Publications by authors named "Takahiro Shindo"

20 Publications

  • Page 1 of 1

Poor Myocardial Compaction in a Patient with Recessive MYL2 Myopathy.

Int Heart J 2021 Mar 17;62(2):445-447. Epub 2021 Mar 17.

Department of Pediatrics, Graduate School of Medicine, The University of Tokyo.

Recessive mutations in the Myosin regulatory light chain 2 (MYL2) gene are the cause of an infantile-onset myopathy, associated with fatal myocardial disease of variable macromorphology. We here present the first Japanese family affected with recessive MYL2 myopathy. Affected siblings manifested typical features and the proband's autopsy findings were compatible with the diagnosis of noncompaction cardiomyopathy. The rapidly progressive clinical course of this recessive MYL2 cardiomyopathy highlights the crucial role of c-terminal tails in MYL2 protein in maintaining cardiac morphology and function.
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http://dx.doi.org/10.1536/ihj.20-639DOI Listing
March 2021

Relationship between post-IVIG IgG levels and clinical outcomes in Kawasaki disease patients: new insight into the mechanism of action of IVIG.

Clin Rheumatol 2020 Dec 27;39(12):3747-3755. Epub 2020 May 27.

Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-0033, Japan.

Introduction/objectives: The dosing of intravenous immunoglobulin (IVIG) therapy for Kawasaki disease (KD) has been a matter of debate for decades, with recent studies implicating that larger doses lead to better outcomes. Despite this, few have investigated post-IVIG infusion immunoglobulin G (IgG) levels in relation to outcomes of KD such as response to IVIG and development of coronary artery abnormalities (CAAs). The present study investigated how varying levels of post-infusion IgG affected these outcomes.

Method: We collected demographic and laboratory data, including post-infusion IgG, from children with KD who were admitted to six hospitals in Japan between 2006 and 2012. We conducted multivariate analyses to examine the relationship between independent variables and non-response to IVIG and development of CAAs. We used random forest, a decision tree-based machine learning tool, to investigate the marginal effect of varying post-infusion IgG levels on non-response to IVIG and development of CAAs.

Results: Of 456 patients included in the study, 130 (28.5%) were non-responders and 38 (8.3%) developed CAAs. Sodium, post-infusion IgG, and AST were significantly associated with non-response. Post-infusion IgG and sodium were significantly associated with CAA development. The random forest plots revealed a decrease in non-response and CAA rates with increasing post-infusion IgG until post-infusion IgG was near the median (2821 mg/dL), after which the non-response and CAA rates leveled off.

Conclusions: Greater post-infusion IgG is associated with better response to IVIG and decreased CAA development in KD patients, but this effect levels off at post-infusion IgG levels greater than the median. Key points • Though previous studies have shown that post-intravenous immunoglobulin (IVIG) infusion immunoglobulin G (IgG) is associated with non-response to IVIG therapy and coronary artery abnormality (CAA) development in Kawasaki disease (KD) patients, no study has investigated the relationship between varying levels of post-infusion IgG and these clinical outcomes. • Our study showed that non-response to IVIG therapy and CAA development in Kawasaki disease patients follow a decreasing trend with increasing post-infusion IgG at post-infusion IgG levels below the median. • At values of post-infusion IgG greater than the median, non-response and CAA development rates remain relatively constant with increasing post-infusion IgG. • Our study suggests that when post-infusion IgG is greater than the median, IgG may have fully bound to the therapeutic targets of KD, and in these patients, there may be limited benefit in administering additional IVIG.
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http://dx.doi.org/10.1007/s10067-020-05153-wDOI Listing
December 2020

Ecthyma gangrenosum in a 3-year-old boy post-heart transplantation.

Transpl Infect Dis 2019 Aug 30;21(4):e13131. Epub 2019 Jun 30.

Division of Infectious Diseases, Department Medical Subspecialties, National Center for Child Health and Development, Setagaya-ku, Japan.

