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Kawasaki disease (KD) is an acute febrile illness of childhood characterized by systemic vasculitis, especially coronary arteritis. Aortic valve regurgitation (AVR) is a relatively common complication. There have been no reports to date of heart failure and left ventricular non-compaction (LVNC) after acute KD, although the precise etiology of this condition remains unclear. A 6-month-old boy with KD was admitted to hospital. Despite high-dose i.v. gammaglobulin for dilation of the coronary artery, moderate AVR appeared, and thereafter he developed heart failure. A rough, dense LV myocardium indicated LVNC. On genetic testing a heterogenous 163G > A substitution changing a valine to isoleucine in LIM domain binding protein 3 ( LDB3) was identified. Additional cardiac stress, such as that caused by AVR and/or KD might have triggered cardiac failure in the form of LVNC due to LDB3 mutation. [ABSTRACT FROM AUTHOR]

To assess the reliability and validity of the Japanese translation version of the Pediatric Quality of Life Inventory™ 4.0 Short Form 15 (PedsQL™ 4.0 SF15). The PedsQL™ 4.0 SF15 was administered to 229 schoolchildren aged 6–13 years and 100 pediatric outpatients aged 5–18 years and their parents. Internal consistency reliability exceeded 0.70 for both proxy-reported and self-reported scales. Test–retest reliability demonstrated large values for parent proxy-report (range: 0.68–0.79) and moderate to large values for child self-report (range: 0.46–0.73). Parent proxy-report health-related quality of life (HRQOL) was higher than child self-report in all scales except for School Functioning. The correlations between the reports of the parents and children were moderate to high. Gender differences were observed in Social Functioning, School Functioning, and Psychosocial Health Summary, with girls reporting higher HRQOL than boys. Factor analysis indicated that four factors were extracted from the PedsQL™ 4.0 SF15 and these four factors corresponded mainly to the four scales. Known groups validity was established for proxy-report and self-report with higher HRQOL being reported for healthy children than those with psychosomatic complaints including headache and abdominal pain. The Japanese translation version of the PedsQL™ 4.0 SF15 demonstrates good reliability and validity and could be used as a measure of HRQOL for transcultural comparisons of pediatric research in school settings and healthcare services research. [ABSTRACT FROM AUTHOR]

Background: The purpose of the present study was to establish the normal values of flow propagation velocity (FPV) in healthy children and examine the variables that affect FPV in clinical situations. Methods: Two hundred and thirty- five healthy children and adolescents were assessed (aged 0–22.6 years, mean age 7.4 ± 5.4 years; male, n = 142; female, n = 93). FPV was obtained from an apical four-chamber view and determined as the slope of aliasing velocity of early diastolic transmitral flow on the color M-mode using Aloka SSD-5500 with 5.0 MHz transducer. Aliasing velocity was set at 50–70% of the peak transmitral flow velocity. Peak transmitral flow velocities in early diastole (E) and during atrial contraction (A), and the ratio of early to late peak velocity (E/A) were obtained. Tei index was also measured for analysis of general left ventricular performance. Left ventricular mass index (LVMI) was obtained from conventional echo measurement. E, E/A, Tei index and LVMI were compared with FPV in healthy subjects. Results: FPV obtained from all subjects ranged from 23.7 to 96.0 cm/s (61.3 ± 13.6 cm/s). Normal value of FPV was less dependent on age, body size, heart rate and left ventricular dimension. In contrast, although there was no significant correlation between FPV and ejection fraction, statistically significant correlation was found between FPV, LVMI ( P = 0.0008) and Tei index ( P = 0.025). Conclusions: FPV is independent of age, body size and heart rate and is useful to assess left ventricular relaxation in children. [ABSTRACT FROM AUTHOR]

T-wave inversion (TWI) is not considered useful for diagnosing pediatric arrhythmogenic right ventricular cardiomyopathy (ARVC), because right precordial TWI in ARVC resembles a normal juvenile pattern. The aims of this study were to clarify the electrocardiographic (ECG) characteristics of pediatric ARVC to distinguish those patients from healthy children. Between 1979 and 2017, 11 ARVC patients under 18 years old were registered and compared with school screening ECGs from 48,401 healthy children. The mean age at the first arrhythmic event or diagnosis was 13.3 ± 4.7 years. Nine patients were asymptomatic initially and were found by ECG screening, but 6 developed severe symptoms during the follow-up. Healthy children had a normal juvenile pattern, while ARVC children, especially symptomatic patients, had a significant tendency to have inferior and anterior TWI. The phenomenon of T-wave discontinuity (TWD) in which the TWI became deeper from V1 to V3 and suddenly turned positive in V5 was significantly more frequent in ARVC (60%) than healthy children (0.55%). Anterior TWI and TWD were also significantly more frequent in those who developed severe symptoms. The sensitivity and specificity of TWD were 60% (95% CI, 31–83%), and 99% (95% CI, 99–99%) to distinguish ARVC from healthy children, as well as 100% (95% CI, 71–100%) and 80% (95% CI, 51–80%), respectively, to predict severe symptoms in the future. The ECG is useful to distinguish ARVC children, even in the early phase. Anterior TWI and TWD could detect ARVC children and to predict the possible serious conditions. • This study clarified the frequency of T-wave inversion (TWI) in a large cohort of healthy children. • T-wave discontinuity (TWD), as well as TWI in the inferior and anterior leads could differentiate ARVC children. • TWD and anterior TWI are possible signs to predict a serious prognosis in the future. • ARVC children tend not to have VTs but have PVCs and late potentials. [ABSTRACT FROM AUTHOR]