277 results on '"Kei, Inai"'
Search Results
52. Clinical implications of eicosapentaenoic acid/arachidonic acid ratio (EPA/AA) in adult patients with congenital heart disease
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Hirofumi Tomimatsu, Tokuko Shinohara, Kei Inai, Toshio Nakanishi, and Miki Kanoh
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Adult ,Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Heart disease ,Coronary Artery Disease ,030204 cardiovascular system & hematology ,Gastroenterology ,Coronary artery disease ,03 medical and health sciences ,0302 clinical medicine ,Japan ,Risk Factors ,Internal medicine ,Fatty Acids, Omega-3 ,Humans ,Medicine ,Prospective Studies ,030212 general & internal medicine ,Prospective cohort study ,Heart Failure ,Arachidonic Acid ,business.industry ,Incidence ,Incidence (epidemiology) ,Hazard ratio ,Atherosclerosis ,Prognosis ,medicine.disease ,Eicosapentaenoic acid ,Cardiac surgery ,Eicosapentaenoic Acid ,Biochemistry ,Heart failure ,Female ,Cardiology and Cardiovascular Medicine ,business ,Biomarkers ,Follow-Up Studies - Abstract
Recent studies showed that a low ratio between the levels of eicosapentaenoic acid and those of arachidonic acid (EPA/AA) is associated with higher incidence of coronary artery disease and poor prognosis of heart failure, arrhythmia, and cardiac sudden death. However, the clinical implications of EPA/AA in adult patients with congenital heart disease remain unclear. We aimed to assess the prognostic value of EPA/AA regarding cardiac events in adult patients with congenital heart disease. We measured the serum levels of eicosapentaenoic acid and arachidonic acid in 130 adult patients (median age, 31 years) stratified into two groups according to their EPA/AA (low, ≤0.22; high, >0.22). We prospectively analyzed the association between EPA/AA and incidence of cardiac events during a mean observation period of 15 months, expressed in terms of hazard ratio (HR) with 95% confidence interval (95% CI). In the subgroup of patients with biventricular circulation (2VC) (n = 76), we analyzed the same clinical endpoints. In our study population, EPA/AA was not associated with the incidence of arrhythmic events (HR, 1.52; 95% CI, 0.82-2.85; p = 0.19), but low EPA/AA was a predictor of heart failure hospitalization (HR, 2.83; 95% CI, 1.35-6.30; p
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- 2017
53. Significance of right atrial tension for the development of complications in patients after atriopulmonary connection Fontan procedure: potential indicator for Fontan conversion
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Takuo Furukawa, Atsuhito Takeda, Hideaki Senzaki, Tokuko Shinohara, Kei Inai, Kohta Takei, Toshio Nakanishi, Hirokuni Yamazawa, and Gaku Izumi
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Adult ,Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Adolescent ,medicine.medical_treatment ,Hemodynamics ,Pulmonary Artery ,030204 cardiovascular system & hematology ,Fontan Procedure ,Fontan procedure ,Young Adult ,03 medical and health sciences ,Postoperative Complications ,0302 clinical medicine ,Japan ,Humans ,Medicine ,Heart Atria ,030212 general & internal medicine ,Child ,Retrospective Studies ,Cardiac catheterization ,medicine.diagnostic_test ,business.industry ,Area under the curve ,Arrhythmias, Cardiac ,Thrombosis ,Vascular surgery ,medicine.disease ,Cardiac surgery ,Surgery ,ROC Curve ,Angiography ,Female ,Cardiology and Cardiovascular Medicine ,business - Abstract
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 that RA tension, which incorporates information about both RA pressure and volume, might help predict the risk of developing complications. We retrospectively studied 51 consecutive APC Fontan patients (median postoperative period 14 years). RA tension was computed from the RA pressure and RA radius, which was calculated from RA volume measured by RA angiography. The correlation between the cardiac catheterization hemodynamic data and the complications of APC Fontan was investigated. Of the 51 patients, 28 had complications, including liver fibrosis (n = 28), arrhythmia (n = 8), protein-losing enteropathy (n = 1), and RA thrombosis (n = 1). Among the hemodynamic data, RA volume and RA tension, but not RA pressure, were significantly higher in patients with complications than in those without (P = 0.004 and P = 0.001, respectively). The cut-off level for RA tension to predict Fontan complications was 26,131 dyne/cm by receiver operating characteristic curve (area under the curve 0.79, sensitivity 71.4%, and specificity 73.9%). The present study demonstrated the significance of RA tension rather than high venous pressure for the development of Fontan complications. Amid the uncertainty about clinical outcomes, the present results, subject to further validation, may contribute to the indications for Fontan conversion.
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- 2017
54. Management of Adult Patients with Fontan Physiology
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Kei Inai
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medicine.medical_specialty ,Adult patients ,business.industry ,Pharmaceutical Science ,030204 cardiovascular system & hematology ,Fontan physiology ,Surgery ,03 medical and health sciences ,0302 clinical medicine ,Complementary and alternative medicine ,medicine ,Pharmacology (medical) ,030212 general & internal medicine ,business - Published
- 2017
55. Non-physiological Aortic Flow and Aortopathy in Adult Patients with Transposition of the Great Arteries after the Jatene Procedure: A Pilot Study Using Echo Planar 4D Flow MRI.
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Yumi Shiina, Kei Inai, and Michinobu Nagao
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AORTA abnormalities ,AORTIC valve insufficiency ,TRANSPOSITION of great vessels ,VORTEX motion ,MAGNETIC resonance imaging - Abstract
Purpose: Dilated aortic root and ascending aorta (AAO) with progressive aortic regurgitation is a wellknown sequela after arterial switch operation (ASO) in adults with transposition of the great arteries (TGA). We aimed to quantitatively assess aortic flow profiles in adults with TGA after ASO (Jatene procedure with LeCompte maneuver) using echo planar imaging (EPI) 4D flow MRI. Methods: Prospectively, 9 consecutive adults (30.2 ± 6.6 years) after ASO (Jatene operation with LeCompte technique), 13 consecutive adults (34.3 ± 7.2 years) after the atrial switch operation with Senning procedure, and 8 age-matched control patients, who underwent turbo field echo (TFE) EPI 4D flow MRI (average scan time of approximately 4 min), were enrolled. Results: TGA after ASO showed a markedly dilated sinus of Valsalva, compared to TGA after atrial switch operation (26.6. ± 4.9 vs. 18.6. ± 1.5 mm/cm2). Vorticity, helicity, wall share stress (WSS), and energy loss (EL) in the aortic root and the AAO in TGA were greater than in the controls. Vorticity, helicity, WSS, and EL in the aortic root and the AAO were also greater in TGA after ASO than after atrial switch operation. More acute aortic arch angle correlated with greater vorticity of the aortic root, and the significant diameter ratio of the sinus of Valsalva and the AAO was relevant to greater vorticity, helicity, and EL in TGA after ASO. Conclusion: A non-physiological blood flow pattern of the aortic root was identified in TGA adults after the ASO (Jatene procedure with LeCompte maneuver). Missing spiral looping of the great arteries and the unique structure after the Jatene procedure may play an adjunctive role in promoting aortopathy. The evaluation of aortic flow profile using EPI 4D flow MRI may be useful for risk stratification for aortopathy in this population. [ABSTRACT FROM AUTHOR]
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- 2021
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56. Blood coagulation abnormalities and the usefulness of D-dimer level for detecting intracardiac thrombosis in adult Fontan patients
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Tokuko Shinohara, In-Sam Park, Toshio Nakanishi, Kei Inai, and Daiji Takeuchi
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Adult ,Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Heart Diseases ,medicine.medical_treatment ,Long Term Adverse Effects ,030204 cardiovascular system & hematology ,Fontan Procedure ,Fibrin Fibrinogen Degradation Products ,Fontan procedure ,03 medical and health sciences ,0302 clinical medicine ,Japan ,Internal medicine ,parasitic diseases ,Fibrinolysis ,D-dimer ,medicine ,Humans ,cardiovascular diseases ,030212 general & internal medicine ,Platelet activation ,Thrombus ,Blood Coagulation ,Retrospective Studies ,Prothrombin time ,medicine.diagnostic_test ,business.industry ,Area under the curve ,Thrombosis ,medicine.disease ,cardiovascular system ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business ,Biomarkers - Abstract
Background Coagulation abnormality is associated with a high incidence of intracardiac thrombus (ICT) and systemic thromboembolism in Fontan patients. The biomarker for detecting ICT is currently unknown. Methods We retrospectively investigated the underlying coagulation abnormality and useful biomarkers to screen for ICT in adult Fontan patients. We measured various biomarkers of blood coagulation, fibrinolysis, and platelet activity in 122 Fontan patients (Fontan group: median age [P25–P75]: 27 [20–34] years) and compared them to those in 50 patients with atrial septal defect (ASD group: 31 [24–40] years). Results Regardless of whether the patient had ICT, the Fontan group showed significantly lower levels of antithrombin III, thrombomodulin, and α2-antiplasmin; lower protein C and protein S activities; and significantly higher levels of thrombin-antithrombin complex and α2-plasmin inhibitor complex than the ASD group. Among various biomarkers, D-dimer level measured by using latex immunoassay was significantly higher in the patients with ICT (thrombus group: n =21) than in the patients without ICT (non-thrombus group: n =101). Fifteen (26%) of 57 patients on warfarin achieved prothrombin time international normalized ratios (PT-INRs) of >2. The proportion of patients with PT-INRs of >2 tended to be lower in the thrombus group than in the non-thrombus group (13% vs 31%). Persistent atrial arrhythmia and D-dimer level were significant risk factors associated with ICT formation in the multivariate analysis (persistent atrial arrhythmia: hazard ratio [HR], 6.89; 95% confidence interval [CI], 1.44–34.5; D-dimer: HR, 0.29; 95% CI, 0.13–0.50). Receiver-operating characteristic curve analysis revealed that the appropriate cutoff D-dimer level for screening for ICT was 1.8μg/mL (area under the curve, 0.94), with a negative predictive value of 95%. Conclusions In the adult Fontan patients, blood coagulation abnormalities existed regardless of the absence of ICT. D-dimer level may be a useful biomarker for screening for ICT in adult Fontan patients.
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- 2016
57. Prognostic predictive value of gene mutations in Japanese patients with hypertrophic cardiomyopathy
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Masaya Sugimoto, Hiroki Sato, Tsutomu Nishizawa, Shigeaki Nonoyama, Yoichi Kawamura, Shigeru Tateno, Yoshiyuki Furutani, Ayako Chida, Shinya Fuji, Masatoshi Kawana, Fukiko Ichida, Toshio Nakanishi, Mitsuyo Shimada, Mamoru Ayusawa, Takashi Soga, Hirokuni Yamazawa, Kenji Kuraishi, Masako Fujiwara, Rumiko Matsuoka, Mitsuhiro Nishimura, Kei Inai, Eriko Shimada, Michiko Furutani, Shigetoyo Kogaki, and Jun Ishihara
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Adult ,Male ,0301 basic medicine ,Heterozygote ,medicine.medical_specialty ,Adolescent ,Kaplan-Meier Estimate ,macromolecular substances ,030204 cardiovascular system & hematology ,Gene mutation ,Sudden death ,Young Adult ,03 medical and health sciences ,0302 clinical medicine ,Japan ,Predictive Value of Tests ,Internal medicine ,medicine ,Humans ,cardiovascular diseases ,Family history ,Child ,Genetic Association Studies ,Myosin Heavy Chains ,business.industry ,Hypertrophic cardiomyopathy ,Cardiomyopathy, Hypertrophic ,Middle Aged ,Vascular surgery ,medicine.disease ,Pedigree ,Cardiac surgery ,Death, Sudden, Cardiac ,Phenotype ,030104 developmental biology ,Case-Control Studies ,Mutation ,Mutation (genetic algorithm) ,cardiovascular system ,Cardiology ,Regression Analysis ,Female ,MYH7 ,Carrier Proteins ,Cardiology and Cardiovascular Medicine ,business ,Cardiac Myosins ,Follow-Up Studies - Abstract
Although some studies have attempted to find useful prognostic factors in hypertrophic cardiomyopathy (HCM), those results are not fully helpful for use in actual clinical practice. Furthermore, several genetic abnormalities associated with HCM have been identified. However, the genotype-phenotype correlation in HCM remains to be elucidated. Here, we attempted to assess patients with different types of gene mutations causing HCM and investigate the prognosis. A total of 140 patients with HCM underwent a screening test for myofilament gene mutations by direct sequencing of eight sarcomeric genes. Patients with a single mutation in cardiac troponin T, cardiac troponin I, α-tropomyosin, and regulatory and essential light chains were excluded from the study because the number of cases was too small. The clinical presentations and outcomes of the remaining 127 patients with HCM, 31 β-myosin heavy chain (MYH7) mutation carriers, 19 cardiac myosin-binding protein C (MYBPC3) mutation carriers, and 77 mutation non-carriers were analyzed retrospectively. MYBPC3 mutation carriers had a high frequency of ventricular arrhythmia and syncope. Kaplan-Meier curves revealed no significant difference in prognosis among the three groups, but a lack of family history of sudden death (SD) and a past history of syncope were significantly related to poor prognosis. An absence of family history of SD and past history of syncope are useful prognostic factors in patients with HCM. MYH7 and MYBPC3 mutations did not significantly influence prognosis compared to non-carriers. However, patients with the MYBPC3 mutation should be closely followed for the possibility of SD.