Ecthyma gangrenosum (EG) is a serious bacterial infection in immunocompromised patients. EG in transplant recipients is rarely reported and may go unrecognized, which may delay initiation of appropriate treatment. We report a case of EG in a pediatric heart transplant recipient who was treated successfully with antibiotics and surgical debridement.
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http://dx.doi.org/10.1111/tid.13131DOI Listing
August 2019

Cardiac Rhythm Disturbances in Heterotaxy Syndrome.

Pediatr Cardiol 2019 Jun 14;40(5):909-913. Epub 2019 Mar 14.

Department of Pediatrics, University of Tokyo Hospital, Tokyo, Japan.

Objective: Heterotaxy syndrome is associated with complex cardiac malformations and cardiac conduction system abnormalities. Those with right atrial isomerism (RAI) have dual sinus nodes and dual atrioventricular nodes predisposing them to supraventricular tachycardia (SVT). Those with left atrial isomerism (LAI) lack a normal sinus node and are at risk of sinus node dysfunction (SND) and atrioventricular block (AV block). We report the occurrence and risk factors associated with arrhythmias in heterotaxy syndrome.

Methods: A retrospective review of all heterotaxy syndrome patients born and treated at our institution between 2000 and 2014 was performed.

Results: A total of 40 patients were identified; 16/40 (40%) with LAI and 24/40 (60%) with RAI. There were 12 deaths during follow-up [LAI 3/16 (19%), RAI 9/24 (38%); p = 0.30]. Twenty-one patients had arrhythmias during a mean follow-up period of 5.4 years; 14/16 (87%) in LAI and 7/24 (29%) in RAI (p < 0.001). Freedom from arrhythmia at 1,3,5 years of age was 75.0%, 37.9%, 22.7% in LAI, and 83.3%, 77.5%, 69.6% in RAI, respectively(p = 0.00261). LAI had a three-fold increase in developing arrhythmias. Left atrial isomerism was the only factor identified to be associated with arrhythmia occurrence.

Conclusions: Arrhythmias were commonly seen in heterotaxy syndrome particularly in left isomerism with more than half of the patients having arrhythmias by 3 years of age. Atrial situs was the only risk factor identified to be associated with arrhythmias, and close follow-up is warranted in these patients.
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http://dx.doi.org/10.1007/s00246-019-02087-2DOI Listing
June 2019

Identification of a Novel Titin Variant Underlying Myocardial Involvement in Neurofibromatosis Type 1.

Can J Cardiol 2018 10 31;34(10):1369.e5-1369.e7. Epub 2018 Jul 31.

Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan. Electronic address:

Because of the rare co-occurrence, it remains a question whether cardiomyopathy is a true association of neurofibromatosis type 1. A boy with café-au-lait spots manifested restrictive cardiomyopathy. Whole exome sequencing confirmed the genetic diagnosis of neurofibromatosis and further identified a novel titin (TTN) missense variant. The significance of the variant is supported by its de novo origin, in silico predictions, and evolutionary conservation. Modern genetics raises an intriguing explanation for the unexpected phenotype and adds to the evolving role of TTN variants in cardiomyopathy.
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http://dx.doi.org/10.1016/j.cjca.2018.07.473DOI Listing
October 2018

Identification of liver fibrosis using the hepatic vein waveform in patients with Fontan circulation.

Hepatol Res 2019 Mar 27;49(3):304-313. Epub 2018 Sep 27.

Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.

Aim: Liver fibrosis caused by congestive hepatopathy has emerged as an important complication after Fontan procedure. We evaluated the utility of the hepatic vein (HV) waveform using Doppler ultrasound for identification of liver fibrosis in Fontan patients.

Methods: We investigated the HV waveforms in 41 Fontan patients and assessed correlations with clinical parameters, liver fibrosis markers, and hemodynamic data.

