Efficiency and feasibility of semi-automated software for measuring left atrial volume in routine echocardiography in a pediatric population.

Purpose: The traditional method for measuring left atrial volume (LAV) involves manual tracing. Recently, semi-automated techniques for measuring LAV, based on 2D speckle tracking echocardiography (STE) and 3D echocardiography (3DE), have become commercially available. This study aimed to investigate the efficiency and feasibility of these semi-automated software methods for LAV measurement in pediatric patients.

Modeling of multiple atrial septal defects with inferior vena cava rim deficiency: Three-dimensional visualization of the rim based on transthoracic echocardiographic imaging.

The morphologic features of the multiple atrial septal defects assessed by TTE-based 3D imaging were similar to those by 3D-TEE. TTE-based 3D model had excellent visibility, allowing observation of 3D structure of the rims of the defects. It may be useful method for assessment of the multiple atrial septal defects.

Electrocardiographic Changes with Age in Japanese Patients with Noonan Syndrome.

Little information is available on age-related electrocardiographic changes in patients with Noonan syndrome. This single-center study evaluated the electrocardiograms of patients with Noonan syndrome. We divided the patients ( = 112; electrocardiograms, 256) into four groups according to age: G1 (1 month-1 year), G2 (1-6 years), G3 (6-12 years), and G4 (>12 years).

Use of the index of pulmonary vascular disease for predicting long-term outcome of pulmonary arterial hypertension associated with congenital heart disease.

Aims: Limited data exist on risk factors for the long-term outcome of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH). We focused on the index of pulmonary vascular disease (IPVD), an assessment system for pulmonary artery pathology specimens. The IPVD classifies pulmonary vascular lesions into four categories based on severity: (1) no intimal thickening, (2) cellular thickening of the intima, (3) fibrous thickening of the intima, and (4) destruction of the tunica media, with the overall grade expressed as an additive mean of these scores.

Assessment of the left ventricular volume of tetralogy of Fallot by electrocardiogram.

Background: Sufficient left ventricular volume is required for patients with tetralogy of Fallot (TOF) who are going to have biventricular repair. In this study, we investigated the utility of the electrocardiogram to evaluate left ventricular volume in patients with TOF.

Method: Patients whose left ventricular (LV) end-diastolic volume was lower than 80% of normal were defined as having a small LV.

Cardiac features of Noonan syndrome in Japanese patients.

Background: Cardiovascular disease is one of the most important problems in long-term follow-up for Noonan syndrome. We examined cardiovascular issues and clinical manifestations, with a focus on the cardiovascular disease and prognosis of patients with Noonan syndrome.

Methods: This single-centre study evaluated patients who were clinically and genetically diagnosed with Noonan syndrome.

A retrospective study of perioperative clinical seizures and epilepsy in children after operation for CHD.

This study investigated the incidence and risk factors of perioperative clinical seizure and epilepsy in children after operation for CHD. We included 777 consecutive children who underwent operation from January 2013 to December 2016 at Kanagawa Children's Medical Center, Kanagawa, Japan. Perinatal, perioperative, and follow-up medical data were collected.

Correlation of exercise-induced peripheral venous hypertension with exercise intolerance in patients with Fontan circulation.

Owing to the absence of a sub-pulmonary ventricle, the central venous pressure rises in patients with Fontan circulation. During exercise, central venous pressure may rise further to increase the systemic ventricular preload and cardiac output. We performed a single-centre prospective trial of cardiopulmonary exercise test while monitoring peripheral venous pressure which strongly correlates with central venous pressure.

Transient phrenic nerve palsy induced by cardiac catheterisation in an infant.

We report on a 7-month-old male with transient phrenic nerve palsy induced by diagnostic cardiac catheterisation. The phrenic nerve palsy, which is a rare complication, was due to extravascular bleeding from a branch of the internal mammary artery.

Fetal case of right atrial isomerism with infracardiac total anomalous pulmonary venous connection and agenesis of the ductus venosus.

After birth, the ductus venosus becomes an important route connecting the pulmonary and systemic venous systems for survival in infracardiac total anomalous pulmonary venous connection. We encountered a fetal case of right atrial isomerism with infracardiac total anomalous pulmonary venous connection and agenesis of ductus venosus. Prenatal echocardiography suggested that the fetus had severe pulmonary venous obstruction; however, no obstructive lesions were detected at the level of the vertical vein that drained into the portal veins.

Familial total anomalous pulmonary venous return with 15q11.2 (BP1-BP2) microdeletion.

