Past Endemic Izumi Fever or Yersinia pseudotuberculosis Infection Which Sporadically Reappears.

Izumi fever (IF), also known as Far East scarlet-like fever (FESLF), is caused by Yersinia pseudotuberculosis and it has clinical features resembling those of Kawasaki disease (KD). As both diseases are rare in adolescents and young adults, it is challenging to recognize them, thus often leading to a delayed diagnosis. We herein present two cases of IF or FESLF (IF/FESLF). The first case was misdiagnosed as KD, which led to a diagnostic delay. The second case was recognized earlier owing to our experience with the first case. Although cultures were negative in both cases, presumably due to the prior use of antimicrobial agents, our clinical suspicion and a paired serological assay for anti-Yersinia pseudotuberculosis antibodies finally led to a successful diagnosis.

A geometrical pitfall of Area-Length method; -Is left ventricle volume evaluation of repaired Tetralogy of Fallot by angiocardiography accurate?

Biplane Area-Length (AL) method by left ventriculography (LVG) has been widely adopted as a standard method to estimate left ventricular volume. However, we have experienced difficulties in adopting the value by AL method for the children with Tetralogy of Fallot (TOF) due to the discrepancy among volumetric modalities. This study validated some limitations of AL method, considering the basic principles of its formulation. A single center retrospective cohort study was conducted for 1 year. The confirmed 22 cases with repaired TOF at our hospital were enrolled. The clinical characteristics, some cardiac MRI analyses, and all the cardiac catheterization studies were collected. Angiographic data were compared with historic cohorts of Kawasaki disease without any coronary artery lesions by using AL method. Cardiac MRI analyses of ten TOF patients were additionally available. LVG studies showed that the length of the long axis on anteroposterior view (AP) was not equal to that on lateral view (LT) due to anatomically apical elevation in TOF, followed by a significant difference found in the sagittal lengths of the LV long axis between AP and LT (P = 0.003). Because the difference critically affected the formula depending on biplane AL method, the calculated LVEDV of TOF group appeared overestimated, compared with the control group (TOF vs control group: 119.5% ± 6.3% vs 96.4 ± 3.5% of Normal, P = 0.006). Available cardiac MRI analyses of some patients in TOF group revealed 55% increase of LVEDV by AL method (angiocardiography 116 ± 7.0 vs CMR 75 ± 3.7 ml/m2, P = 0.0025). A pitfall exists when applying biplane AL method to measure LV volume especially for TOF patients, because the long axis on AP view is not always identical to that on LT view.

A Case Report of a Rare Heterozygous Variant in the Desmin Gene Associated With Hypertrophic Cardiomyopathy and Complete Atrioventricular Block.

Hypertrophic cardiomyopathy (HCM) is the primary cause of sudden cardiac death in children and adolescents. Patients with HCM frequently have ventricular tachycardia and ventricular fibrillation, although complete atrioventricular block (CAVB) is very rare. We report a case of HCM with CAVB in an 8-year-old girl who underwent transvenous implantable cardioverter-defibrillator placement after resuscitation. In this patient, we identified a de novo heterozygous missense variant, Arg406Trp (c.1216C > T), in the desmin (DES) gene. Pathogenic variants in the DES gene result in cardiomyopathy, conduction disorders, and skeletal muscle weakness. This recently identified variant may cause HCM with CAVB.

La cardiomyopathie hypertrophique (CMH) est la première cause de mort subite d'origine cardiaque chez les enfants et les adolescents. Les patients atteints de CMH présentent fréquemment une tachycardie ventriculaire et une fibrillation ventriculaire, bien que le bloc auriculo-ventriculaire complet (BAVC) soit très rare. Nous rapportons un cas de CMH avec BAVC chez une fillette de 8 ans qui a reçu un défibrillateur cardioverteur implantable par voie transveineuse après réanimation. Chez cette patiente, nous avons isolé un variant faux sens hétérozygote de novo, Arg406Trp (c.1216C > T), dans le gène de la desmine (DES). Les variants pathogènes du gène DES entraînent une cardiomyopathie, des troubles de la conduction et une faiblesse des muscles squelettiques. Ce variant récemment identifié peut causer une CMH avec BAVC.