Results: Based on our preliminary analysis of 64 adult patients with chronic liver disease who underwent liver biopsy, we classified HV waveforms into five types with reference to the degree of flattening (from type 1, normal triphasic waveform; to type 5, a monophasic waveform indicating cirrhosis), and confirmed a significant correlation between waveform pattern and fibrosis stage. Notably, we detected HV waveforms in all of the Fontan patients and classified them into five types. The HV waveform pattern positively correlated with γ-glutamyl transferase and hyaluronic acid levels, and negatively correlated with albumin level and platelet count, but did not correlate with central venous pressure or brain natriuretic peptide level, suggesting that HV waveform could reflect pathophysiological changes in the liver without being affected by hepatic congestion. The highest area under the receiver operating characteristic curve of the HV waveform for detecting advanced liver fibrosis, as defined by ultrasonic findings and clinical features, was 0.829 (81.8% sensitivity, 73.3% specificity), which was higher than that of other non-invasive fibrosis markers.

Conclusions: Hepatic vein waveforms change in accordance with liver fibrosis progression in Fontan patients, and can be a useful indicator of liver fibrosis after the Fontan procedure.
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http://dx.doi.org/10.1111/hepr.13248DOI Listing
March 2019

Accelerated Cardiomyocyte Proliferation in the Heart of a Neonate With LEOPARD Syndrome-Associated Fatal Cardiomyopathy.

Circ Heart Fail 2018 04;11(4):e004660

Department of Pediatrics (Y.N., R.I., H.T., S.K., K.S., H.A., T.S., Y.H., A.O.), Department of Pathology (K.I., M.H.), Department of Cardiovascular Medicine (N.T.), and Department of Developmental Medical Sciences (M.S.), Graduate School of Medicine, The University of Tokyo, Japan.

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http://dx.doi.org/10.1161/CIRCHEARTFAILURE.117.004660DOI Listing
April 2018

Effect of i.v. immunoglobulin in the first 4 days of illness in Kawasaki disease.

Pediatr Int 2018 Apr 26;60(4):334-341. Epub 2018 Feb 26.

Department of Pediatrics, University of Tokyo, Tokyo, Japan.

Background: Although early treatment of Kawasaki disease (KD) with i.v. immunoglobulin (IVIG) is expected to prevent coronary artery abnormalities, the effectiveness of IVIG by day 4 of illness remains to be determined.

Methods: This was a multi-institutional, retrospective cohort study. Patients diagnosed with KD at ≤4 days of illness were divided into two groups: those who received initial IVIG before and on day 5 of illness. Baseline characteristics were adjusted using propensity scores. The primary endpoint was the need for additional treatment.

Results: Of 339 patients diagnosed with KD by day 4, 181 and 158 received IVIG before and on day 5 of illness, respectively. Patients in the early treatment group had more adverse prognostic factors: infancy, early onset of the principal symptoms, and abnormal laboratory data. We thus adjusted baseline characteristics before treatment decisions using propensity scores. Propensity score matching of the two groups yielded 100 observations. More patients required additional treatment in the matched early treatment group: 37% vs 24% (adjusted OR, 1.7; 95%CI: 1.06-2.8; P = 0.047). The difference was more pronounced for risk of relapse after initial resolution of fever: 14% vs 5.0% (adjusted OR, 3.2; 95%CI: 1.3-7.7; P = 0.02). The risk of coronary artery lesion did not differ significantly.

Conclusions: IVIG treatment by day 4 of illness is associated with the requirement for additional treatment even after adjustment of baseline characteristics. Increased resistance to IVIG when given by day 4 should be considered in order to improve the treatment regimen for early-diagnosed KD.
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http://dx.doi.org/10.1111/ped.13512DOI Listing
April 2018

A Qualitative Assessment of Adolescent Girls' Perception of Living with Congenital Heart Disease: Focusing on Future Pregnancies and Childbirth.

J Pediatr Nurs 2018 Jan - Feb;38:e12-e18. Epub 2017 Nov 16.