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- 2016
58. Potential Value of Native T1 Mapping in Symptomatic Adults with Congenital Heart Disease: A Preliminary Study of 3.0 Tesla Cardiac Magnetic Resonance Imaging
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Kota Taniguchi, Tatsunori Takahashi, Kei Inai, Michinobu Nagao, and Yumi Shiina
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Adult ,Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Heart disease ,Heart Ventricles ,030204 cardiovascular system & hematology ,03 medical and health sciences ,0302 clinical medicine ,Cardiac magnetic resonance imaging ,Predictive Value of Tests ,Internal medicine ,medicine ,Humans ,Tetralogy of Fallot ,Retrospective Studies ,Heart Failure ,medicine.diagnostic_test ,business.industry ,Myocardium ,Vascular surgery ,Middle Aged ,medicine.disease ,Brain natriuretic peptide ,Fibrosis ,Magnetic Resonance Imaging ,Cardiac surgery ,medicine.anatomical_structure ,030228 respiratory system ,Ventricle ,Heart failure ,Case-Control Studies ,Pediatrics, Perinatology and Child Health ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business ,Biomarkers - Abstract
The native T1 value at 3.0 Tesla is a sensitive marker of diffuse myocardial damage. We evaluated the clinical usefulness of native T1 mapping in symptomatic adults with congenital heart disease (CHD), particularly in the systemic right ventricle (RV). Prospectively, 45 consecutive symptomatic adults with CHD were enrolled: 20 with systemic RV and 25 with tetralogy of Fallot underwent cardiac magnetic resonance (CMR) imaging at 3.0 Tesla. The Modified Look-Locker Inversion recovery sequence was used for T1 mapping. Cardiovascular events in the systemic RV were defined as heart failure and tachyarrhythmia. Brain natriuretic peptide (BNP) and indexed systemic ventricular end-diastolic volume were significantly higher in the systemic RV group. The native T1 value and extracellular volume (ECV) of the septal and lateral walls were higher in the systemic RV group, suggesting high impairment of the myocardium in the systemic RV group. There was a strong correlation between the native T1 value and ECV of the septum (r = 0.58, P = 0.03) and lateral wall (r = 0.56, P = 0.046) in the systemic RV group. Seven patients with systemic RV had cardiovascular events. In univariate logistic regression analysis, BNP and native T1 values of the insertion point were important for predicting cardiovascular events. The native T1 value at 3.0 Tesla may be a sensitive, contrast-free, and non-invasive adjunct marker of myocardial damage in CHD and predictive of cardiovascular events in the systemic RV.
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- 2019
59. The relationship between extracellular volume fraction in symptomatic adults with tetralogy of Fallot and adverse cardiac events
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Koichiro Niwa, Masateru Kawakubo, Kota Taniguchi, Michinobu Nagao, Kei Inai, Yumi Shiina, and Tatsunori Takahashi
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Adult ,Male ,medicine.medical_specialty ,030204 cardiovascular system & hematology ,03 medical and health sciences ,Young Adult ,0302 clinical medicine ,Internal medicine ,medicine ,Humans ,030212 general & internal medicine ,Adverse effect ,Tetralogy of Fallot ,Ejection fraction ,medicine.diagnostic_test ,business.industry ,Hazard ratio ,Magnetic resonance imaging ,Stroke Volume ,Middle Aged ,medicine.disease ,Cardiac surgery ,medicine.anatomical_structure ,Ventricle ,Heart failure ,Case-Control Studies ,cardiovascular system ,Cardiology ,Ventricular Function, Right ,Female ,Cardiology and Cardiovascular Medicine ,business - Abstract
To investigate the relationship between the extracellular volume fraction (ECV) measured using cardiac magnetic resonance (CMR) T1 mapping and cardiac events in symptomatic adults with tetralogy of Fallot (TOF).A total of 60 consecutive symptomatic adults (35.4±13.8 years old) with repaired TOF who were in New York Heart Association functional class 2-4 were prospectively enrolled. Native T1 values and ECV of the free walls of the right ventricle (RV), the left ventricle, and the ventricular septum were obtained by CMR T1 mapping using a 3.0T scanner and the saturation recovery method. Correlations between T1 mapping measurements, conventional parameters, and predictors of adverse cardiac events were analyzed. The patients with episodes of only atrial arrhythmia were excluded.Significant correlations were observed between RV functional parameters, RV-ECV, and septum-ECV. Follow-up period was 747.1±315 days and 13 patients had cardiac events: Acute heart failure and/or sustained ventricular tachycardia occurred in 8 patients and 5 patients underwent cardiac surgery. Cox-hazard analysis revealed that septum-ECV and RV-ECV were predictors of adverse events (hazard ratio of septum-ECV and RV-ECV: 1.41 and 1.19, 95% CI: 1.05-1.89, 1.004-1.41 with p-values of 0.02, and 0.045, respectively). Adults with septum-ECV29.0% and RV ejection fraction (EF)45.0% were more likely to experience cardiac events (log rank test: p0.043).RV-ECV and septum-ECV correlate with RV functional parameters. Biventricular ECVs can be predictors of adverse cardiac events in adults with TOF. In particular, the combination of septum-ECV and RVEF was a useful predictor, compared to the use of a single CMR parameter.
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- 2019
60. Clinical Significance of Central Venous Pressure During Exercise After Fontan Procedure
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Hisashi Sugiyama, Kei Inai, Seiji Asagai, Gen Harada, and Eriko Shimada
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Adult ,Male ,medicine.medical_specialty ,Time Factors ,Central Venous Pressure ,medicine.medical_treatment ,Cardiac index ,030204 cardiovascular system & hematology ,Fontan Procedure ,Fontan procedure ,03 medical and health sciences ,chemistry.chemical_compound ,Young Adult ,0302 clinical medicine ,Internal medicine ,Medicine ,Humans ,Clinical significance ,Exercise ,Retrospective Studies ,Creatinine ,business.industry ,Central venous pressure ,Vascular surgery ,Brain natriuretic peptide ,Cardiac surgery ,030228 respiratory system ,chemistry ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business - Abstract
Various late complications are associated with the Fontan procedure. In patients who undergo the Fontan procedure, the central venous pressure (CVP) tends to be higher than normal. However, the relationship between CVP (at rest and during exercise) and late complications associated with the Fontan procedure is unknown. Thirty-four patients who underwent the Fontan procedure were enrolled in this study. The median age was 19.3 years, and the median time after the Fontan procedure was 12.7 years. With exercise, the CVP increased from a median of 11 to 19 mmHg, and the cardiac index increased from a median of 2.1 to 4.4 l/min/m2. In 38% of the patients, CVP measured at the resting condition and during exercise differed. Laboratory results indicated no significant difference between the patients in the high-CVP group and low-CVP group at rest. In contrast, during exercise, brain natriuretic peptide (67 ± 38 vs. 147 ± 122 pg/ml, p
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- 2019
61. Clinical impact of cardiac computed tomography derived three-dimensional strain for adult congenital heart disease: a pilot study
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Tatsunori Takahashi, Yumi Shiina, Yamato Shimomiya, Kei Inai, and Michinobu Nagao
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Adult ,Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Heart disease ,Cardiac computed tomography ,Volume of interest ,medicine.medical_treatment ,Ventricular Dysfunction, Right ,Strain (injury) ,Pilot Projects ,030204 cardiovascular system & hematology ,Cardiac pacemaker ,Ventricular Function, Left ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,Ventricular Dysfunction, Left ,0302 clinical medicine ,Imaging, Three-Dimensional ,Predictive Value of Tests ,Internal medicine ,Multidetector Computed Tomography ,Medicine ,Humans ,Radiology, Nuclear Medicine and imaging ,cardiovascular diseases ,Prospective Studies ,Cardiac imaging ,Tetralogy of Fallot ,Observer Variation ,business.industry ,Cardiac Pacing, Artificial ,Reproducibility of Results ,Middle Aged ,medicine.disease ,medicine.anatomical_structure ,Ventricle ,cardiovascular system ,Cardiology ,Ventricular Function, Right ,Female ,Cardiology and Cardiovascular Medicine ,business - Abstract
To assess ventricular function and dyssynchrony using three-dimensional (3D) computed tomography (CT) strain in adult congenital heart disease (ACHD). We prospectively analyzed a multiphase cardiac CT data set for 22 adult patients with CHD, including 8 patients with congenital systemic right ventricle (RV) and 14 patients with repaired Tetralogy of Fallot (TOF). Eight patients had a cardiac pacemaker. Volume of Interest was drawn on a multiplanar reconstruction of the ventricle with strain overlay using a 3D-strain algorithm. Ventricular strain, inter- and intraventricular dyssynchrony, and right ventricle outflow tract (RVOT)–apex dyssynchrony were calculated. RVOT–apex dyssynchrony by ventriculography was also compared in 15 patients. Pulmonary ventricular strain, systemic ventricular strain, and septal wall strain were lower in ACHD patients than in the controls, and lower in the ACHD with pacing group than without pacing group as well. Maximum interventricular time difference and intraventricular time difference were longer than in ACHD than in the controls, and longer in the ACHD with pacing group than without pacing group as well. RVOT–apex delay was significantly longer in patients with a pacemaker than in those without a pacemaker (118.1 ± 31.9 ms vs. 76.1 ± 36.2 ms, p = 0.03). RVOT delay determined by 3D CT strain significantly correlated with that determined by ventriculography (Pearson r = 0.55, p = 0.03). 3D CT strain can detect reduced biventricular contraction and inter- and intraventricular and RVOT–apex mechanical dyssynchrony can be assessed in patients with ACHD.
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- 2019
62. Role of BRCA1-associated protein (BRAP) variant in childhood pulmonary arterial hypertension
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Yoshiyuki Furutani, Masaki Nii, Hiroki Sato, Kei Inai, Toru Furukawa, Shigeaki Nonoyama, Tomotaka Nakayama, Ayako Chida-Nagai, Amer A. Rana, Masaki Shintani, Hiroyuki Akagawa, Toshio Nakanishi, Rana, Moo [0000-0002-2330-4643], and Apollo - University of Cambridge Repository
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0301 basic medicine ,Proband ,Male ,Pulmonology ,Apoptosis ,Pathogenesis ,medicine.disease_cause ,Pathology and Laboratory Medicine ,Biochemistry ,Muscle, Smooth, Vascular ,0302 clinical medicine ,Medicine and Health Sciences ,Medicine ,Exome ,Familial Primary Pulmonary Hypertension ,Small interfering RNAs ,Post-Translational Modification ,Phosphorylation ,Pulmonary Arteries ,Child ,Exome sequencing ,Cells, Cultured ,Mutation ,Multidisciplinary ,Pulmonary Hypertension ,Cell Death ,Arteries ,Pedigree ,Nucleic acids ,Cell Processes ,030220 oncology & carcinogenesis ,Child, Preschool ,Female ,Anatomy ,Signal Transduction ,Research Article ,Adult ,Adolescent ,Ubiquitin-Protein Ligases ,Science ,Pulmonary Artery ,Transfection ,Research and Analysis Methods ,03 medical and health sciences ,Young Adult ,Exome Sequencing ,Genetics ,Humans ,Molecular Biology Techniques ,Non-coding RNA ,Molecular Biology ,Cell Proliferation ,business.industry ,Infant ,Biology and Life Sciences ,Proteins ,ACVRL1 ,Cell Biology ,medicine.disease ,Pulmonary hypertension ,BMPR2 ,BMPR1B ,Gene regulation ,030104 developmental biology ,Cancer research ,Cardiovascular Anatomy ,RNA ,Blood Vessels ,Gene expression ,business - Abstract
Although mutations in several genes have been reported in pulmonary arterial hypertension (PAH), most of PAH cases do not carry these mutations. This study aimed to identify a novel cause of PAH. To determine the disease-causing variants, direct sequencing and multiplex ligation-dependent probe amplification were performed to analyze 18 families with multiple affected family members with PAH. In one of the 18 families with PAH, no disease-causing variants were found in any of BMPR2, ACVRL1, ENG, SMAD1/4/8, BMPR1B, NOTCH3, CAV1, or KCNK3. In this family, a female proband and her paternal aunt developed PAH in their childhood. Whole-exome next-generation sequencing was performed in the 2 PAH patients and the proband’s healthy mother, and a BRCA1-associated protein (BRAP) gene variant, p.Arg554Leu, was identified in the 2 family members with PAH, but not in the proband’s mother without PAH. Functional analyses were performed using human pulmonary arterial smooth muscle cells (hPASMCs). Knockdown of BRAP via small interfering RNA in hPASMCs induced p53 signaling pathway activation and decreased cell proliferation. Overexpression of either wild-type BRAP or p.Arg554Leu-BRAP cDNA constructs caused cell death confounding these studies, however we observed higher levels of p53 signaling inactivation and hPASMC proliferation in cells expressing p.Arg554Leu-BRAP compared to wild-type BRAP. In addition, p.Arg554Leu-BRAP induced decreased apoptosis of hPASMCs compared with wild-type BRAP. In conclusion, we have identified a novel variant of BRAP in a Japanese family with PAH and our results suggest it could have a gain-of-function. This study sheds light on new mechanism of PAH pathogenesis.
