Pericardial tamponade due to erosion of a Figulla Flex II device after closure of an atrial septal defect.

We present a case of an 8-year-old boy suffering from sudden-onset severe chest pain and cardiogenic shock due to cardiac tamponade caused by erosion of a Figulla Flex II device. His symptoms developed 4 days after transcatheter closure of an atrial septal defect. After emergent pericardiocentesis, surgery was performed to remove the device, close the atrial septal defect, and repair the laceration in the anterior-superior wall of the right atrium and the perforation on the aortic wall adjacent to the right atrial free wall perforation site.

Risk factors of non-sustained ventricular tachycardia by technetium-perfusion imaging in patients with coronary artery lesions caused by Kawasaki disease.

Background: Sudden death can occur in some patients with non-sustained ventricular tachycardia (NSVT) after myocardial infarction (MI) in those with coronary artery lesions (CAL) caused by Kawasaki disease (KD). The aim of this study was to determine the risk factors for NSVT in the late period after KD by technetium-99m-tetrofosmin myocardial perfusion imaging (MPI).

Methods: We retrospectively analyzed the relation between the appearance of NSVT and the findings in MPI single-photon-emission computed tomography (SPECT) in 75 patients (55 males and 20 females) who had had CAL caused by KD.

Clinical and Echocardiographic Impact of Tafazzin Variants on Dilated Cardiomyopathy Phenotype in Left Ventricular Non-Compaction Patients in Early Infancy.

Background: Left ventricular non-compaction (LVNC) is a cardiomyopathy morphologically characterized by 2-layered myocardium and numerous prominent trabeculations, and is often associated with dilated cardiomyopathy (DCM). Variants in the gene encoding tafazzin (TAZ) may change mitochondrial function and cause dysfunction of many organs, but they also contribute to the DCM phenotype in LVNC, and the clinical and echocardiographic features of children with this phenotype are poorly understood.

Methods and results: We enrolled 92 DCM phenotype LVNC patients and performed next-generation sequencing to identify the genetic etiology.

Left ventricular reverse remodeling with infantile dilated cardiomyopathy and pitfalls of carvedilol therapy.

Background: The left ventricular reverse remodeling (LVRR) in idiopathic dilated cardiomyopathy (DCM) and the treatment with carvedilol in infants with severe heart failure remain poorly understood.

Methods: We reviewed the medical records of 5 infants around 12 months old referred to our hospital with severe heart failure due to DCM. Increased left ventricular fractional shortening (LVFS) by more than 10% and the percent of normal of left ventricular end-diastolic dimension (%LVDd) less than 120% were defined as LVRR in this study.

Heterogeneity of Ventricular Sympathetic Nervous Activity is Associated with Clinically Relevant Ventricular Arrhythmia in Postoperative Patients with Tetralogy of Fallot.

This study aimed to clarify whether there is an association between ventricular sympathetic nervous activity (SNA) and clinically relevant ventricular arrhythmia (a run of ≥ 3 consecutive ventricular beats, RVA) in postoperative patients with tetralogy of Fallot (TOF). We performed a retrospective study in a national referral center of pediatric cardiology in Japan. Twenty-four postoperative TOF patients (13 males, median age 17 years) undergoing myocardial (123)I metaiodobenzylguanidine (MIBG) scintigraphy were included.

Efficacy and safety of percutaneous transluminal balloon dilation to prevent progression of banding site stenosis after bilateral pulmonary artery banding.

Objectives: To investigate the efficacy and safety of percutaneous transluminal balloon dilation (PTBD) for the treatment of bilateral pulmonary artery banding (bil-PAB) site stenosis.

Background: Although bil-PAB is an alternative initial treatment for high-risk neonates with hypoplastic left heart syndrome (HLHS) or critical aortic stenosis (cAS), those patients often suffer from desaturation because of progressive stenosis of the bil-PAB sites during the interstage period.

Methods: We retrospectively evaluated the efficacy and safety of 11 consecutive PTBD procedures performed between 2006 and 2012 to treat bil-PAB site stenosis in four high-risk infants (three females) with HLHS or cAS.

Evaluation of Valsalva sinus wall deformation due to compression by the Amplatzer septal occluder and the potential for erosion development.

Objectives: We studied whether the pressure exerted by the Amplatzer septal occluder (ASO) disk on the Valsalva sinus wall (VW) is a risk factor for erosion and attempted to determine the pressure that may trigger erosion.

Background: The causes and mechanisms of erosion after ASO placement remain unclear.

Methods: We reviewed 665 consecutive patients with atrial septal defects who underwent ASO closure at our hospital from 2005 to 2012.

Acute rupture of chordae tendineae of the mitral valve in infants: a nationwide survey in Japan exploring a new syndrome.

Background: Recently, infant cases of acute heart failure attributable to rupture of the mitral chordae tendineae have been reported. However, little is known about the pathogenesis and clinical course of this condition.

Methods And Results: Ninety-five children with rupture of mitral chordae tendineae were identified in nationwide surveys of Japan diagnosed from 1995 to 2013.

Clinical characteristics and long-term outcome of acute myocarditis in children.

The clinical course of acute myocarditis (AM) in children varies from being asymptomatic to causing sudden cardiac death. The aim of this study was to clarify the clinical characteristics and the long-term outcome of AM in children. We enrolled 24 children (aged from 0.

Prevalence and distribution of sarcomeric gene mutations in Japanese patients with familial hypertrophic cardiomyopathy.

Background: Hypertrophic cardiomyopathy (HCM), which is inherited as an autosomal dominant trait, is the most prevalent hereditary cardiac disease. Although there are several reports on the systematic screening of mutations in the disease-causing genes in European and American populations, only limited information is available for Asian populations, including Japanese.

Methods And Results: Genetic screening of disease-associated mutations in 8 genes for sarcomeric proteins, MYH7, MYBPC3, MYL2, MYL3, TNNT2, TNNI3, TPM1, and ACTC, was performed by direct sequencing in 112 unrelated Japanese proband patients with familial HCM; 37 different mutations, including 13 novel ones in 5 genes, MYH7, MYBPC3, TNNT2, TNNI3, and TPM1, were identified in 49 (43.

The 30-year outcome for patients after myocardial infarction due to coronary artery lesions caused by Kawasaki disease.

This study determined the long-term outcome for patients after myocardial infarction (MI) due to Kawasaki disease (KD). Retrospective analysis was performed for 60 patients who had experienced MI between 1976 and 2007. Their ages at the initial MI ranged from 3 months to 33 years (median, 2 years).

Acute coronary syndrome in adult patients with coronary artery lesions caused by Kawasaki disease: review of case reports.

Information about acute coronary syndrome caused by Kawasaki disease-related coronary artery lesions in adults is sketchy. We reviewed the clinical features of 50 adult patients who had an acute coronary syndrome caused by coronary artery lesions due to Kawasaki disease or probable Kawasaki disease from 1980 to 2008. Of the 50 patients, 43 (90%) were male and seven were female (10%).