variants have also been identified in a few cardiomyopathy patients and considered causative. We identified a TBX5 variant (c.
View Article and Find Full Text PDFBackground: Left ventricular noncompaction (LVNC) is a hereditary cardiomyopathy that is associated with high morbidity and mortality rates. Recently, LVNC was classified into several phenotypes including congenital heart disease (CHD). However, although LVNC and CHD are frequently observed, the role and clinical significance of genetics in these cardiomyopathies has not been fully evaluated.
View Article and Find Full Text PDFLeft ventricular noncompaction (LVNC) is a hereditary cardiomyopathy and is associated with high morbidity and mortality. However, the role and significance of school screening for LVNC have not been fully elucidated. In this multicenter, retrospective cohort study, a total of 105 children with LVNC were included from 2000 to 2017.
View Article and Find Full Text PDFBackground: Both congenital heart disease (CHD) and very-low birthweight (VLBW) infants are at a very high risk of neurodevelopmental delay. We investigated neurological development at 3 years in pediatric patients with CHD after surgical intervention, those of VLBW, and healthy controls.
Methods: We enrolled pediatric patients with CHD (n = 67), VLBW (n = 67), and healthy controls (n = 81).