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- 2019
63. 13. Secondary sarcopenia assessed by computed tomography can predict hospitalization for failing Fontan circulation
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Koichiro Niwa, Kei Inai, Yumi Shiina, and Michinobu Nagao
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medicine.medical_specialty ,medicine.diagnostic_test ,business.industry ,RC666-701 ,Internal medicine ,Sarcopenia ,Cardiology ,Diseases of the circulatory (Cardiovascular) system ,Medicine ,Computed tomography ,business ,medicine.disease ,Fontan circulation - Published
- 2021
64. Potential of Liver T1 Mapping for the Detection of Fontan-associated Liver Disease in Adults.
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Yumi Shiina, Kei Inai, Ryoko Ohashi, and Michinobu Nagao
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LIVER disease diagnosis ,CARDIAC magnetic resonance imaging ,CENTRAL venous pressure ,LIVER function tests ,CARDIAC catheterization - Abstract
Purpose: The native T
1 value at 3T MRI is a sensitive marker for diffuse fibrosis or damage in various organs including the heart, liver, and pancreas. Despite the fact that Fontan-associated liver disease (FALD) is a crucial issue in adults with Fontan circulation, there are only a few studies with liver T1 mapping in children and adolescents. We investigated the potential of the liver native T1 mapping in detecting FALD in adult patients. Methods: We prospectively enrolled 16 consecutive adults with Fontan circulation (age 31.3 ± 8.5 years), who were in New York Heart Association Functional class II-IV. Twenty with tetralogy of Fallot (TOF), and 20 age-matched controls also underwent cardiac magnetic resonance (CMR) imaging at 3T. Myocardial T1 mapping with a Modified Look-Locker Inversion recovery sequence was applied to liver T1 mapping. Patients in the Fontan group underwent the right heart catheter and liver function tests, including those for fibrotic markers. Results: Liver native T1 values in the Fontan group were significantly higher than that in TOF and controls (P < 0.001). In the Fontan group, the liver native T1 value was significantly correlated with age, g -glutamyltransferase, model for end-stage liver disease XI score, and albumin-bilirubin score (P = 0.01, 0.01, 0.044, 0.001). However, it demonstrated no correlation with central venous pressure, pulmonary vessel resistance, or fibrotic markers. Conclusion: Liver native T1 value derived from CMR may be a non-invasive adjunctive and/or screening marker to detect FALD. [ABSTRACT FROM AUTHOR]- Published
- 2021
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65. Dual VENC 4D flow magnetic resonance imaging demonstrates arterial-pulmonary collaterals in an adult with tetralogy of Fallot
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Masateru Kawakubo, Seiji Asagai, Kei Inai, Michinobu Nagao, and Yumi Shiina
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Adult ,medicine.medical_specialty ,medicine.diagnostic_test ,business.industry ,Magnetic resonance imaging ,General Medicine ,medicine.disease ,Magnetic Resonance Imaging ,Text mining ,Internal medicine ,Tetralogy of Fallot ,medicine ,Cardiology ,Humans ,Radiology, Nuclear Medicine and imaging ,Cardiology and Cardiovascular Medicine ,business ,Magnetic Resonance Angiography - Published
- 2020
66. Vitamin D Kinetics and Parathyroid Gland Function in Patients with Congenital Heart Disease
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Gaku Izumi, Eriko Shimada, Toshio Nakanishi, and Kei Inai
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medicine.medical_specialty ,Heart disease ,business.industry ,Renal function ,General Medicine ,030204 cardiovascular system & hematology ,medicine.disease ,Brain natriuretic peptide ,vitamin D deficiency ,03 medical and health sciences ,0302 clinical medicine ,medicine.anatomical_structure ,Internal medicine ,Heart failure ,Pediatrics, Perinatology and Child Health ,medicine ,Vitamin D and neurology ,Cardiology ,Radiology, Nuclear Medicine and imaging ,Surgery ,Secondary hyperparathyroidism ,Parathyroid gland ,030212 general & internal medicine ,Cardiology and Cardiovascular Medicine ,business - Abstract
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 vitamin D and parathormone levels in patients with congenital heart diseases and chronic heart failure. Design We measured 25-hydroxyvitamin D and parathormone serum levels in 103 adult patients with congenital heart diseases (age range 20–89 years). Of 103 patients, 54 were in New York Heart Association functional classes II or III. Their clinical data regarding cardiothoracic ratio, fractional shortening of the systemic ventricle, brain natriuretic peptide plasma levels, and pulse oximetry were also evaluated. Results Of 54 patients with chronic heart failure, 50 (93%) exhibited vitamin D deficiency (25-hydroxyvitamin D serum levels 65 pg/mL). These two parameters were inversely correlated. In multivariate analyses including age, gender, 25-hydroxyvitamin D, parathormone, pulse oximetry, cardiothoracic ratio, calcium, phosphorus, glomerular filtration rate, albumin, creatine kinase, end-diastolic diameter and fractional shortening of the systemic ventricle, and ratio of early diastolic transmitral flow velocity to mitral annular velosity, only the parathormone serum levels (P
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- 2016
67. Long-term Outcomes after Truncus Arteriosus Repair: A Single-center Experience for More than 40 Years
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Mitsugi Nagashima, Tokuko Shinohara, Hisashi Sugiyama, Seiji Asagai, In-Sam Park, Kei Inai, Tetsuko Ishii, Hirofumi Tomimatsu, and Toshio Nakanishi
- Subjects
medicine.medical_specialty ,medicine.medical_treatment ,Persistent truncus arteriosus ,Ventricular Outflow Obstruction ,030204 cardiovascular system & hematology ,Single Center ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,medicine.artery ,medicine ,Radiology, Nuclear Medicine and imaging ,Cardiac catheterization ,business.industry ,General Medicine ,Rastelli procedure ,medicine.disease ,Surgery ,Catheter ,Stenosis ,030228 respiratory system ,Pediatrics, Perinatology and Child Health ,Pulmonary artery ,cardiovascular system ,Cardiology ,Cardiology and Cardiovascular Medicine ,business - Abstract
Objectives This study aimed to analyze long-term survival and functional outcomes after truncus arteriosus repair in a single institution with more than 40 years of follow-up. Methods Medical records were analyzed retrospectively in 52 patients who underwent the Rastelli procedure for truncus arteriosus repair between 1974 and 2002. Thirty-five patients survived the initial repair. The median age at the initial operation was 2.8 months (range, 0.1–123 months) and the body weight was 3.9 kg (range, 1.6 to 15.0 kg). Results The median age at follow-up was 23.6 years (range, 12.4 to 44.5 years). The median follow-up duration was 23.4 years (range, 12.3 to 40.7 years). The actuarial survival rate was 97% at 10 years and 93% at both 20 years and 40 years after the initial operation. At follow-up, most patients were in New York Heart Association (NYHA) functional classes I (73%) and II (24%). Thirty-six percent of patients had full-time jobs, 40% were students, and 21% were unemployed. Most patients (97%) had undergone conduit reoperations. Freedom from reoperation for right ventricular (RV) outflow and pulmonary artery (PA) stenosis was 59% at 5 years, 28% at 10 years, and 3% at 20 years after the initial operation. Freedom from catheter interventions for RV outflow and PA stenosis was 59% at 5 years, 47% at 10 years, and 38% at 20 years after the initial operation. Freedom from truncal valve replacement was 88% at 5 years, 85% at 10 years, and 70% at 20 years after the initial operation. Conclusions In this single-center retrospective study, with long-term follow-up after repair of truncus arteriosus, long-term survival and functional outcomes were acceptable, despite the requirement for reoperation and multiple catheter interventions for RV outflow and PA stenosis in almost all patients, and the frequent requirement for late truncal valve operations.
- Published
- 2016
68. Compound Mutations Cause Increased Cardiac Events in Children with Long QT Syndrome: Can the Sequence Homology-Based Tools be Applied for Prediction of Phenotypic Severity?
- Author
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Kei Inai, Emiko Hayama, Toshio Nakanishi, Michiko Furutani, Yoshiyuki Furutani, Rumiko Matsuoka, Tsutomu Nishizawa, Gaku Izumi, Mitsuyo Shimada, and Hirokuni Yamazawa
- Subjects
0301 basic medicine ,Adolescent ,Long QT syndrome ,Sequence Homology ,030204 cardiovascular system & hematology ,medicine.disease_cause ,Sudden death ,Young Adult ,03 medical and health sciences ,0302 clinical medicine ,Humans ,Medicine ,Young adult ,Child ,Gene ,Genetics ,Mutation ,business.industry ,Incidence (epidemiology) ,Infant, Newborn ,Infant ,Arrhythmias, Cardiac ,medicine.disease ,Phenotype ,Long QT Syndrome ,030104 developmental biology ,Child, Preschool ,KCNQ1 Potassium Channel ,Pediatrics, Perinatology and Child Health ,Ventricular fibrillation ,Cardiology and Cardiovascular Medicine ,business - Abstract
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. Although predicting phenotypic severity is clinically important, the availability of prediction tools for LQTS is unknown. To determine whether the severity of the LQTS phenotype can be predicted by the presence of compound mutations in children is needed. We detected 97 single mutations (Group S) and 13 compound mutations (Group C) between 1998 and 2012, age at diagnosis ranging 0-19 years old (median age is 9.0) and 18.0 years of follow-up period. The phenotypes and Kaplan-Meier event-free rates of the two groups were compared for cardiac events. This study investigated phenotypic severity in relation to the location of mutations in the protein sequence, which was analyzed using two sequence homology-based tools. In results, compound mutations in children were associated with a high incidence of syncope within the first decade (Group S: 32 % vs. Group C: 61 %), requiring an ICD in the second decade (Group S: 3 % vs. Group C: 56 %). Mortality in these patients was high within 5 years of birth (23 %). Phenotypic prediction tools correctly predicted the phenotypic severity in both Groups S and C, especially by using their coupling method. The coupling prediction method is useful in the initial evaluation of phenotypes both with single and compound mutations of LQTS patients. However, it should be noted that the compound mutation makes more severe phenotype.
- Published
- 2016
69. A Pediatric Case of Arrhythmogenic Right Ventricular Cardiomyopathy
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Yoshiyuki Furutani, Hiroki Mori, Toshio Nishikawa, Toshio Nakanishi, Kenta Uto, Saeko Yoshizawa, Keiko Toyohara, Kei Inai, Kentaro Ikeda, Mitsuyo Shimada, and Mikiko Shimizu
- Subjects
0301 basic medicine ,03 medical and health sciences ,medicine.medical_specialty ,030104 developmental biology ,business.industry ,Internal medicine ,medicine ,Cardiology ,business ,Right ventricular cardiomyopathy - Published
- 2016
70. Response to Letter to ‘Pregnancy and delivery outcomes from patients with repaired anomalous origin of the left coronary artery from the pulmonary artery’
- Author
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Miki Kanoh and Kei Inai
- Subjects
medicine.medical_specialty ,Pregnancy ,030219 obstetrics & reproductive medicine ,business.industry ,Obstetrics and Gynecology ,medicine.disease ,03 medical and health sciences ,0302 clinical medicine ,Left coronary artery ,Text mining ,030220 oncology & carcinogenesis ,Internal medicine ,medicine.artery ,Pulmonary artery ,medicine ,Cardiology ,business - Published
- 2018
71. Mode of death and predictors of mortality in adult Fontan survivors: A Japanese multicenter observational study
- Author
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Masaru Miura, Mamie Watanabe, Koichiro Niwa, Kenji Kuraishi, In-Sam Park, Hiroyuki Yamagishi, Hisanori Sakazaki, Makoto Nakamura, Ono Hiroshi, Michikazu Nakai, Kenichiro Yamamura, Kunihiro Nishimura, Kei Inai, Hideo Ohuchi, Ki-Sung Kim, and Kenji Waki
- Subjects
Adult ,Male ,Pediatrics ,medicine.medical_specialty ,Adolescent ,Heart Diseases ,Disease ,030204 cardiovascular system & hematology ,Fontan Procedure ,Sudden death ,03 medical and health sciences ,Young Adult ,0302 clinical medicine ,Japan ,Predictive Value of Tests ,Cause of Death ,Neoplasms ,Medicine ,Humans ,030212 general & internal medicine ,Prospective Studies ,Survival rate ,business.industry ,Mortality rate ,Protein losing enteropathy ,Perioperative ,Middle Aged ,medicine.disease ,Survival Rate ,Heart failure ,Female ,Cardiology and Cardiovascular Medicine ,business ,Hyponatremia ,Follow-Up Studies - Abstract
Mortality rates may be high in adult Fontan patients; however, the clinical determinants remain unclear.We conducted a prospective multicenter study of adult Fontan survivors to determine the 5-year mortality rate and clarify the determinants.We followed 600 adult Fontan survivors from 40 Japanese institutions (307 men, 28 ± 7 years old, follow-up: 18 ± 6 years). The New York Heart Association (NYHA) functional class I and II was 51% and 42%, respectively. During the follow-up period of 4.1 ± 1.6 years, 33 patients died, and the 5-year survival rate was 93.5%. The mode of death was heart failure in 11 patients (34%), arrhythmia or sudden death in 8 (24%), cancer in 5 (15%), perioperative problems and hemostatic problems in 4 each (12% for each), and infection in 1 (3%). Left isomerism, prior hospitalization, protein losing enteropathy (PLE), pulmonary arteriovenous fistulae, NYHA functional class, impaired hemodynamics, hyponatremia, hepatorenal dysfunction, and use of diuretics were associated with a high mortality rate (p 0.05-0.0001). Further, PLE (hazard ratio [HR]: 14.4), left isomerism (HR: 3.5), and NYHA (HR: 2.4) independently predicted a high 5-year high mortality (p 0.05 for all). The incidence of cancer-related mortality increased markedly with age40 years.Majority of the Japanese adult Fontan survivors had good functional status, with an acceptable 5-year survival rate. However, the significant prevalence of non-cardiac mortality highlights Fontan pathophysiology as a multi-organ disease that requires a multidisciplinary management strategy to improve the long-term outcome.