How does the situation before a tackle influence a tackler's head placement in rugby union?: application of the decision tree analysis.

OBJECTIVES: Tacklers need to decide where to place their head based on the evasive manoeuvres of the ball-carrier and positional relationship with the ball-carrier before tackle. Therefore, it is difficult for tacklers to improve incorrect head placement at the moment of contact. Moreover, the characteristics prior to tackle have a relationship with the tackler's head placement. However, how situations lead to incorrect head placement remains unknown. The aim of this study was to identify pre-contact situations that lead to incorrect head placement by using decision tree analysis. METHODS: Tackles leading to concussions were used to identify events that provoked injury using the video recordings of matches. Injury-free tackle was used as a control. All tackles were classified according to head placements and coded from seven pre-contact factors configured aspect of both tacklers and ball-carriers. RESULTS: Three situations that led to incorrect head placement were identified. Evasive manoeuvres implemented by the ball-carrier significantly contributed to the head placement at the time of contact. CONCLUSION: Our findings suggest that tacklers should keep their heads up to identify the movements of the ball-carrier, which might lead to tackling the head on the correct side at the moment of tackling and decrease the risk of tackler-related concussions.

A case report: Twin sisters with restrictive cardiomyopathy associated with rare mutations in the cardiac troponin I gene.

Restrictive cardiomyopathy (RCM) is a rare cardiomyopathy in children, and its prognosis until now, has been poor. Recently some sarcomeric mutations have been reported as disease-causing genes of RCM. However, the genotype-phenotype correlation is not fully understood. Additionally, prognostic factors including sudden death in patients with RCM have not been elucidated. We report our experience in treating twin sisters with RCM or hypertrophic cardiomyopathy with RCM phenotype, both carriers of the same mutation in TNNI3, which encodes one of the major sarcomeric proteins in myofibrils. They were both diagnosed with RCM by cardiac catheterization at the age of 11 years. Despite appropriate follow-up and medical treatment, one died suddenly at the age of 11 years and the other also died at the age of 15 years due to heart failure while awaiting heart transplantation. In addition to our cases, other reports of younger fatal cases with RCM carrying TNNI3 mutations may suggest it as one of the prognostic factors. Genetic diagnosis is important in the clinical diagnosis, management, and treatment of cardiomyopathy. .

Characteristics and factors of concussion events for tacklers in collegiate rugby union.

The purpose of this study was to determine the factors related to the occurrence of concussion in rugby tacklers. Match video records showing tackles leading to concussion were used to identify injury-inciting events. Additionally, noninjury tackles by concussed tacklers (prior to concussion) and position-matched tacklers from the same matches were used as a control cohort. All tackles were coded according to the tackling characteristics. The odds ratio (OR) was reported by logistic regression. The results demonstrated that a side step of the ball-carrier reduced the risk of concussion for tacklers (OR = 0.13 [95% CI, 0.03-0.58]; P = .008). Conversely, the tackler's head/neck contacting the ball-carrier (OR = 18.62 [95% CI, 4.59-75.49]; P < .001) and not remaining bound to the ball-carrier since making initial contact (OR = 4.38 [95% CI, 1.69-11.34]; P = .002) were identified as risk factors for the concussion of tacklers. These results suggest that avoidance movements of the ball-carrier prior to tackling reduced the risk of concussion and that incorrect tackling techniques contributed to an increased occurrence of concussion. Furthermore, the probability of concussion for tacklers increased when their head was in front or to one side of the ball-carrier, and the direction of the tackle also affected the risk of concussion, especially from the side. Therefore, it is important to emphasize the tackler's head position in conjunction with the direction of tackle.

Significance of right atrial tension for the development of complications in patients after atriopulmonary connection Fontan procedure: potential indicator for Fontan conversion.

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.

Serum concentration of procollagen type III amino-terminal peptide is increased in patients with successfully repaired coarctation of the aorta with left ventricular hypertrophy.