Department of Family Nursing, Division of Health Sciences and Nursing, Graduate School of Medicine, The University of Tokyo, Japan. Electronic address:

Purpose: Congenital heart disease (CHD) is the most common birth anomaly in Japan, occurring in approximately 10.6 of every 1,000 live births. Advancements in medical and surgical care have increased births by women diagnosed with CHD. The study's purpose was to examine the perceptions of pregnancy and childbirth among adolescent girls with CHD.

Design And Methods: Twelve semi-structured interviews were conducted, and the data were analyzed using a modified grounded-theory approach.

Results: Three categories and 16 subcategories were extracted. Adolescent girls with CHD reported feelings of distress and anxiety while struggling with their disease, and feared how their disease might negatively influence their future pregnancy. These concerns were related to a desire to become familiar with CHD. The girls also explored how their disease would be managed during pregnancy and childbirth. Overall, these perceptions were influenced by the girls' acceptance of their disease, and support from family, friends, and healthcare professionals.

Conclusions: Healthcare professionals might assess adolescent girls' awareness of their disease before discussing pregnancy and childbirth risks. To encourage them to understand and cope with their disease, healthcare professionals might provide interventions tailored to the timing, stage, and degree of pregnancy and childbirth awareness. This could allow safer life planning, especially concerning pregnancy and childbirth decisions.

Practice Implications: To address adolescent girls' needs, healthcare professionals should continuously assess their awareness of pregnancy and childbirth as well as their psychological status, alongside CHD issues.
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http://dx.doi.org/10.1016/j.pedn.2017.11.003DOI Listing
September 2018

Minimally invasive, pericardial implantable cardioverter-defibrillator placement in a young child.

J Arrhythm 2017 Oct 26;33(5):523-524. Epub 2017 Jul 26.

University of Tokyo Hospital, Department of Pediatrics, 7-3-1 Hongo, Bunkyoku, Tokyo, Japan.

We report the successful minimally invasive placement of a pericardial implantable cardioverter-defibrillator (ICD) in a 16-kg child. A transvenous ICD dual coil was advanced through a small subxiphoid incision and screwed into the oblique sinus pericardium under fluoroscopic guidance. An additional sense-pace lead was sutured onto the right ventricular apex, and the generator was placed in the upper abdominal wall through the same incision. Threshold testing demonstrated successful defibrillation at 15 J. After implantation, the patient had two episodes of appropriate shock for ventricular fibrillation. The ICD system continues to show stable impedance at 6 months of follow-up.
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http://dx.doi.org/10.1016/j.joa.2017.06.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5634712PMC
October 2017

Spontaneous Rotation of Nonlinear Pattern Formed by Aqueous Colloidal Suspension between ITO Electrodes during Electrolysis Perpendicular to Gravity.

J Phys Chem B 2017 06 2;121(23):5835-5841. Epub 2017 Jun 2.

Department of Organic Materials Science, Yamagata University , 4-3-16 Jyonan, Yonezawa, Yamagata 992-8510, Japan.

A colloidal fluid is found to rotate spontaneously during electrolysis when gravity acts perpendicular to the direction of an applied electric field. An aqueous dispersion containing charged colloidal particles is placed inside an O-ring sandwiched between two parallel ITO electrodes. A clip is used to hold the assembly together to prevent the liquid from leaking out. The assembly is positioned such that the electrodes stand vertically, i.e., the electric field during electrolysis points perpendicular to gravity. When a direct-current voltage is applied to initiate the electrolysis of water, a nonlinear colloidal pattern is formed by electroconvective flow. Moreover, the entire fluid rotates spontaneously about the O-ring center with a constant angular velocity. The rotational dynamics are governed by how strong and where the assembly is clipped relative to the gravitational direction. A new phenomenological relationship between the angular velocity, compression vector, and gravity is derived. Coupling of an electrochemical reduction reaction of the ITO film with electroconvection during electrolysis is proposed as a mechanism for the rotational motion.
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http://dx.doi.org/10.1021/acs.jpcb.7b04009DOI Listing
June 2017

Novel Risk Assessment Tool for Immunoglobulin Resistance in Kawasaki Disease: Application Using a Random Forest Classifier.