- Published
- 2018
72. Feature-Tracking MRI Fractal Analysis of Right Ventricular Remodeling in Adults with Congenitally Corrected Transposition of the Great Arteries
- Author
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Masami Yoneyama, Umiko Ishizaki, Shuji Sakai, Masateru Kawakubo, Yuzo Yamasaki, Yumi Shiina, Kei Inai, and Michinobu Nagao
- Subjects
medicine.medical_specialty ,business.industry ,030204 cardiovascular system & hematology ,medicine.disease ,Fractal analysis ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,0302 clinical medicine ,medicine.anatomical_structure ,Text mining ,Congenitally corrected transposition ,Strain pattern ,Ventricle ,Great arteries ,Internal medicine ,Cardiology ,Medicine ,Feature tracking ,Radiology, Nuclear Medicine and imaging ,sense organs ,business ,Ventricular remodeling ,Original Research - Abstract
Purpose: To assess a recently available technique for quantification of right ventricular (RV) trabeculae that is based on fractal analysis performed by using cardiac MRI feature tracking, in patients with congenitally corrected transposition of the great arteries (cc-TGA). / Materials and Methods: A total of 19 patients (eight men, 11 women; mean age, 35 years ± 10 [standard deviation]) with consecutive cc-TGA who underwent cardiac MRI were enrolled in the study. For analysis, patients were divided into two groups: six patients (four men, two women; mean age, 34 years ± 14) with an end-systolic RV volume index higher than 72 mL/m2 (indicative of adverse RV remodeling) and 13 patients (four men, nine women; mean age, 36 years ± 9) in whom this index was lower than or equal to 72 mL/ m2 (indicative of adapted RV). The following outcomes were quantified in the midsection of the RV: fractional fractal dimension (FD) and diastolic FD, circumferential strain, and radial strain. Receiver operating characteristic (ROC) analysis was performed to determine the cutoff FD values for the detection of adverse RV remodeling. Correlations among fractional FD, diastolic FD, circumferential strain, and radial strain were calculated by using Pearson correlation coefficient (r) analysis. / Results: The following ROC values were identified for fractional and diastolic FD: cutoff, 0.09 and 1.39, respectively; area under the ROC curve, 0.95 and 0.68, respectively; sensitivity, 1.00 and 0.33, respectively; and specificity, 0.92 and 1.00, respectively. Fractional FD correlated with circumferential strain and radial strain (r = −0.70 and 0.69, respectively; P, [Key Points] / ・In patients with congenitally corrected transposition of the great arteries, the fractional fractal dimension derived by using featuretracking MRI, as compared with the conventional diastolic fractal dimension, facilitates a more accurate diagnosis of the development of right ventricular trabeculae owing to adverse remodeling. / ・ Fractal analysis performed by using feature-tracking MRI is a robust analytical method with high reproducibility. / ・ As fractal analysis performed with feature-tracking MRI does not require additional manual processing, it can be easily included with strain analysis in cardiac workflow analysis in clinical practice.
- Published
- 2019
73. Prediction of Fontan-Associated Liver Disease Using a Novel Cine Magnetic Resonance Imaging 'Vortex Flow Map' in the Right Atrium
- Author
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Tatsunori Takahashi, Yumi Shiina, Kei Inai, Yamato Shimomiya, Yuka Matsuo, Umiko Ishizaki, Michinobu Nagao, Shuji Sakai, and Kenji Fukushima
- Subjects
Adult ,Male ,Adolescent ,medicine.medical_treatment ,Magnetic Resonance Imaging, Cine ,030204 cardiovascular system & hematology ,Fontan Procedure ,030218 nuclear medicine & medical imaging ,Fontan procedure ,03 medical and health sciences ,Young Adult ,0302 clinical medicine ,Cardiac magnetic resonance imaging ,Predictive Value of Tests ,medicine ,Humans ,Heart Atria ,Hyaluronic Acid ,Retrospective Studies ,medicine.diagnostic_test ,Cardiac cycle ,business.industry ,Liver Diseases ,Hazard ratio ,Magnetic resonance imaging ,Bilirubin ,General Medicine ,Sagittal plane ,medicine.anatomical_structure ,Coronal plane ,Case-Control Studies ,Hydrodynamics ,Female ,Cardiology and Cardiovascular Medicine ,business ,Nuclear medicine ,TBIL - Abstract
BACKGROUND Long-term hepatic dysfunction is an increasingly recognized complication of the Fontan operation for univentricular hearts. The purpose of this study was to determine whether Fontan-associated liver disease (FALD) could be predicted by flow dynamics in the right atrium (RA) of Fontan circulation.Methods and Results:Cardiac MRI and the serum levels of total bilirubin (TBil) and hyaluronic acid (HA) were analyzed in 36 patients who underwent an atriopulmonary connection type of Fontan operation. The mean follow-up period was 53 months. Three views (axial, coronal, and sagittal) of the cine images were scanned for the maximum cross-section of the RA obtained with1.5-Tesla scanner. We developed a "vortex flow map" to demonstrate the ratio of the circumferential voxel movement in each phase to the total movement throughout a cardiac cycle towards the center of the RA. The maximum ratio was used as the magnitude of vortex flow (MVF%) in the 3 views of the RA cine imaging. Patients with coronal MVF ≥13.6% had significantly lower free rates of TBil ≥1.8 mg/dL than those with coronal MVF
- Published
- 2018
74. Global strain and dyssynchrony of the single ventricle predict adverse cardiac events after the Fontan procedure: Analysis using feature-tracking cine magnetic resonance imaging
- Author
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Umiko Ishizaki, Kei Inai, Michinobu Nagao, Yumi Shiina, Tatsunori Takahashi, Shuji Sakai, and Hiroki Mori
- Subjects
Adult ,Male ,medicine.medical_specialty ,Adolescent ,Heart Diseases ,medicine.medical_treatment ,Magnetic Resonance Imaging, Cine ,030204 cardiovascular system & hematology ,Logistic regression ,Fontan Procedure ,Fontan procedure ,03 medical and health sciences ,Young Adult ,0302 clinical medicine ,Postoperative Complications ,Cardiac magnetic resonance imaging ,Predictive Value of Tests ,Internal medicine ,medicine ,Ventricular Dysfunction ,Humans ,cardiovascular diseases ,030212 general & internal medicine ,Proportional Hazards Models ,Retrospective Studies ,medicine.diagnostic_test ,business.industry ,Magnetic resonance imaging ,Heart ,Middle Aged ,Global strain ,medicine.anatomical_structure ,Logistic Models ,Ventricle ,cardiovascular system ,Cardiology ,Feature tracking ,Female ,Cardiology and Cardiovascular Medicine ,business ,Mace - Abstract
The aim of this study was to determine whether major adverse cardiac events (MACE) during the late phase of the Fontan procedure could be predicted by strain measurements of single ventricles using cardiac magnetic resonance imaging with feature tracking (CMR-FT).One hundred adolescent patients who underwent the Fontan procedure (mean age, 21 years) were examined retrospectively with CMR-FT to assess the systemic single-ventricle function. Vertical long-axis cine imaging was divided into six myocardial segments. Global longitudinal strain (GLS) was determined by averaging the peak strain values of each of the six segments. The dyssynchrony index was defined as the standard deviation of the time to peak strain for six segments. The primary outcome was MACE, defined as cardiac death and unscheduled hospitalization.MACE occurred in 18 patients during a mean follow-up of 62 months. According to the multivariate logistic regression analysis results for potential predictor variables, GLS and the dyssynchrony index are independent predictors of MACE. Patients with GLS ≥11.8% had significantly higher MACE-free rates than did those with GLS11.8% [log-rank value, 14.15; p = 0.0002; hazard ratio, 6.82; 95% confidence interval (CI), 2.51-18.56]. Patients with a dyssynchrony index63.5 ms had significantly higher MACE-free rates than did those with dyssynchrony index ≥63.5 ms (log-rank value, 28.17; p 0.0001; hazard ratio, 21.69; 95% CI, 6.96-67.56).GLS and the dyssynchrony index found using CMR-FT are independent predictors of MACE for adolescent patients after the Fontan procedure and provide information regarding risk stratification beyond clinical parameters and biomarkers.
- Published
- 2018
75. Stroke volume ratio derived from magnetic resonance imaging as an indicator of interventricular dyssynchrony predicts future cardiac event in patients with biventricular Fontan circulation
- Author
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Michinobu Nagao, Kei Inai, Tatsunori Takahashi, and Yumi Shiina
- Subjects
Adult ,Male ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Adolescent ,Heart Ventricles ,Magnetic Resonance Imaging, Cine ,030204 cardiovascular system & hematology ,Interventricular dyssynchrony ,Fontan Procedure ,Ventricular Function, Left ,03 medical and health sciences ,Young Adult ,0302 clinical medicine ,Cardiac magnetic resonance imaging ,Predictive Value of Tests ,Internal medicine ,medicine.artery ,medicine ,Humans ,cardiovascular diseases ,030212 general & internal medicine ,Child ,Heart Failure ,Aorta ,medicine.diagnostic_test ,business.industry ,Magnetic resonance imaging ,Stroke Volume ,Stroke volume ,Vascular surgery ,Middle Aged ,Cardiac surgery ,medicine.anatomical_structure ,Ventricle ,cardiovascular system ,Cardiology ,Ventricular Function, Right ,Female ,Cardiology and Cardiovascular Medicine ,business - Abstract
The prognostic factors in patients with biventricular heart who underwent Fontan surgery remain unclear. This study wanted to assess the hypothesis that interventricular dyssynchrony evaluated by cardiac magnetic resonance imaging (MRI) can predict future cardiac events in patients with biventricular heart who have undergone Fontan surgery. We prospectively enrolled consecutive patients with biventricular Fontan circulation from 2003 to 2016, and performed protocolized cardiac MRI. We determined the stroke volume ratio (SVr) using the following formula to assess interventricular dyssynchrony: (stroke volume (SV) of the two whole ventricles)/(SV of the right ventricle + SV of the left ventricle), by tracing cine MRI data. If interventricular dyssynchrony existed, blood flowed and returned to each ventricle through the ventricular septal defect; therefore, the SVr in this instance should be less than 1.0. We enrolled 40 patients. SVr ranged from 0.81 to 1.0 (median 0.95). Low SVr ( 0.95) was associated with worse New York Heart Association functional class, longer QRS duration, right bundle branch block, low biventricular indexed stroke volume, and low biventricular ejection fraction. During the follow-up period (median 53.5 months), 10 cardiac events occurred (six cases of acute exacerbation of heart failure, three cases of supraventricular tachycardia, and one case of exacerbation of protein-losing enteropathy). Univariate analysis showed four clinical predictors: SVr 0.95 [hazard ratio (HR) 9.3, 95% confidential interval (CI) 1.7-171.5]; biventricular ejection fraction 0.45 (HR 9.4, 95% CI 2.2-65.3); left ventricular indexed end-diastolic volume 73 mL/m
- Published
- 2018
76. Influence of Pregnancy on Cardiac Function and Hemodynamics in Women With Ebstein’s Anomaly
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Masaki Ogawa, Tokuko Shinohara, Hirofumi Tomimatsu, Eriko Shimada, Miki Kanoh, Toshio Nakanishi, Kei Inai, and Mikiko Shimizu
- Subjects
Cardiac function curve ,medicine.medical_specialty ,Pregnancy ,Vaginal delivery ,business.industry ,Clinical course ,Hemodynamics ,medicine.disease ,Heart failure ,Ebstein's anomaly ,Internal medicine ,medicine ,Cardiology ,Tricuspid Valve Regurgitation ,business - Abstract
Introduction We examined the perinatal outcomes and right ventricular function before pregnancy, during pregnancy, and after delivery in women with Ebstein's anomaly. Material and methods We retrospectively investigated the clinical course and mode of delivery and monitored hemodynamic parameters throughout pregnancy in 17 women with Ebstein's anomaly who delivered at our institution during the period of 1995-2015. Results Eight women, including nine pregnancies, underwent elective cesarean section, and nine women, including 14 pregnancies, underwent vaginal delivery. Elective cesarean section was performed in cases with significant heart failure or arrhythmias and in the presence of more than two of the following: cardiothoracic ratio ≥60%, moderate or severe tricuspid valve regurgitation, tricuspid valve regurgitation pressure gradient ≥35 mmHg during pregnancy. The cardiothoracic ratio and tricuspid valve regurgitation pressure gradient significantly increased during pregnancy compared with prepregnancy values. The New York Heart Association classification deteriorated from class I to class II or III in five cases during pregnancy. Conclusions Although pregnancy was relatively safe among women with Ebstein's anomaly, some women developed cyanosis, arrhythmia, and heart failure, leading to elective cesarean section. Monitoring clinical and hemodynamic changes throughout pregnancy is advised to minimize maternal cardiac risk and select the appropriate mode of delivery.
