Sarcomere gene variants did not improve cardiac function in pediatric patients with dilated cardiomyopathy from Japanese cohorts.

Dilated cardiomyopathy (DCM) is a progressive myocardial disorder characterized by impaired cardiac contraction and ventricular dilation. However, some patients with DCM improve when experiencing left ventricular reverse remodeling (LVRR). Currently, the detailed association between genotypes and clinical outcomes, including LVRR, particularly among children, remains uncertain.

Global longitudinal strain is a surrogate marker for time constant of isovolumic relaxation in post-Fontan operation patients with single right ventricle and preserved ejection fraction.

Background: The time constant of isovolumic relaxation is an established index of ventricular relaxation, a major component of diastolic function, even in a single right ventricle. However, the specific echocardiographic parameters for estimating diastolic dysfunction are insufficient for a single right ventricle. This study aimed to investigate the echocardiographic indices associated with time constant of isovolumic relaxation in post-Fontan operation patients with a single right ventricle.

The Ventricular Hypokinesis at the Apical Pacing Site due to the Early Shortening and Rebound Stretch in a Case with Fontan Procedure.

The left ventricular (LV) apex is recommended as the first choice for positioning the epicardial pacing. We encountered a patient with congenital heart disease (CHD) showing hypokinesis of the LV apical pacing site after implantation of a pacemaker with epicardial leads. This phenomenon was revealed by the early shortening and systolic rebound stretch of the same lesion on two-dimensional speckle tracking echocardiography, which developed in the intraventricular dyssynchrony between the LV apex and base.

Catheter Intervention for Flow Regulatory Clips on Palliative Shunts and Conduits in Patients with Congenital Heart Disease.

Catheter intervention (CI) for a Blalock-Taussig shunt (BTS) or a ventricle-to-pulmonary artery conduit (VPC) is often required after a palliative surgery for congenital heart disease. Flow regulatory clips help improve interstage mortality; their use necessitates CIs to prevent cyanosis. To study the CI outcomes in patients who underwent palliative surgery with either BTSs or VPCs with flow regulatory clips.

Predictors of long-term mortality among perioperative survivors of Fontan operation.

Aims: The criteria for 'good' Fontan haemodynamics have been poorly defined in relation to long-term outcomes. The aim of this study was to identify the risk factors for mortality among haemodynamic parameters obtained early after the Fontan operation.

Methods And Results: Clinical data of all perioperative survivors of the Fontan operation performed before 2011, from nine institutions, were collected through a retrospective chart review.

Outcomes of hypertrophic cardiomyopathy in Japanese children: a retrospective cohort study.

There has been no multicenter study on the prognosis of pediatric hypertrophic cardiomyopathy (HCM) in Japan. Therefore, we conducted a retrospective multicenter observational study on the long-term survival rate in patients diagnosed with HCM under the age of 18 between 1990 and 2014. Twenty institutions participated.

Outcomes of Dilated Cardiomyopathy in Japanese Children - A Retrospective Cohort Study.

Background: There has been no nationwide survey on the prognosis of pediatric dilated cardiomyopathy (DCM) in Japan. Therefore, we designed this retrospective multicenter study to investigate the long-term survival rate in pediatric patients with DCM in Japan.

Methods and results: In this multicenter retrospective observational study, data were reviewed for 106 patients aged <18 years who had been diagnosed with DCM at any 1 of 18 Japanese institutions between 1990 and 2014.

Angiographic diagnosis for accurate assessment of congenital porto-systemic shunt and extrahepatic portal vein obstruction in children.

Background: Congenital porto-systemic shunt (CPSS) is a rare disease and can cause fatal complications. Accurate angiographic assessment is mandatory for proper treatment. Although technically difficult, we developed assessment techniques and assessed their accuracy.

Adult case of tetralogy of Fallot associated with atrioventricular septal defect.

The patient was a 28-year-old woman who was misdiagnosed with tetralogy of Fallot and straddling mitral valve after birth. She underwent a left modified Blalock-Taussig shunt at the age of 1 year. At age 28, she presented with fatigue and progressive cyanosis.

Successful transcatheter closure of coronary artery fistula in a patient with anomalous aortic origin of the left main coronary artery from the right aortic sinus.

Transcatheter closure is an established method to treat coronary artery fistula (CAF). We present transcatheter closure in a 6-year-old girl with CAF and anomalous aortic origin of the left main coronary artery from the right aortic sinus. The CAF originated from the left coronary artery (LCA), coursed through the interventricular septum (intraseptal course) with prominent dilation, and drained into the right ventricular outflow tract.

Antenatal Therapy for Fetal Supraventricular Tachyarrhythmias: Multicenter Trial.

Background: Standardized treatment of fetal tachyarrhythmia has not been established.

Objectives: This study sought to evaluate the safety and efficacy of protocol-defined transplacental treatment for fetal supraventricular tachycardia (SVT) and atrial flutter (AFL).

Methods: In this multicenter, single-arm trial, protocol-defined transplacental treatment using digoxin, sotalol, and flecainide was performed for singleton pregnancies from 22 to <37 weeks of gestation with sustained fetal SVT or AFL ≥180 beats/min.

Anomalous origin of the coronary artery coursing between the great vessels presenting with a cardiovascular event (J-CONOMALY Registry).

Aims: Anomalous origin of the coronary artery (AOCA) with an inter-arterial course (IAC) between the great vessels poses a risk for a life-threatening cardiovascular event. We assessed, in a registry-based study, the clinical features, treatment strategies, and prognoses of life-threatening cardiovascular events ensuant to AOCA.

Methods And Results: Included were 65 AOCA patients (48 men/17 women, aged 41 ± 23 years) from 40 clinical centres who had experienced sudden cardiac arrest (SCA) (n = 30), acute myocardial infarction (AMI) (n = 5), angina (n = 23), or syncope (n = 7).

In-Depth Insight Into the Mechanisms of Cardiac Dysfunction in Patients With Type 1 Diabetes Mellitus Using Layer-Specific Strain Analysis.

Background: Although the subclinical left ventricular (LV) dysfunction caused by diabetes mellitus (DM) results in a high risk of death and heart failure, the details of cardiac dysfunction across a wide age range remain unclear. The aim of this study was to assess LV dysfunction in patients with type 1 DM (T1DM) using layer-specific strain analysis by echocardiography.

Methods and results: The 52 patients (median age: 23 [range: 5-40] years) with T1DM were divided into 3 age groups (D1: 5-14 years, D2: 15-24 years, D3: 25-40 years); 78 age- and sex-similar controls were divided into 3 corresponding groups (C1, C2, and C3).

Role of BRCA1-associated protein (BRAP) variant in childhood pulmonary arterial hypertension.

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.

New insight into the intraventricular pressure gradient as a sensitive indicator of diastolic cardiac dysfunction in patients with childhood cancer after anthracycline therapy.

Cardiac dysfunction due to cardiotoxicity from anthracycline chemotherapy is a leading cause of morbidity and mortality in survivors of childhood cancer. The intraventricular pressure gradient (IVPG) of the left ventricle (LV) is the suction force of blood from the left atrium to the LV apex during early diastole and is a sensitive indicator of diastolic function. We assessed IVPG as a new indicator of the cardiac dysfunction in survivors of childhood cancer after anthracycline therapy.