A 15q11.2 microdeletion (BP1-BP2) is associated with congenital heart diseases (CHDs), developmental delay, and epilepsy. This deletion co-occurs with CHD in 20-30% patients, but a familial case of CHD and a 15q11.

Nemaline myopathy with dilated cardiomyopathy in childhood.

We present a case of a 9-year-old boy with nemaline myopathy and dilated cardiomyopathy. The combination of nemaline myopathy and cardiomyopathy is rare, and this is the first reported case of dilated cardiomyopathy associated with childhood-onset nemaline myopathy. A novel mutation, p.

Single-lobe lung transplantation for rapidly deteriorating pulmonary venoocclusive disease.

Pulmonary venoocclusive disease, classified as a subgroup of pulmonary arterial hypertension, is known to show poor prognosis and lung transplantation is the only possible treatment. Single living-donor lobar lung transplantation is a unique method of treatment, mostly for small children, and size matching is the most important factor to conduct single living-donor lobar lung transplantation safely. We report a successful single living-donor lobar lung transplantation for a 6-year-old girl with pulmonary venoocclusive disease who received the graft from her mother.

Device closure of atrial septal defect: immediate and mid-term results.

Background: Transcatheter closure of atrial septal defects (ASDs) has become an alternative to open surgical procedures. The Amplatzer septal occluder (ASO) has been approved since 2005 in Japan, but there are still many concerns about adverse events, and information about outcomes and complications is limited. The objective of this study was to assess the immediate and mid-term outcomes of device closure of secundum ASDs.

Intrapericardial and retrocardial implantation of implantable cardioverter-defibrillator lead in a child with type 3 long QT syndrome.

A 6-year-old girl with type 3 long QT syndrome was safely and successfully implanted with an implantable cardioverter-defibrillator (ICD) system. Prior to implantation, she had experienced uncontrollable life-threatening arrhythmia in spite of high-dose administration of mexiletine. An ICD coil lead for transvenous use was placed in the intrapericardial and retrocardial space and was connected to a generator placed in front of the posterior sheath of the right abdominal rectal muscle.

A symptomatic neonate with tetralogy of Fallot, an absent pulmonary valve, and a single coronary artery with a major fistula.

We present a case with a rare combination of tetralogy of Fallot with an absent pulmonary valve, and a single coronary artery with a major fistula to the main pulmonary artery. Myocardial ischemia developed in response to increasing shunt flow through the coronary fistula, resulting in heart failure. We ligated the coronary fistula and plicated the anterior wall of the dilated pulmonary arteries during the neonatal period.

Early diagnosis of Kawasaki disease in patients with cervical lymphadenopathy.

Background: Among typical patients with Kawasaki disease (KD), a few KD patients present with only fever and cervical lymphadenopathy at admission (KDL). These patients have a significant risk for misdiagnosis, delay in treatment for KD, and development of coronary artery abnormalities. Therefore, the development of an easy tool for early diagnosis in these patients is desirable.

Four cases of Kawasaki syndrome complicated with myocarditis.

Background: Myocarditis frequently occurs in the acute phase of Kawasaki syndrome (KS), and a few severe cases have been reported. Four cases of myocarditis in KS required additional catecholamine treatment because of severe left ventricular dysfunction (LVD).

Case Reports: Three cases were relatively older children and 2 cases were complicated with encephalopathy.

Interval representative of transmural dispersion of repolarization in children and young adolescents with congenital long QT syndrome.

Background: It has been shown experimentally that the interval from the nadir of the initial negative T wave to the end of the T wave is representative of transmural dispersion of repolarization (TDR) when complex T waves are present. In the clinical setting, however, the interval representative of TDR in patients with long QT syndrome (LQTS) is a controversial subject.

Methods And Results: Five symptomatic patients (3 boys, 2 girls; 3 LQT1, 2 LQT2) were evaluated by a face immersion test before and after treatment to compare the configuration of the T wave.

Heart rate variability and ambulatory blood pressure monitoring in young patients with hypertrophic cardiomyopathy.

Background: Sudden cardiac death commonly occurs in young patients with hypertrophic cardiomyopathy (HCM); however, their heart rate variability (HRV) and blood pressure (BP) response to daily life activities is not well known.

Methods And Results: HRV and ambulatory BP monitoring were performed in 20 patients (age range: 7-21 years) and 57 age-matched healthy volunteers (age range: 10-22 years). Time domain variables and spectral data were obtained at hourly intervals throughout the day.