- Published
- 2019
77. Relationships Among Red Cell Distribution Width, Anemia, and Interleukin-6 in Adult Congenital Heart Disease
- Author
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Toshio Nakanishi, Tokuko Shinohara, Kei Inai, Daiji Takeuchi, and Kenji Miyamoto
- Subjects
Adult ,Erythrocyte Indices ,Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Heart disease ,Anemia ,Microcytic anemia ,Interquartile range ,Internal medicine ,Humans ,Medicine ,Retrospective Studies ,Univariate analysis ,Interleukin-6 ,business.industry ,Hazard ratio ,Red blood cell distribution width ,General Medicine ,medicine.disease ,Confidence interval ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies - Abstract
Red cell distribution width (RDW) is known to be associated with anemia and mortality in cardiovascular diseases, while anemia itself is related to increased mortality. RDW may also be related to cytokine activation. We investigated the potential of RDW to predict anemia-adjusted mortality in patients with adult congenital heart disease (ACHD) and we evaluated the relationships among RDW, anemia, and interleukin-6 (IL-6).This was a single-center, retrospective cohort study. Blood RDW and IL-6 levels were measured in 144 patients with ACHD (median age [interquartile range (IQR)], 28 [22-36] years), 84% in New York Heart Association class I/II. During a mean 4.8-year follow-up, 21 (15%) patients died of cardiovascular causes. Elevated RDW (15.0%) correlated significantly with mortality risk in a univariate analysis (RDW hazard ratio [HR]: 1.570; 95% confidence interval [CI]: 1.208-2.040 per 1 standard deviation increase; P=0.001). Elevated RDW levels correlated significantly with increased anemia-adjusted mortality (adjusted RDW HR: 1.912; 95% CI: 1.369-2.670; P0.001). The high RDW group had significantly elevated serum IL-6 levels (RDW15%, median [IQR], 3.7 [0.9-13.9] pg/ml vs. RDW ≤15%, 1.4 [0.8-2.5 pg/ml]; P=0.001), as did patients with anemia (anemia, 1.9 [0.9-5.2] pg/ml vs. no anemia, 1.4 [0.8-2.5 pg/ml]; P=0.021).Elevation of RDW may be related with increased IL-6 and anemia-adjusted cardiovascular mortality in patients with ACHD.
- Published
- 2015
78. Influence of pregnancy on cardiac function and hemodynamics in women with Ebstein's anomaly
- Author
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Toshio Nakanishi, Masaki Ogawa, Hirofumi Tomimatsu, Tokuko Shinohara, Kei Inai, Mikiko Shimizu, Eriko Shimada, and Miki Kanoh
- Subjects
Cardiac function curve ,medicine.medical_specialty ,Pregnancy ,business.industry ,Vaginal delivery ,Obstetrics and Gynecology ,Hemodynamics ,General Medicine ,030204 cardiovascular system & hematology ,medicine.disease ,03 medical and health sciences ,0302 clinical medicine ,Cardiothoracic ratio ,Internal medicine ,Ebstein's anomaly ,Heart failure ,medicine ,Cardiology ,030212 general & internal medicine ,Tricuspid Valve Regurgitation ,business - Abstract
Introduction We examined the perinatal outcomes and right ventricular function before pregnancy, during pregnancy, and after delivery in women with Ebstein's anomaly. Material and methods We retrospectively investigated the clinical course and mode of delivery and monitored hemodynamic parameters throughout pregnancy in 17 women with Ebstein's anomaly who delivered at our institution during the period of 1995-2015. Results Eight women, including nine pregnancies, underwent elective cesarean section, and nine women, including 14 pregnancies, underwent vaginal delivery. Elective cesarean section was performed in cases with significant heart failure or arrhythmias and in the presence of more than two of the following: cardiothoracic ratio ≥60%, moderate or severe tricuspid valve regurgitation, tricuspid valve regurgitation pressure gradient ≥35 mmHg during pregnancy. The cardiothoracic ratio and tricuspid valve regurgitation pressure gradient significantly increased during pregnancy compared with prepregnancy values. The New York Heart Association classification deteriorated from class I to class II or III in five cases during pregnancy. Conclusions Although pregnancy was relatively safe among women with Ebstein's anomaly, some women developed cyanosis, arrhythmia, and heart failure, leading to elective cesarean section. Monitoring clinical and hemodynamic changes throughout pregnancy is advised to minimize maternal cardiac risk and select the appropriate mode of delivery.
- Published
- 2017
79. Vortex Flow in the Right Atrium Surrogates Supraventricular Arrhythmia and Thrombus After Atriopulmonary Connection-Type Fontan Operation: Vortex Flow Analysis Using Conventional Cine Magnetic Resonance Imaging
- Author
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Kenji Fukushima, Kei Inai, Tatsunori Takahashi, Yamato Shimomiya, Michinobu Nagao, Umiko Ishizaki, and Yumi Shiina
- Subjects
Adult ,Male ,medicine.medical_specialty ,Cardiac Catheterization ,Adolescent ,Magnetic Resonance Imaging, Cine ,030204 cardiovascular system & hematology ,Fontan Procedure ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,Young Adult ,0302 clinical medicine ,Cardiac magnetic resonance imaging ,medicine ,Tachycardia, Supraventricular ,Humans ,Heart Atria ,Thrombus ,Retrospective Studies ,Supraventricular arrhythmia ,Cardiac cycle ,medicine.diagnostic_test ,business.industry ,Magnetic resonance imaging ,Thrombosis ,Middle Aged ,medicine.disease ,Cardiac surgery ,Coronal plane ,Pediatrics, Perinatology and Child Health ,cardiovascular system ,Exercise Test ,Female ,Supraventricular tachycardia ,Cardiology and Cardiovascular Medicine ,Nuclear medicine ,business ,Biomarkers ,Biomedical engineering - Abstract
We developed a novel imaging technique, designated as vortex flow (VF) mapping, which presents a vortex flow visually on conventional two-dimensional (2D) cine MRI. Using it, we assessed circumferential VF patterns and influences on RA thrombus and supraventricular tachycardia (SVT) in AP connection-type Fontan circulation. Retrospectively, we enrolled 27 consecutive patients (25.1 ± 9.2 years) and 7 age-matched controls who underwent cardiac MRI. Conventional cine images acquired using a 1.5-Tesla scanner were scanned for axial and coronal cross section of the RA. We developed “vortex flow mapping” to demonstrate the ratio of the circumferential voxel movement at each phase to the total movement throughout a cardiac cycle towards the RA center. The maximum ratio was used as a magnitude of vortex flow (MVF%) in RA cine imaging. We also measured percentages of strong and weak VF areas (VFA%). Furthermore, in 10 out of 27, we compared VF between previous CMR (3.8 ± 1.5 years ago) and latest CMR. Of the patients, 15 had cardiovascular complications (Group A); 12 did not (Group B). A transaxial image showed that strong VFA% in Group A was significantly smaller than that in Group B or controls. A coronal view revealed that strong VFA% was also smaller, and weak VFA% was larger in Group A than in Group B or controls (P
- Published
- 2017
80. Pregnancy and delivery outcomes from patients with repaired anomalous origin of the left coronary artery from the pulmonary artery
- Author
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Miki, Kanoh, Kei, Inai, Tokuko, Shinohara, Eriko, Shimada, Mikiko, Shimizu, Hirofumi, Tomimatsu, Masaki, Ogawa, and Toshio, Nakanishi
- Subjects
Adult ,Pregnancy ,Coronary Vessel Anomalies ,Pregnancy Complications, Cardiovascular ,Pregnancy Outcome ,Humans ,Female ,Pulmonary Artery ,Delivery, Obstetric - Abstract
We investigated the clinical courses before and during pregnancy and after delivery in patients with repaired anomalous origin of the left coronary artery from the pulmonary artery to determine the impact of the hemodynamic changes and cardiac function on the selection of the appropriate mode of delivery.Six patients who underwent coronary artery reimplantation delivered 10 infants. We scrutinized the patients' hemodynamic changes on echocardiographs and the plasma brain natriuretic peptide levels before and during pregnancy and after delivery, the perinatal outcomes and maternal and fetal events.All patients were asymptomatic and categorized as having New York Heart Association functional class I before pregnancy. In 8 of 10 pregnancies, vaginal deliveries were performed; two elective cesarean sections were performed because of symptomatic heart failure. The hemodynamic parameters were stable throughout pregnancy and postdelivery, and no maternal or fetal events occurred in the patients who underwent vaginal deliveries. One cesarean section patient developed significant heart failure during the late second and third trimesters, which was accompanied by hemodynamic changes, including increased brain natriuretic peptide levels, left ventricular diastolic dysfunction and worsening arrhythmias, and thrombosis and post-partum hemorrhage occurred postdelivery. The baby had intrauterine growth retardation and small for gestational age. None of the babies had congenital anomalies.Pregnancy was safe in most of the asymptomatic patients long after anomalous origin of the left coronary artery from the pulmonary artery was repaired. Symptomatic heart failure might occur during pregnancy in patients with persisting myocardial damage. Pregnancy and delivery should be carefully managed.
- Published
- 2017
81. The prognostic value of high sensitivity cardiac troponin T in patients with congenital heart disease
- Author
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Toshio Nakanishi, Eriko Shimada, Kei Inai, Seiji Asagai, and Masayuki Abiko
- Subjects
Adult ,Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Cardiac troponin ,Heart disease ,medicine.medical_treatment ,Kaplan-Meier Estimate ,030204 cardiovascular system & hematology ,Patient Readmission ,Sensitivity and Specificity ,Coronary artery disease ,03 medical and health sciences ,Young Adult ,0302 clinical medicine ,Troponin complex ,Troponin T ,Predictive Value of Tests ,Internal medicine ,medicine ,Humans ,In patient ,Prospective Studies ,Cardiac catheterization ,Proportional Hazards Models ,Heart Failure ,business.industry ,Incidence ,Arrhythmias, Cardiac ,medicine.disease ,Prognosis ,Highly sensitive ,Death ,Cardiovascular Diseases ,Heart failure ,Multivariate Analysis ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business ,030217 neurology & neurosurgery ,Biomarkers - Abstract
Cardiac troponin T (cTnT) is a specific marker of myocardial injury that is elevated in patients with coronary artery disease or heart failure; it has been investigated as a prognostic marker. A highly sensitive, commercially available assay has been developed to detect cardiac troponin T (hs-cTnT). This study aimed to evaluate the clinical implications and prognostic value of hs-cTnT in patients with congenital heart disease (CHD).We evaluated 122 consecutive patients hospitalized at our institution because of heart failure or scheduled cardiac catheterization. We measured the serum concentration of hs-cTnT at the time of hospitalization, and we prospectively followed-up all patients for 3 years and monitored rates of cardiovascular events (e.g. cardiac death, readmission owing to worsening of heart failure or arrhythmia, and reintervention) as endpoints.We classified the patients according to their hs-cTnT level into non-detectable (ND group, hs-cTnT0.003ng/mL), detectable normal (DN group, 0.003ng/mL ≤hs-cTnT0.014ng/mL), or elevated (EL group, 0.014ng/mL ≤hs-cTnT) group; 20 of 122 (16.4%) patients were in the EL group, in which 17 cardiovascular events occurred during follow-up. In the multivariate Cox proportional hazard analyses, the EL group [p=0.024, hazard ratio (HR) 2.7, 95% confidence interval (CI) 1.1-5.8] was an independent significant predictor of cardiovascular events. A Kaplan-Meier curve revealed a high incidence of cardiovascular events in the EL group (EL vs ND log rank p0.0001, HR 7.6, 95% CI 3.2-20.0, EL vs DN log rank p0.0001, HR 4.1, 95% CI 2.1-7.8).Because the EL group is more likely to have an adverse outcome, elevated hs-cTnT level can be a prognostic marker in patients with CHD.