Pediatr Infect Dis J 2017 Sep;36(9):821-826

From the *Department of Pediatrics, Kikkoman General Hospital, Chiba, Japan; †Department of Pediatrics, The University of Tokyo, Tokyo, Japan; ‡Department of Pediatrics, Yaizu City Hospital, Shizuoka, Japan; §Department of Pediatrics, Ome Municipal Hospital, Tokyo, Japan; ¶Department of Pediatrics, Ohta-Nishinouchi Hospital, Fukushima, Japan; ‖Department of Pediatrics, Chigasaki Municipal Hospital, Kanagawa, Japan; **Department of Pediatrics, Saitama Citizens Medical Center, Saitama, Japan; and ††Department of Pediatrics, Fujieda Municipal General Hospital, Shizuoka, Japan.

Background: Resistance to intravenous immunoglobulin (IVIG) therapy is a risk factor for coronary lesions in patients with Kawasaki disease (KD). Risk-adjusted initial therapy may improve coronary outcome in KD, but identification of high risk patients remains a challenge. This study aimed to develop a new risk assessment tool for IVIG resistance using advanced statistical techniques.

Methods: Data were retrospectively collected from KD patients receiving IVIG therapy, including demographic characteristics, signs and symptoms of KD and laboratory results. A random forest (RF) classifier, a tree-based machine learning technique, was applied to these data. The correlation between each variable and risk of IVIG resistance was estimated.

Results: Data were obtained from 767 patients with KD, including 170 (22.1%) who were refractory to initial IVIG therapy. The predictive tool based on the RF algorithm had an area under the receiver operating characteristic curve of 0.916, a sensitivity of 79.7% and a specificity of 87.3%. Its misclassification rate in the general patient population was estimated to be 15.5%. RF also identified markers related to IVIG resistance such as abnormal liver markers and percentage neutrophils, displaying relationships between these markers and predicted risk.

Conclusions: The RF classifier reliably identified KD patients at high risk for IVIG resistance, presenting clinical markers relevant to treatment failure. Evaluation in other patient populations is required to determine whether this risk assessment tool relying on RF has clinical value.
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http://dx.doi.org/10.1097/INF.0000000000001621DOI Listing
September 2017

Experiences With Aggressive Cardiac Rehabilitation in Pediatric Patients Receiving Mechanical Circulatory Supports.

Int Heart J 2016 Dec 9;57(6):769-772. Epub 2016 Nov 9.

Department of Rehabilitation, Graduate School of Medicine, The University of Tokyo.

Although some patients with fulminant myocarditis can be rescued owing to the improvements in mechanical circulatory support therapy, there are few reports providing evidence of cardiac rehabilitation during mechanical circulatory supports, particularly among pediatric patients. We treated two pediatric patients who underwent aggressive cardiac rehabilitation during mechanical support. Five days after the initiation of extracorporeal membrane oxygenation therapy aggressive cardiac rehabilitation was started in a 10-year-old girl with fulminant myocarditis. After explantation of the device, she was discharged on postoperative day 23. A 6-year-old girl with fulminant myocarditis started receiving cardiac rehabilitation two days after the initiation of an extracorporeal left ventricular assist device, despite having hemiplegia due to a recent broad stroke. She achieved an exercise capacity of supported walking for 280 meters after 127 days of cardiac rehabilitation and then went abroad to undergo heart transplantation when she was in the best physical condition possible. Early initiation of cardiac rehabilitation may be safe and effective for successful pediatric mechanical circulatory support therapy; this acts as a bridge to explantation or heart transplantation.
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http://dx.doi.org/10.1536/ihj.16-067DOI Listing
December 2016

Clinical implications of mitral valve geometric alterations in children with dilated cardiomyopathy.

Cardiol Young 2016 Oct 28;26(7):1365-72. Epub 2015 Dec 28.

