Rare pediatric case of two episodes of fulminant myocarditis and cardiac dysfunction after viral infection.

Unlabelled: Myocarditis presents with a broad spectrum of clinical severity, ranging from subclinical illness to sudden death. Children with fulminant myocarditis often require inotropic or mechanical circulatory support; however, recurrent acute myocarditis is extremely rare. There is limited evidence to guide the management of recurrent acute myocarditis because the relevant literature is sparse.

Risk factors for development of ventricular tachycardia in patients with ventricular premature contraction with a structurally normal heart.

Background: We examined risk factors for development of ventricular tachycardia (VT) in pediatric patients with ventricular premature contractions (VPCs) and a structurally normal heart.

Methods: The subjects were 81 844 first graders and 88 244 seventh graders of Kagoshima City School-based cardiovascular screening (SCV-screening) between 2001 and 2015. We retrospectively reviewed the clinical data of students who were diagnosed as having VPC.

Probability of diagnosing long QT syndrome in children and adolescents according to the criteria of the HRS/EHRA/APHRS expert consensus statement.

Aims: The present study aimed to determine the probability of diagnosing long QT syndrome (LQTS) in children and adolescents based on the HRS/EHRA/APHRS criteria for LQTS. We used data of a school-based electrocardiographic screening programme in Japan.

Methods And Results: The total numbers of subjects who participated in the screening programme between 2008 and 2013 in Kagoshima, Japan, were 33 051 first- and 34 751 seventh-grade students, aged 6 and 12 years, respectively.

Fatal systemic air embolism in a neonate with absent aortic valve.

A 32-year-old pregnant woman was referred at 33 weeks' gestation for prenatal ultrasound demonstrating fetal hydrops due to absent aortic valve with free aortic valve insufficiency. Elective caesarian section at 34 week's gestation was performed. Surgical intervention was planned immediately after labor at which time mitral valve closure and atrial septostomy using cardiopulmonary bypass would be performed.

Impact and issues of detecting fetal congenital heart defects in Kyushu, Japan.

Aim: To determine the current status of fetal CHD screening in our region and to establish a CHD screening system in Japan.

Material And Methods: Subjects were 168 fetuses prenatally-diagnosed with CHD at four referral centers in Japan from 2003 to 2007. Subjects were divided into two groups: group A (n = 84) included cases without extracardiac sonographic abnormalities and known risk factors for CHD and group B (n = 84) included those with extracardiac sonographic abnormalities or risk factors.

Mutation of junctophilin type 2 associated with hypertrophic cardiomyopathy.

Junctophilin subtypes, designated as JPH1 approximately 4, are protein components of junctional complexes and play essential roles in cellular Ca2+ signaling in excitable cells. Knockout mice lacking the cardiac-type Jph2 die of embryonic cardiac arrest, and the mutant cardiac myocytes exhibit impaired formation of peripheral couplings and arrhythmic Ca2+ signaling caused by functional uncoupling between dihydropyridine and ryanodine receptor channels. Based on these observations, we hypothesized that mutations of JPH2 could cause human genetic cardiac diseases.