- Published
- 2017
82. Postoperative Residua and Sequelae
- Author
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Kei Inai
- Subjects
medicine.medical_specialty ,Cirrhosis ,Heart disease ,business.industry ,Disease ,medicine.disease ,Surgery ,Catheter ,Quality of life ,Great arteries ,cardiovascular system ,Medicine ,Enteropathy ,cardiovascular diseases ,business ,Tetralogy of Fallot - Abstract
Current advances in surgical treatment and catheter intervention enabled a large number of patients survive well into adulthood. In proportion as the adult survivors increase, increasing number of late complications occurred. In particular, postoperative residua and sequelae severely affect patients’ health-related quality of life especially in adults. In this chapter, the author focused on postoperative complications in adult patients with congenital heart disease and took up the problems after surgery for tetralogy of Fallot, D-transposition of the great arteries, congenitally corrected transposition of great arteries, and Fontan operation, because these diseases are popular in the clinical setting. Especially, right ventricular failure of tetralogy of Fallot and systemic right ventricular failure of transposition of the great arteries are important concerns. Moreover, post-Fontan complications are known as “Fontan disease” and various organ failures, i.e., liver cirrhosis and protein loosing enteropathy, as well as cardiac dysfunction. Nowadays, ACHD specialists are constantly facing the difficulties how to treat these complications. The author also mentioned necessary interventions regarding both catheter use and surgery in these patients.
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- 2017
83. Mutations of <scp> NOTCH3 </scp> in childhood pulmonary arterial hypertension
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Tomotaka Nakayama, Kei Inai, Ayako Chida, Shinichi Ohtsuki, Yoichi Kawamura, Tsutomu Saji, Masaki Shintani, Hiroki Sato, Shigeaki Nonoyama, Yoshiyuki Furutani, Toshio Nakanishi, Yoshihisa Matsushita, Takahiro Eitoku, and Emiko Hayama
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Pathology ,medicine.medical_specialty ,Cell growth ,business.industry ,Endoplasmic reticulum ,Mutant ,Original Articles ,Endoglin ,Gene mutation ,BMPR2 ,Pathogenesis ,NOTCH3 ,pulmonary arterial hypertension ,Genetics ,Cancer research ,Medicine ,Missense mutation ,gene mutation ,ER stress ,business ,Molecular Biology ,Genetics (clinical) - Abstract
Mutations of BMPR2 and other TGF-β superfamily genes have been reported in pulmonary arterial hypertension (PAH). However, 60–90% of idiopathic PAH cases have no mutations in these genes. Recently, the expression of NOTCH3 was shown to be increased in the pulmonary artery smooth muscle cells of PAH patients. We sought to investigate NOTCH3 and its target genes in PAH patients and clarify the role of NOTCH3 signaling. We screened for mutations in NOTCH3, HES1, and HES5 in 41 PAH patients who had no mutations in BMPR2, ALK1, endoglin, SMAD1/4/8, BMPR1B, or Caveolin-1. Two novel missense mutations (c.2519 G>A p.G840E, c.2698 A>C p.T900P) in NOTCH3 were identified in two PAH patients. We performed functional analysis using stable cell lines expressing either wild-type or mutant NOTCH3. The protein-folding chaperone GRP78/BiP was colocalized with wild-type NOTCH3 in the endoplasmic reticulum, whereas the majority of GRP78/BiP was translocated into the nuclei of cells expressing mutant NOTCH3. Cell proliferation and viability were higher for cells expressing mutant NOTCH3 than for those expressing wild-type NOTCH3. We identified novel NOTCH3 mutations in PAH patients and revealed that these mutations were involved in cell proliferation and viability. NOTCH3 mutants induced an impairment in NOTCH3-HES5 signaling. The results may contribute to the elucidation of PAH pathogenesis.
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- 2014
84. Survey of the current status and management of Eisenmenger syndrome: A Japanese nationwide survey
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Kei Inai, Ryo Inuzuka, Taku Inohara, Koichiro Niwa, Atsushi Yao, Hideo Ohuchi, and Hisanori Sakazaki
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Adult ,Endothelin Receptor Antagonists ,Male ,medicine.medical_specialty ,Heart disease ,Angiotensin-Converting Enzyme Inhibitors ,Heart failure ,Angiotensin II Receptor Blockers ,Nationwide survey ,Pulmonary hypertension ,Pulmonary vasodilator ,Angiotensin Receptor Antagonists ,Young Adult ,Japan ,Surveys and Questionnaires ,Internal medicine ,Prevalence ,medicine ,Humans ,Molecular Targeted Therapy ,Aged ,Congenital heart disease ,business.industry ,Endothelin receptor antagonist ,Incidence ,Incidence (epidemiology) ,Anticoagulants ,Digitalis Glycosides ,Eisenmenger syndrome ,Eisenmenger Complex ,Middle Aged ,medicine.disease ,Practice Guidelines as Topic ,Cardiology ,Drug Therapy, Combination ,Female ,Cardiology and Cardiovascular Medicine ,business - Abstract
Background The management of Eisenmenger syndrome (ES) has dramatically changed since the advent of disease-targeted therapy (DTT). However, guidelines for ES management, including DTT, have not been established. We aimed to clarify the current incidence, underlying disease, and management of ES in Japan, using a nationwide survey. Methods A written questionnaire was sent to members of the Japanese Society for Adult Congenital Heart Disease, through which information was obtained from 86 institutions. Results A total of 251 patients with ES (80.5% cases ≥ 20 years of age) were followed as of February 2012; DTT was performed in 124 (49.4%) patients. Unrepaired simple anatomy was reported as an underlying condition in 165 patients (65.7%). Among patients with ES, 55 (21.9%), 128 (51%), 53 (21.1%), and 12 (4.8%) were classified into functional classes I, II, III, and IV, respectively. DTT was routinely performed at 52 (60.5%) institutions, but there were variations in the DTT therapeutic strategy at these institutions. Combined therapy was more often used than monotherapy; an endothelin receptor antagonist was the most frequently prescribed medication. There were institutional differences regarding heart failure treatment and indications for anticoagulation. Digitalis and angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers were widely used, but beta-blockers were infrequently used to manage heart failure. Conclusions This survey describes the current status, including prevalence and underlying disease, and variations in the practical management of ES in Japan. The results will help in the creation of future guidelines for ES management.
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- 2014
85. Genetic Abnormality and Congenital Heart Disease
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Kei Inai
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medicine.medical_specialty ,Heart disease ,business.industry ,Internal medicine ,medicine ,Cardiology ,GENETIC ABNORMALITY ,medicine.disease ,business - Published
- 2014
86. Outcomes from anomalous origin of the left coronary artery from the pulmonary artery repair: Long-term complications in relation to residual myocardial abnormalities
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Hirofumi Tomimatsu, Kei Inai, Tokuko Shinohara, Toshio Nakanishi, and Miki Kanoh
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Long term complications ,Adult ,Male ,medicine.medical_specialty ,Adolescent ,030204 cardiovascular system & hematology ,Ventricular Function, Left ,03 medical and health sciences ,Young Adult ,0302 clinical medicine ,Left coronary artery ,Internal medicine ,medicine.artery ,Bland White Garland Syndrome ,medicine ,Humans ,030212 general & internal medicine ,Postoperative Period ,Child ,Retrospective Studies ,Ejection fraction ,business.industry ,Infant ,Mitral Valve Insufficiency ,medicine.disease ,Surgery ,medicine.anatomical_structure ,Treatment Outcome ,Ventricle ,Heart failure ,Child, Preschool ,Pulmonary artery ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business ,Perfusion ,Vascular Surgical Procedures ,Artery - Abstract
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary artery irregularity. This study aimed to clarify the long-term postoperative outcomes in ALCAPA patients, and to compare the infantile and adult types.We retrospectively analyzed the clinical data from 33 patients with ALCAPA who underwent surgical repairs after 1980. The patients were grouped based on whether presentation occurred before (infantile type: n=14) or after (adult type: n=19) 1 year of age.The mean follow-up duration was 16 years. Preoperatively, the infantile type had greater impairment of the left ventricle ejection fraction (LVEF) (45±15%) compared with the adult type (59±10%) (p0.01). Coronary revascularization significantly improved the postoperative LVEF (67±5%) (p0.01) in the patients with the infantile type. The postoperative LVEF did not change in the adult type. The mitral regurgitation (MR) severity improved postoperatively, but the between-group difference was not significant. Postoperatively, none of the patients with the infantile type and 37% of the patients with the adult type had left ventricular asynergy (p=0.01), and both groups showed postoperative perfusion defects (79% vs 95%, p=0.29). Compared with the infantile type, the adult type had a significant prognostic value for composite cardiovascular events that comprised cardiac death, arrhythmias, MR deterioration, and hospitalization as a consequence of heart failure (p=0.04).Most patients showed favorable clinical outcomes postoperatively, but myocardial damage remained long after surgery and cardiovascular events occurred postoperatively. Hence, meticulous long-term follow-up is warranted.
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- 2016
87. Clinical Characteristics of Brain Abscess in Cyanotic Congenital Heart Disease in Japan
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Hiroshi Kanamaru, Hiroyuki Matsuura, Masao Nakagawa, Kenji Suda, Tateki Hamaoka, Yoshio Arakaki, Tomoaki Murakami, Kenji Yasuda, Nobuo Momoi, Yasunobu Hayabuchi, Kunitaka Joo, Naoki Fusazaki, Masato Yokozawa, Jun Maeda, Yoshiyuki Furutani, Fukiko Ichida, Koutaro Koyama, Rumiko Matsuoka, Nobuki Tauchi, Shinji Oana, Mitsuhiro Kamisago, Yasuo Ono, Kei Inai, Hirotaka Ohki, Katsuhiko Mori, Kenji Kuroe, Hitoshi Horigome, Hiroki Kajino, Hiroyuki Yamagishi, Masatoshi Iijima, Satoshi Yasukouchi, Eiki Nishihara, Hiromichi Nakajima, Isao Shiraishi, Satoshi Ikehara, Yuuichi Nomura, Toshiki Kobayashi, Toshio Nakanishi, and Toshitetsu Yasuda
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medicine.medical_specialty ,business.industry ,Cyanotic congenital heart disease ,Internal medicine ,medicine ,Cardiology ,medicine.disease ,business ,Brain abscess ,Surgery - Published
- 2013
88. Survey of Reoperation Indications in Tetralogy of Fallot in Japan
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Aya Miyazaki, Atsushi Yao, Norifumi Nakanishi, Kozo Matsuo, Koichiro Niwa, Masaaki Kawada, Atsushi Mizuno, Kei Inai, Mamie Watanabe, Yoshiki Mori, and Hideo Ohuchi
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Adult ,Male ,medicine.medical_specialty ,Heart disease ,medicine.medical_treatment ,Sudden death ,Sudden cardiac death ,Japan ,Surveys and Questionnaires ,Internal medicine ,medicine ,Humans ,Survival rate ,Tetralogy of Fallot ,business.industry ,Incidence (epidemiology) ,General Medicine ,medicine.disease ,Implantable cardioverter-defibrillator ,Surgery ,Stenosis ,Cross-Sectional Studies ,Death, Sudden, Cardiac ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business - Abstract
BACKGROUND Although the survival rate for repaired Tetralogy of Fallot (TOF) is dramatically improving, anatomical and functional residua and sequelae followed by arrhythmias and sudden death are still challenging issues to be resolved. Reoperation can reduce the incidence of arrhythmias and sudden death, but there is no consensus on the indications of reoperation for patients with TOF, especially in Japan. METHODS AND RESULTS A cross-sectional questionnaire survey of reoperation indications in patients with TOF was performed through a Japanese multicenter study. The questionnaire, which focused on the number of repaired TOF patients aged >15 years old, reoperation indications and management, was sent to the institutions belonging to Japanese Society for Adult Congenital Heart Disease. In total, 41.5% (78/188) of the institutions replied. The total number of repaired TOF patients was 4,010, and sudden cardiac death was observed in 45.236/4,010 (5.9%) experienced reoperation. Pulmonary stenosis (32%) and pulmonary regurgitation (29%) were the most common reasons for reoperation. There were only 2 implantable cardioverter defibrillator or resynchronization therapy defibrillator implantations. The physiological/anatomical indications of reoperation differed among the hospitals. CONCLUSIONS Approximately 1.1% of patients suffered sudden death and 6% of repaired TOF patients had reoperation. The indications of reoperation, however, varied among the institutions. Therefore, detailed information for each case of sudden death or reoperation should be collected and analyzed in order to establish guidelines for reoperation.
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- 2013
89. ACVRL1 gene variant in a patient with vein of Galen aneurysmal malformation
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Ayako, Chida, Masaki, Shintani, Hajime, Wakamatsu, Yoshiyuki, Tsutsumi, Yuo, Iizuka, Nanako, Kawaguchi, Yoshiyuki, Furutani, Kei, Inai, Shigeaki, Nonoyama, and Toshio, Nakanishi
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otorhinolaryngologic diseases ,Article - Abstract
Although mutations in the RASA1 gene in vein of Galen aneurysmal malformation (VGAM) and an endoglin gene mutation in a VGAM patient with a family history of hereditary hemorrhagic telangiectasia (HHT) have been identified, most VGAM cases have no mutation in these genes. We sought to detect mutations in other genes related to HHT. We screened for mutations in RASA1 and three genes (endoglin, activin receptor-like kinase 1 (ACVRL1), encoding ALK1, and SMAD4) related to HHT in four VGAM patients. One variant (c.652 C>T p.R218W) in ACVRL1 was identified. Immunoblotting revealed that the ALK1-R218W protein could not promote SMAD1/5/8 phosphorylation by BMP9 stimulation. On the other hand, wild-type ALK1 could enhance the phosphorylation as expected. Furthermore, the transcriptional activation of ALK1-R218W was less efficient than that of wild-type ALK1. We identified 1 variant in ACVRL1 in a VGAM patient. These findings suggest that the ACVRL1 variant-R218W may be associated with the pathogenesis of VGAM.