1Division of Cardiology,National Center for Child Health and Development,The University of Tokyo,Tokyo,Japan.

We aimed to elucidate the relationship between severity of secondary mitral regurgitation and mitral valve geometry in children with dilated cardiomyopathy. The medical records of 16 children with dilated cardiomyopathy (median age, 1.2 years; range, 0.4-12.3 years) were reviewed. Mitral valve geometry was evaluated by measuring coaptation depth using echocardiographic apical four-chamber views at the initial presentation. Patients were dichotomised according to the mitral regurgitation severity: patients with moderate or severe secondary mitral regurgitation (n=6) and those with mild secondary mitral regurgitation (n=10). A total of 58 healthy children were considered as normal controls, and a regression equation to predict coaptation depth by body surface area was derived: coaptation depth [mm]=4.37+1.34×ln (body surface area [m2]) (residual standard error, 0.49; adjusted R2, 0.68; p<0.0001). Compared with patients with mild secondary mitral regurgitation, those with moderate or severe secondary mitral regurgitation had significantly larger coaptation depth z-scores (6.4±2.3 versus 1.9±1.4, p<0.005), larger mitral annulus diameter z-scores (3.6±2.6 versus 0.9±1.8, p<0.05), higher left ventricular sphericity index (0.89±0.07 versus 0.79±0.06, p<0.005), and greater left ventricular fraction shortening (0.15±0.05 versus 0.09±0.05, p<0.05). In conclusion, geometric alteration in the mitral valve and the left ventricle is associated with the severity of secondary mitral regurgitation in paediatric dilated cardiomyopathy, which would provide a theoretical background to surgical intervention for secondary mitral regurgitation in paediatric populations.
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http://dx.doi.org/10.1017/S1047951115002668DOI Listing
October 2016

Fever pattern and C-reactive protein predict response to rescue therapy in Kawasaki disease.

Pediatr Int 2016 Mar 23;58(3):180-4. Epub 2016 Feb 23.

Department of Pediatrics, University of Tokyo, Tokyo, Japan.

Background: Evidence to guide rescue therapy in refractory Kawasaki disease (KD) is lacking. The aim of this study was to determine the most important variables in predicting non-response to rescue therapy in refractory KD.

Methods: We retrospectively analyzed 171 patients diagnosed with refractory KD resistant to initial i.v. immunoglobulin (IVIG). Participants received rescue therapy consisting of IVIG monotherapy or IVIG plus prednisolone. Characteristics and laboratory variables were compared between rescue therapy non-responders and responders. Multivariate logistic regression analysis was performed to determine the independent predictors of non-response to rescue therapy.

Results: Among the 171 participants, 54 (31.6%) were non-responders to rescue therapy. On univariate analysis, fever pattern after initial IVIG, day of illness at rescue therapy, rescue therapy regimen and six laboratory variables (pre-IVIG sodium, C-reactive protein [CRP]; post-IVIG white blood cell count, platelet count, sodium, CRP) were useful in discriminating between non-responders and responders. These nine variables were included in multivariate logistic regression analysis. Persistent fever after initial IVIG (aOR, 2.39; 95%CI: 1.07-5.37) and post-IVIG CRP (aOR, 1.09; 95%CI: 1.02-1.17, per 1 mg/dL increase) were identified as independent predictors of non-response to rescue therapy. IVIG rescue monotherapy (aOR, 3.05; 95%CI: 1.05-8.84) also predicted non-response after adjusting for fever pattern and post-IVIG CRP.

Conclusions: Persistent fever and elevated CRP after initial IVIG are predictive of non-response to rescue therapy for refractory KD. For patients at high risk of non-response, IVIG plus prednisolone, or even further intensified rescue therapy regimens may be preferable.
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http://dx.doi.org/10.1111/ped.12762DOI Listing
March 2016

Dissipative energy loss within the left ventricle detected by vector flow mapping in children: Normal values and effects of age and heart rate.