Pathological left ventricular hypertrophy (LVH) with myocardial fibrosis is an independent risk factor for cardiovascular mortality. Previous studies indicated that patients with coarctation of the aorta (CoA) have increased left ventricular mass (LVM) including LVH, even after successful CoA repair. It is unclear whether the increased LVM is pathological one with cardiac fibrosis. Group A consisted of 17 patients with successfully repaired CoA. Group B consisted of 17 postoperative subjects who matched the age and postoperative periods of group A. Group C comprised 28 subjects for the geometric standard of the left ventricle. The LVM index (LVMI) and the relative wall thickness (RWT) of group A and B were compared with the values of 17 age-matched subjects from group C. The serum concentration of procollagen type III amino-terminal peptide (P-III-P), a biomarker for myocardial fibrosis, in group A was compared with the concentration in group B. The correlations between the serum P-III-P concentration and LVMI and RWT were studied in group A and non-A group. In group A, RWT and LVMI were significantly higher than those in group C (0.37 ± 0.05 vs. 0.31 ± 0.02, p < 0.01; 44.8 ± 11.2 vs. 36.5 ± 7.6, p = 0.04, respectively), and the serum P-III-P concentration was significantly higher than that in group B (1.59 ± 0.74 vs. 1.07 ± 0.33, p = 0.04). Serum P-III-P concentrations were well correlated with RWT and LVMI (r = 0.89, p < 0.01; r = 0.63, p < 0.01, respectively) in group A. LVH in patients with successfully repaired CoA may have an abnormal pathogenesis associated with myocardial fibrosis.

The cause of B-type natriuretic peptide elevation and the dose-dependent effect of angiotensin-converting enzyme inhibitor on patients late after tetralogy of Fallot repair.

Patients after surgical repair of tetralogy of Fallot (TOF) may experience various complications that result in neurohormonal activation, including plasma B-type natriuretic peptide (BNP) elevation. Right ventricular (RV) dilation is a frequent complication, and few treatments are available. This study aimed to identify the factor or factors leading to BNP elevation and to clarify the effects of angiotensin-converting enzyme inhibitor (ACE-I) on changes in BNP levels in patients with repaired TOF. Plasma BNP levels and hemodynamic data derived from cardiac catheterization were analyzed. In addition, longitudinal BNP levels and ACE-I dosages were analyzed for patients administered ACE-I. For 31 patients with repaired TOF, who mainly had RV dilation, log BNP levels were significantly correlated with the RV end-diastolic volume index (P = 0.02) as well as ventricular volume and pressure (P < 0.01). For 11 patients medicated with ACE-I, BNP levels were significantly lower at the time of maximal ACE-I dosage than at the time of minimal dosage (P < 0.01). Furthermore, BNP levels decreased as the ACE-I dosage per body weight increased (P < 0.01). In conclusion, elevation of BNP in patients after TOF repair could reflect volume and pressure load in the RV end-diastolic phase, and ACE-I may reduce BNP levels in a dose-dependent manner.

Aortic pressure wave reflection in children.

Aortic pressure wave reflection is one of the risk factors for developing cardiovascular disease in adults, and the degree of pressure wave reflection increases with aging. However, enhanced pressure wave reflection has also been reported in infants and children. The purpose of this study was to clarify pressure wave reflection during childhood and to determine the reference for the augmentation index, which is one of the most useful parameters used to represent the degree of aortic pressure wave reflection in children. This study enrolled 72 patients with normal aortic circulation. The aortic pressure waveform was recorded using a pressure sensor-mounted catheter, and the augmentation index was thus calculated. The augmentation index tended to decrease with increasing age until around 15 years of age and then increased thereafter. For children below 15 years of age, multiple regression analysis revealed a significant negative correlation between body height and augmentation index. In children, increases in aortic pressure wave reflection are probably attributable to changes in body height.

Aortopexy for tracheomalacia with dextrocardia, pulmonary artery sling, and congenital tracheal stenosis.

We report a rare case of coexisting pulmonary artery sling, congenital tracheal stenosis, and dextrocardia caused by right lung hypoplasia. Successful treatment of severe postoperative tracheomalacia was achieved by aortopexy, aiming displacement of the aortic arch across orthogonally in front of the trachea due to dextrocardia. The aim of this surgery was different from the usual aortopexy for tracheomalacia, which lifts the tracheal wall with the aorta. The three-dimensional evaluation considering the patient's associated malformations led to a successful result.