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- 2016
90. Long-term Outcomes after Truncus Arteriosus Repair: A Single-center Experience for More than 40 Years
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Seiji, Asagai, Kei, Inai, Tokuko, Shinohara, Hirofumi, Tomimatsu, Tetsuko, Ishii, Hisashi, Sugiyama, In-Sam, Park, Mitsugi, Nagashima, and Toshio, Nakanishi
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Adult ,Heart Defects, Congenital ,Male ,Reoperation ,Cardiac Catheterization ,Truncus Arteriosus ,Time Factors ,Adolescent ,Arterial Occlusive Diseases ,Constriction, Pathologic ,Kaplan-Meier Estimate ,Pulmonary Artery ,Disease-Free Survival ,Ventricular Outflow Obstruction ,Young Adult ,Risk Factors ,Activities of Daily Living ,Humans ,Survivors ,Cardiac Surgical Procedures ,Child ,Tokyo ,Vascular Patency ,Retrospective Studies ,Infant ,Treatment Outcome ,Child, Preschool ,Female - Abstract
This study aimed to analyze long-term survival and functional outcomes after truncus arteriosus repair in a single institution with more than 40 years of follow-up.Medical records were analyzed retrospectively in 52 patients who underwent the Rastelli procedure for truncus arteriosus repair between 1974 and 2002. Thirty-five patients survived the initial repair. The median age at the initial operation was 2.8 months (range, 0.1-123 months) and the body weight was 3.9 kg (range, 1.6 to 15.0 kg).The median age at follow-up was 23.6 years (range, 12.4 to 44.5 years). The median follow-up duration was 23.4 years (range, 12.3 to 40.7 years). The actuarial survival rate was 97% at 10 years and 93% at both 20 years and 40 years after the initial operation. At follow-up, most patients were in New York Heart Association (NYHA) functional classes I (73%) and II (24%). Thirty-six percent of patients had full-time jobs, 40% were students, and 21% were unemployed. Most patients (97%) had undergone conduit reoperations. Freedom from reoperation for right ventricular (RV) outflow and pulmonary artery (PA) stenosis was 59% at 5 years, 28% at 10 years, and 3% at 20 years after the initial operation. Freedom from catheter interventions for RV outflow and PA stenosis was 59% at 5 years, 47% at 10 years, and 38% at 20 years after the initial operation. Freedom from truncal valve replacement was 88% at 5 years, 85% at 10 years, and 70% at 20 years after the initial operation.In this single-center retrospective study, with long-term follow-up after repair of truncus arteriosus, long-term survival and functional outcomes were acceptable, despite the requirement for reoperation and multiple catheter interventions for RV outflow and PA stenosis in almost all patients, and the frequent requirement for late truncal valve operations.
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- 2016
91. The Role of Cell Autonomous Signaling by BMP in Endocardial Cushion Cells in AV Valvuloseptal Morphogenesis
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Kei Inai, Jessica L. Burnside, Yuji Mishina, Bin Zhou, and Yukiko Sugi
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congenital, hereditary, and neonatal diseases and abnormalities ,Mesenchyme ,Morphogenesis ,Anatomy ,Biology ,Embryonic stem cell ,BMPR1A ,Cell biology ,medicine.anatomical_structure ,embryonic structures ,Conditional gene knockout ,cardiovascular system ,medicine ,Cellularization ,Autocrine signalling ,Endocardium - Abstract
Distal outgrowth and fusion of the mesenchymalized AV endocardial cushions are essential morphogenetic events in AV valvuloseptal morphogenesis. BMP-2 myocardial conditional knockout (cKO) mice die early by embryonic day (ED) 10.5 [1], hampering investigation of the role of BMP-2 in AV valvuloseptal morphogenesis after this stage. In our previous study, we localized BMP-2 and type I BMP receptors, BMPR1A and Alk2, in AV endocardial cushions [2, 3]. Based on their expression patterns, we hypothesize that autocrine signaling by BMP-2 within mesenchymalized AV cushions plays a critical role during AV valvuloseptal morphogenesis. To test this hypothesis, we employed recently generated endocardial/endocardial cushion-specific cre-driver line Nfact1 Cre . Unlike a previously generated Nfatc1 enCre line whose cre-mediated recombination is restricted to AV and OT endocardium, this Nfatc1 Cre line confers cre-mediated recombination within the endocardial cells as well as their mesenchymal progeny. Using the Nfactc1 Cre driver line, we disrupted BMPR1A (Alk3) and BMP-2 specifically from AV endocardium and endocardial cushions. BMPR1A endocardial cushion cKO (cKO Endo ) mouse embryos died by ED 12.5 and exhibited failure of cellularization of AV cushions (Fig. 22.1a–c) and disruption of extracellular matrix (ECM) protein deposition in the cushion mesenchyme. On the other hand, AV cushion formation occurred in the BMP-2 cKO Endo mice that survived beyond the AV cushion formation stage because BMP-2 expression remained intact in the AV myocardium during AV cushion formation. BMP-2 cKO Endo mice exhibited perimembranous ventricular septal defects (VSDs) (Fig. 22.1d, e), defective deposition of ECMs in the membranous septum, and AV mitral valve dysplasia, suggesting the cell autonomous requirement of BMP-2 in AV endocardial cushions. BMP-2 cKO Endo did not exhibit muscular VSDs. These data strongly support our hypothesis that cell autonomous signaling by BMP-2 in the endocardial lineage plays a significant role in mesenchymalized AV cushions during AV valvuloseptal morphogenesis.
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- 2016
92. Atrioventricular Valve Abnormalities: From Molecular Mechanisms Underlying Morphogenesis to Clinical Perspective
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Kei Inai
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congenital, hereditary, and neonatal diseases and abnormalities ,Atrioventricular valve ,business.industry ,embryonic structures ,cardiovascular system ,Morphogenesis ,Medicine ,Human heart ,Heart defect ,Anatomy ,Abnormality ,business - Abstract
Malformation of the atrioventricular (AV) cushion is a common congenital heart defect. Ebstein’s anomaly, characterized by a heart defect related to the AV cushion, involves not only a valve defect but also a myocardial abnormality such as Uhl’s anomaly. The morphogenetic features of the heart in the case of these diseases can be used as a reference for investigating valvuloseptal and myocardial formations in the human heart.
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- 2016
93. Missense Mutations of the BMPR1B (ALK6) Gene in Childhood Idiopathic Pulmonary Arterial Hypertension
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Emiko Hayama, Toshio Nakanishi, Yoshiyuki Furutani, Ayako Chida, Tsutomu Saji, Shigeaki Nonoyama, Masaki Shintani, Tomotaka Nakayama, and Kei Inai
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Mutation ,business.industry ,Bone Morphogenetic Protein Receptor Type-1B ,General Medicine ,Gene mutation ,medicine.disease_cause ,BMPR1A ,Bone Morphogenetic Protein Receptor Type-1A ,BMPR2 ,BMPR1B ,Cancer research ,medicine ,Bone morphogenetic protein receptor ,Cardiology and Cardiovascular Medicine ,business - Abstract
Background: Mutations in the bone morphogenetic protein receptor type 2 (BMPR2) gene, the activin receptor-like kinase 1 (ALK1) gene, and SMAD8 gene have been reported in heritable pulmonary arterial hypertension (HPAH) and in idiopathic pulmonary arterial hypertension (IPAH). However, almost 30% of HPAH cases and 60–90% of IPAH cases have no mutations in those genes. This suggests that there remain unidentified genes associated with HPAH and IPAH. Methods and Results: This study screened for mutations in endoglin, SMAD1, SMAD2, SMAD3, SMAD4, SMAD5, SMAD6, SMAD7, bone morphogenetic protein receptor type 1A (BMPR1A) and bone morphogenetic protein receptor type 1B (BMPR1B) genes in 43 IPAH patients who had no mutations in BMPR2, ALK1 and SMAD8. Two missense mutations (c.479 G>A S160N, c.1176 C>A F392L) in BMPR1B were each identified in 2 IPAH patients. Immunoblot analysis revealed that the BMPR1B F392L protein promoted SMAD8 phosphorylation. The response to BMP was analyzed using promoter-reporter activities. The transcriptional activation of the BMPR1B F392L protein with SMAD8 increased above that of wild-type BMPR1B with SMAD8, and those of BMPR1B S160N and F392L with SMAD8 and SMAD4 were each increased above those of the wild-type BMPR1B with SMAD8 and SMAD4. Conclusions: We identified 2 novel mutations in BMPR1B in 2 patients with IPAH. Our study suggests that BMPR1B mutations are associated with the pathogenesis of IPAH. (Circ J 2012; 76: 1501 – 1508)
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- 2012
94. Abstract 12727: The Clinical Impact of Eicosapentaenoic Acid/Arachidonic Acid Ratio in Adult Patients With Congenital Heart Disease
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Miki Kanoh, Kei Inai, Eriko Shimada, Tokuko Shinohara, Mikiko Shimizu, Tetsuko Ishii, Hisashi Sugiyama, and In-Sam Park
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Physiology (medical) ,Cardiology and Cardiovascular Medicine - Abstract
Background: Recent studies showed that intake of long-chain n-3 polyunsaturated fatty acids reduces the incidence of cardiovascular events. Furthermore, low levels of eicosapentaenoic acid (EPA) are associated with heart failure (HF), arrhythmia, and cardiac sudden death. However, there have been few studies regarding the clinical implication of the EPA/arachidonic acid (AA) ratio in adult patients with congenital heart disease (CHD). Objectives and methods: The present study was a prospective study of 64 adult patients with CHD. We measured serum EPA and AA and divided the patients into two groups according to their EPA/AA ratio: Results: There were no significant differences between the low (n = 36) and high (n = 28) groups in age, sex, blood pressure, systemic ventricular ejection fraction, medications, or serum levels of hemoglobin A1c, creatinine, low-density lipoprotein-cholesterol, high-density lipoprotein-cholesterol, triglyceride, or B-type natriuretic peptide. A low EPA/AA ratio had significant prognostic value for arrhythmia (hazard ratio [HR] 3.6, 95% confidence interval [CI] 1.1-14.8, p = 0.03) and composite events of arrhythmia and HF hospitalization (HR 5.6, 95% CI 1.53-27.8, p < 0.01). Subgroup analysis revealed that the patients with two-ventricle circulation in the low group (n = 23) were at significantly higher risk of arrhythmia (HR 6.7, p = 0.02), HF hospitalization (HR 16.2, p < 0.01), and composite events of arrhythmia and HF hospitalization (HR 6.7, p = 0.03) than those in the high group (n = 23). In patients with single-ventricle circulation, serum EPA levels were significantly lower compared with patients with two-ventricle circulation. Conclusions: The EPA/AA ratio had a significant predictive value for cardiac events in adult patients with CHD, especially in patients with two-ventricle circulation. The effect of EPA treatment for patients with CHD should be elucidated in the future.
- Published
- 2015
95. BMP-2 induces cell migration and periostin expression during atrioventricular valvulogenesis
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Roger R. Markwald, Yukiko Sugi, Russell A. Norris, Kei Inai, and Stanley Hoffman
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Inhibitor of Differentiation Protein 1 ,Receptor expression ,Bone Morphogenetic Protein 2 ,Smad Proteins ,Chick Embryo ,Extracellular matrix ,0302 clinical medicine ,Cell Movement ,Transforming Growth Factor beta ,Cell proliferation ,In Situ Hybridization ,0303 health sciences ,Heart ,Cell Differentiation ,Extracellular matrix protein ,Chicken ,Valvulogenesis ,medicine.anatomical_structure ,030220 oncology & carcinogenesis ,Bone Morphogenetic Proteins ,embryonic structures ,Mesenchymal cell migration ,Signal Transduction ,animal structures ,Mesenchyme ,Periostin ,Biology ,Bone morphogenetic protein (BMP) ,Bone morphogenetic protein ,Models, Biological ,Bone morphogenetic protein 2 ,Article ,Avian Proteins ,03 medical and health sciences ,medicine ,Animals ,Cell migration ,RNA, Messenger ,Noggin ,Molecular Biology ,Bone Morphogenetic Protein Receptors, Type I ,Embryonic Stem Cells ,DNA Primers ,030304 developmental biology ,Base Sequence ,Twist-Related Protein 1 ,Cardiac cushions ,Mesenchymal Stem Cells ,Cell Biology ,Molecular biology ,Viral gene transfer ,Endocardial Cushions ,Cell Adhesion Molecules ,Developmental Biology - Abstract
Atrioventricular (AV) endocardium transforms into the cushion mesenchyme, the primordia of the valves and membranous septa, through epithelial–mesenchymal transformation (EMT). While bone morphogenetic protein (BMP)-2 is known to be critical for AV EMT, the role of BMP-2 in post-EMT AV valvulogenesis remains to be elucidated. To find BMP signaling loops, we first localized Type I BMP receptors (BMPRs), BMPR-1A (ALK3), -1B (ALK6) and ALK2 in AV cushion mesenchyme in stage-24 chick embryos. Based on the BMP receptor expression pattern, we examined the functional roles of BMP-2 and BMP signaling in post-EMT valvulogenesis by using stage-24 AV cushion mesenchymal cell aggregates cultured on 3D-collagen gels. Exogenous BMP-2 or constitutively active (ca) BMPR-1B (ALK6)-virus treatments induced migration of the mesenchymal cells into the collagen gels, whereas noggin, an antagonist of BMPs, or dominant-negative (dn) BMPR-1 B (ALK6)-virus treatments reduced cell migration from the mesenchymal cell aggregates. Exogenous BMP-2 or caBMPR-1B (ALK6) treatments significantly promoted expression of an extracellular matrix (ECM) protein, periostin, a known valvulogenic matrix maturation mediator, at both mRNA and protein levels, whereas periostin expression was repressed by adding noggin or dnBMPR-1B (ALK6)-virus to the culture. Moreover, transcripts of Twist and Id1, which have been implicated in cell migration in embryogenesis and activation of the periostin promoter, were induced by BMP-2 but repressed by noggin in cushion mesenchymal cell cultures. These data provide evidence that BMP-2 and BMP signaling induce biological processes involved in early AV valvulogenesis, i.e. mesenchymal cell migration and expression of periostin, indicating critical roles for BMP signaling in post-EMT AV cushion tissue maturation and differentiation.