J Cardiol 2015 Nov 13;66(5):403-10. Epub 2015 Jan 13.

Department of Hemodynamic Analysis, Kitasato University School of Medicine, Sagamihara, Japan.

Background: Vector flow mapping (VFM) is a novel echocardiographic technology which enables visualization of the intraventricular flow velocity vector. Dissipative energy loss (EL) derived from the velocity vector field of intraventricular blood flow is considered to reflect the efficiency of blood flow, and could be an indicator of left ventricular function. We aimed to determine the reference values of the EL derived from VFM within the left ventricle.

Methods: VFM analysis was performed using echocardiography in 64 healthy children. The velocity vector fields of the intra-left ventricular blood flow were obtained from the apical 5-chamber view, and the EL values during systole and diastole were calculated. The measurements were averaged over three cardiac cycles, and indexed to body surface area (BSA).

Results: The mean subject age was 6.8±4.3 years. The mean EL was 4.10±2.35mW/m/m(2) BSA during systole and 16.24±11.63mW/m/m(2) BSA during diastole. On multivariate analysis, age and heart rate (HR) were independent predictors of systolic EL, whereas age, HR, and E wave peak velocity were independent predictors of diastolic EL. The regression equations used to predict the BSA-indexed systolic and diastolic EL were as follows: log10 (systolic EL)=-0.0332-0.00213×age (months)+0.00789×HR (beats/min) (adjusted R(2), 0.833; p<0.0001); and log10 (diastolic EL)=0.277-0.00346×age (months)+0.00570×HR (beats/min)+0.00564×E wave peak velocity (cm/s) (adjusted R(2), 0.867; p<0.0001).

Conclusions: The systolic and diastolic EL were positively correlated with HR and negatively correlated with age. Moreover, the diastolic EL was positively correlated with the E wave peak velocity. The present study provides reference values for the systolic and diastolic EL that can be used in future studies examining patients with heart disease.
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http://dx.doi.org/10.1016/j.jjcc.2014.12.012DOI Listing
November 2015

Central extracorporeal membrane oxygenation requiring pulmonary arterial venting after near-drowning.

Am J Emerg Med 2014 Feb 2;32(2):197.e1-2. Epub 2013 Oct 2.

Department of Cardiothoracic Surgery, The University of Tokyo, Tokyo, Japan.

Extracorporeal membrane oxygenation (ECMO) is an effective respiratory and circulatory support in patients in refractory cardiogenic shock or cardiac arrest. Peripheral ECMO sometimes requires left heart drainage; however, few reports state that pulmonary arterial (PA) venting is required during ECMO support. We present a case of a 14-year-old boy who required PA venting during ECMO support after resuscitation from near-drowning in freshwater. A biventricular assist device with an oxygenator implantation was intended on day 1; however, we were unable to proceed because of increasing of pulmonary vascular resistance from the acute lung injury. Central ECMO with PA venting was then performed. On day 13, central ECMO was converted to biventricular assist device with an oxygenator, which was removed on day 16. This case suggests that PA venting during ECMO support may be necessary in some cases of respiratory and circulatory failure with high pulmonary vascular resistance after near-drowning.
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http://dx.doi.org/10.1016/j.ajem.2013.09.031DOI Listing
February 2014

Serum hyaluronic acid concentration in Fontan circulation: correlation with hepatic function and portal vein hemodynamics.

Pediatr Cardiol 2014 Apr 26;35(4):608-15. Epub 2013 Oct 26.