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- 2008
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96. Prognostic value of multiple biomarkers for cardiovascular mortality in adult congenital heart disease: comparisons of single-/two-ventricle physiology, and systemic morphologically right/left ventricles
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Tokuko Shinohara, Kenji Miyamoto, Toshio Nakanishi, Kei Inai, and Daiji Takeuchi
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Adult ,Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Time Factors ,Acute decompensated heart failure ,Heart disease ,Heart Ventricles ,Physiology ,030204 cardiovascular system & hematology ,Plasma renin activity ,Ventricular Function, Left ,03 medical and health sciences ,Young Adult ,0302 clinical medicine ,Predictive Value of Tests ,Risk Factors ,Medicine ,Humans ,030212 general & internal medicine ,Tokyo ,Proportional Hazards Models ,Retrospective Studies ,business.industry ,medicine.disease ,Brain natriuretic peptide ,Prognosis ,Angiotensin II ,Cardiac surgery ,medicine.anatomical_structure ,Ventricle ,Heart failure ,Ventricular Function, Right ,Female ,Cardiology and Cardiovascular Medicine ,business ,Biomarkers - Abstract
Although there are many biomarkers for heart failure, limited data are available regarding their prognostic value in adult congenital heart disease (ACHD). We investigated the potential of various biomarkers to predict ACHD mortality in a single-center, retrospective cohort study. Blood levels of neurohormones [angiotensin II, endothelin-1 (ET-1), norepinephrine (NE), aldosterone, and plasma renin activity]; inflammatory biomarkers [high-sensitivity C-reactive protein (hs-CRP), high-sensitivity tumor necrosis factor, soluble TNF receptor type I and II (sTNF-RI and sTNF-RII), and interleukin-6 (IL-6)]; and brain natriuretic peptide (BNP) were measured in 103 ACHD patients (median age 28 years). Subjects were divided into patients with single-ventricle physiology (SV group, n = 61) and those with two-ventricle physiology (TV group, n = 42); and into patients with a systemic right ventricle (SRV group, n = 25) and those with a systemic left ventricle (SLV group, n = 78). During a median follow-up period of 6.5 years, 12 patients (11 %) died of acute decompensated heart failure (ADHF). Predictive biomarkers, which are related to the New York Heart Association class and cardiothoracic ratio, were as follows: elevated levels of BNP, ET-1, sTNF-RI, NE, and IL-6 in the overall patient group; IL-6, NE, hs-CRP, BNP, and ET-1 in the TV group; BNP and ET-1 in the SV group; BNP, NE, hs-CRP, sTNF-RI, IL-6, and ET-1 in the SLV group. Elevated levels of ET-1 in SRV groups were slightly although not significantly associated with these. Various clinical biomarkers are associated with ADHF mortality in ACHD patients. The most prominent mortality predictors in biomarker profiles may vary according to differences in ventricular physiology and systemic ventricle morphology.
- Published
- 2015
97. Incidence, Clinical Course, and Risk Factors of Amiodarone-Induced Thyroid Dysfunction in Japanese Adults With Congenital Heart Disease
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Kei Honda, Tokuko Shinohara, Toshio Nakanishi, Keiko Toyohara, Kei Inai, and Daiji Takeuchi
- Subjects
Adult ,Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Heart disease ,Adolescent ,Amiodarone ,Asian People ,Hypothyroidism ,Japan ,Risk Factors ,Internal medicine ,medicine ,Humans ,Risk factor ,Retrospective Studies ,business.industry ,Incidence (epidemiology) ,Incidence ,Hazard ratio ,Retrospective cohort study ,General Medicine ,Middle Aged ,medicine.disease ,Thyrotoxicosis ,Cardiology ,Female ,Thyroid function ,Cardiology and Cardiovascular Medicine ,business ,Complication ,medicine.drug ,Follow-Up Studies - Abstract
Background Although amiodarone (AMD)-induced thyroid dysfunction (AITD) is an important complication of AMD therapy, little is known about AITD in adult Japanese patients with congenital heart disease (CHD). Methods and results We retrospectively studied 131 adult patients with CHD who were on low-dose AMD (median, 150 mg/day). The median patient age was 28 years, and the median follow-up was 44 months. The incidence, clinical course, and risk factors of AITD, including AMD-induced thyrotoxicosis (AIT) and AMD-induced hypothyroidism (AIH), were evaluated. The total incidence of AITD was 30% (AIT: 18%, n=24; AIH: 12%, n=16). Approximately 67% of patients with AIT displayed deterioration of tachyarrhythmia, and 38% patients underwent steroid therapy. Although thyroid function and symptoms associated with AIT improved within 6 months after diagnosis in most patients with AIT (92%), 1 patient died suddenly during an acute phase of AIT. No patient with AIH exhibited deterioration of tachyarrhythmia, and 9 patients underwent thyroid hormone replacement therapy. Cox multivariate analysis identified no independent risk factor for AIT, whereas liver dysfunction (hazard ratio 2.573; 95% confidence interval 1.102-5.795) was an independent risk factor for AIH. Conclusions AITD commonly occurred in adult Japanese patients with CHD even though they were on a low-dose AMD regimen. Risk factors for AITD may vary according to ethnicity and diet.
- Published
- 2015
98. Can pulmonary vasodilator therapy expand the operative indications for congenital heart disease?
- Author
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Kei Inai
- Subjects
Heart Defects, Congenital ,medicine.medical_specialty ,Heart disease ,business.industry ,Vascular disease ,Hypertension, Pulmonary ,Vasodilator Agents ,Hemodynamics ,General Medicine ,Eisenmenger Complex ,medicine.disease ,Pulmonary hypertension ,Shunt (medical) ,Treatment Outcome ,Eisenmenger syndrome ,Internal medicine ,medicine ,Cardiology ,Humans ,Cardiology and Cardiovascular Medicine ,business ,Pulmonary vasodilators ,Pathological - Abstract
The operability of congenital heart disease with left to right shunt depends on the severity of the pulmonary vascular disease induced by the increased pulmonary blood flow. Although some recommendations exist regarding operative indications according to pathological, hemodynamic, and epidemiological factors, the evidence underlying these recommendations is not conclusive. Recently, oral pulmonary vasodilator therapy has been reported to improve outcomes in patients with idiopathic pulmonary arterial hypertension, and this therapy also appears to be effective in patients with congenital heart disease and pulmonary arterial hypertension, including those with postoperative pulmonary hypertension and Eisenmenger syndrome. It is expected that the availability of novel pulmonary vasodilator therapy will expand the operative indications in patients with congenital heart disease with left to right shunt, but there is currently insufficient evidence to definitively determine this. A multicenter double-blind study should be conducted to further examine this issue.
- Published
- 2015
99. Abstract 12140: Prognostic Value of Multiple Biomarkers on Mortality in Adults Congenital Heart Disease: Comparison of Single- and Two-Ventricle Physiology
- Author
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Kenji Miyamoto, Daiji Takeuchi, Kei Inai, Tokuko Shinohara, and Toshio Nakanishi
- Subjects
Physiology (medical) ,Cardiology and Cardiovascular Medicine - Abstract
Introduction: Although many biomarkers are associated with heart failure, limited data is available on their prognostic predictive value in adults with congenital heart disease (ACHD). We investigate the potential of various biomarkers to predict ACHD mortality. Methods and Results: This is a single-center, retrospective cohort study. Blood levels of neurohormones (endothelin-1 [ET1], norepinephrine [NE], aldosterone, and plasma renin activity [PRA]); inflammatory biomarkers (high sensitivity C-reactive protein [hs-CRP], high sensitivity tumor necrosis factor [hs-TNF]-α, soluble TNF receptor type I and II [sTNF-RI and sTNF-RII], and interleukin-6 [IL-6]); and brain natriuretic peptide (BNP) were measured in 115 ACHD (mean age, 30 ± 10 years). NYHA class was I/ II in 86% and III/IV in 14%. The subjects were divided into two groups: patients with single-ventricle (SV group, n = 65) and with two-ventricle physiology (TV group, n = 50). We retrospectively analyzed the relationship between levels of biomarkers and cardiovascular death. During a mean follow-up period of 4.6 years, 17 (14%) cardiovascular deaths occurred, including 7 in the SV group. Univariate cox regression analysis in all subjects showed strong association between elevated levels of ET1, NE, RPA, hs-CRP, sTNF-RI and II, IL-6, and BNP and cardiovascular death (p< 0.05). In the SV group, using multivariate cox regression model, BNP and sTNF-RI were the most powerful predictors in these biomarkers (adjusted hazard ratio [aHR] of BNP: 14.84; 95%CI: 2.21-99.36 per 1 SD increase, p = 0.005) (aHR of sTNF-RI: 2.30; 95%CI: 1.91-4.55 per 1 SD increase, p = 0.017). The optimal cut-off values of BNP and sTNF-RI for mortality were 196 pg/mL and 1.26 ng/mL, respectively. Conversely, in the TV group, only IL-6 was an independent predictor of mortality (aHR: 3.24; 95%CI: 1.57-6.68 per 1 SD increase, p = 0.001), while BNP was not strongly associated with outcomes. The optimal cut-off value of IL-6 for mortality was 2.3 pg/mL. Conclusion: Various biomarkers, including sTNF-R, BNP, and IL-6, are associated with prognosis in overall ACHD. The most prominent mortality predictors might differ, due to differences in SV or TV physiology.
- Published
- 2014
100. Risk factors and serological markers of liver cirrhosis after Fontan procedure
- Author
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Gaku Izumi, Mikiko Shimizu, Toshio Nishikawa, Shuji Sakai, Kenji Miyamoto, Yunosuke Nishihara, Toshio Nakanishi, and Kei Inai
- Subjects
Adult ,Heart Defects, Congenital ,Liver Cirrhosis ,Male ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Cirrhosis ,Time Factors ,Adolescent ,medicine.medical_treatment ,Biopsy ,Autopsy ,030204 cardiovascular system & hematology ,Serum Hyaluronic Acid ,Fontan Procedure ,Fontan procedure ,03 medical and health sciences ,Young Adult ,0302 clinical medicine ,Predictive Value of Tests ,Risk Factors ,Medicine ,Humans ,cardiovascular diseases ,Hyaluronic Acid ,Child ,Retrospective Studies ,business.industry ,Cancer ,Histology ,gamma-Glutamyltransferase ,Vascular surgery ,medicine.disease ,Surgery ,Cardiac surgery ,surgical procedures, operative ,Treatment Outcome ,ROC Curve ,Area Under Curve ,Child, Preschool ,cardiovascular system ,Disease Progression ,030211 gastroenterology & hepatology ,Female ,Cardiology and Cardiovascular Medicine ,business ,Tomography, X-Ray Computed ,Biomarkers - Abstract
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 scans (CT) of the liver was carried out in 57 patients after Fontan procedure. Patients were divided into two groups: LC group (n = 31) and no LC group (n = 26). Age at Fontan procedure, duration after Fontan procedure, catheterization data, and history of failing Fontan circulation were compared between groups. Serological data including γ-GTP and hyaluronic acid were compared. Histology of autopsy specimens was assessed when available. Duration after Fontan procedure was significantly longer in LC group than no LC group. History of failing Fontan circulation was more frequent in LC group than in no LC group. There was no correlation between type of procedure (APC/Bjork/lateral tunnel/TCPC) and LC in this series. Serum hyaluronic acid, γ-GTP, and Forns index were significantly higher in LC group. Significant risk factors for LC were duration after Fontan procedure (>20 years). In autopsy specimens, histopathological changes of LC were observed predominantly in the central venous area. LC diagnosed with CT is frequent in patients long after Fontan procedure, especially after 20 years. Hyaluronic acid and γ-GTP could be useful markers to monitor the progression of liver fibrosis in Fontan patients.
- Published
- 2014
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