Department of Pediatrics, Graduate School of Medicine, University of Tokyo, Tokyo, Japan,

Although liver fibrosis causes significant morbidity in the late postoperative period of the Fontan procedure, the diagnostic value of hyaluronic acid (HA), a serum marker of liver fibrosis, has not been established in Fontan patients. The purpose of this study was to determine whether increased serum HA concentration in Fontan patients is associated with an increase in inspiratory-to-expiratory flow rate ratio (Qin/Qex) of the portal vein (PV), which is indicative of liver fibrosis. We retrospectively studied 28 consecutive patients with Fontan circulation who underwent cardiac catheterisation for various indications. The median age at examination was 5.5 years (range 2.2-5.6). The median HA concentration was 17.7 ng mL(-1) (range 10.0-82.1), which was used to divide our 28 patients into two groups. Patients in the high-HA group had significantly greater Qin/Qex of the PV than those in the low-HA group (1.25 ± 0.12 vs. 1.12 ± 0.11, p < 0.05). Platelet counts were significantly lower in the high-HA group (216 ± 74 vs. 294 ± 104 × 10(9) L(-1), p < 0.05). No significant difference was found in inferior vena caval pressure. In conclusion, increase of HA concentration in Fontan patients accompanies the change in PV hemodynamics peculiar to liver cirrhosis and might be an early indicator of liver fibrosis.
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http://dx.doi.org/10.1007/s00246-013-0827-9DOI Listing
April 2014

Treatment strategy and long-term prognosis for patients with esophageal atresia and congenital heart diseases.

Pediatr Cardiol 2013 Jan 26;34(1):64-9. Epub 2012 May 26.

Department of Pediatrics, Graduate School of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.

A review examined six consecutive cases of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) who underwent cardiac surgery at the authors' institution between 2001 and 2011 for associated complex congenital heart diseases. All the patients had a normal karyotype and showed EA with distal TEF. In all cases, gastrostomy was the initial surgical intervention. Cardiac surgery was performed concurrently with gastrostomy for one patient who had a total anomalous pulmonary venous connection with pulmonary venous obstruction. For two patients with duct-dependent pulmonary circulation, EA/TEF was corrected in the neonatal period, and an aortopulmonary shunt operation was electively performed after the first month of life. For two patients with duct-dependent systemic circulation, repair of EA/TEF was performed concurrently with gastrostomy, followed by palliative cardiac surgery during the neonatal period. For another patient without duct-dependent circulation, repair of EA/TEF was performed in the neonatal period. No mortality occurred during a median follow-up period of 6.2 years. However, respiratory complications including severe tracheomalacia for two patients, recurrent episodes of respiratory infection for three patients, and severe gastroesophageal reflux for five patients caused considerable long-term morbidity.
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http://dx.doi.org/10.1007/s00246-012-0386-5DOI Listing
January 2013

Outcome of prenatally diagnosed isolated congenital complete atrioventricular block treated with transplacental betamethasone or ritodrine therapy.

Pediatr Cardiol 2009 Jan 26;30(1):35-40. Epub 2008 Jul 26.

Division of Pediatric Cardiology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo 157-8535, Japan.

The effectiveness of transplacental drug therapy for prenatally diagnosed isolated congenital complete atrioventricular block (CCAVB) is controversial. Nine cases of prenatal isolated CCAVB were treated from 2002 to 2007. Ritodrine was administered transplacentally to all fetuses and betamethasone to those whose mothers tested positive for maternal anti-SSA/Ro antibodies. Six of the nine patients had an anti-SSA/Ro-positive mother and received transplacental betamethasone 4 mg/day at a median gestational age of 28 weeks (range, 24-31 weeks). No patients exhibited an improvement in the degrees of complete heart block, and one patient died in utero. No serious adverse events occurred. After the mean follow-up period of 1.7 +/- 1.3 years, all five patients treated with transplacental betamethasone experienced a good cardiac function, whereas one of the three patients not treated with transplacental betamethasone experienced cardiomyopathy and died at the age of 4 months. Pacemaker implantation was required for seven of the eight live-born infants. Transplacental betamethasone therapy for the patients with isolated CCAVB neither improved the degree of atrioventricular block nor decreased the rate of patients requiring pacemaker implantation, but it probably reduced the risk for the development of myocardial disease.
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http://dx.doi.org/10.1007/s00246-008-9273-5DOI Listing
January 2009
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