Cardiac sympathetic denervation for catecholaminergic polymorphic ventricular tachycardia in the light of the medical situation in Japan.

Progress of treadmill exercise testing in Case 1 Each electrocardiogram shows the maximum load. Before left cardiac sympathetic denervation, polymorphic ventricular tachycardias were observed. After left cardiac sympathetic denervation, no ventricular arrhythmias were induced during exercise.

Utility of the Angle between the Right Aortic Arch and First Branch for Detecting Double Aortic Arch via Fetal Echocardiography.

Introduction: The presence of a double aortic arch (DAA) is manifested by compressive symptoms, requiring surgery. DAA cases are classified as either complete or incomplete type. DAA and a right aortic arch with mirror image branching (mRAA) have a similar configuration to the first branch artery.

Successful bailout stenting for critical aortic coarctation in a premature baby weighing 590 g.

Severe aortic coarctation (CoA) is a critical congenital heart disease that requires surgery as the first-line treatment in neonates. However, in very small premature infants, aortic arch repair has a relatively high mortality and morbidity rate. Bailout stenting is an alternative method that can be performed safely and effectively with low morbidity.

Utility of novel echocardiographic measurements to improve prenatal diagnosis of coarctation of the aorta.

Prenatal recognition of coarctation of the aorta (CoA) may improve neonatal survival and reduce morbidity. However, prenatal diagnosis of CoA remains challenging, with relatively high false-positive and false-negative rates. This study aimed to identify a novel formula based on fetal echocardiographic measures to predict prenatal identification of CoA.

Improvement in right ventricular function by mitral valve closure in hypoplastic left heart syndrome.

A male neonate presented with the aortic/mitral stenotic variant of hypoplastic left heart syndrome, wherein the suprasystemic left ventricular pressure and relatively large left ventricle had shifted the intraventricular septum. Despite bilateral pulmonary artery banding, the stroke volume was difficult to maintain owing to the compressed right ventricle, causing heart failure symptoms. Percutaneous balloon aortic valvuloplasty decreased the left ventricular pressure, restoring the right ventricular function.

Bidirectional coronary venous return in the partial unroofed coronary sinus syndrome complicated by coronary sinus orifice atresia.

Unroofed coronary sinus syndrome complicated by coronary sinus orifice atresia is a rare congenital anomaly. There are two alternate exits for coronary venous return: unroofed coronary sinus and persistent left superior caval vein. The coronary venous direction could be bidirectional depending on the pressure balance between the left atrium and the systemic vein.

Transcatheter closure of perimembranous ventricular septal defects with Amplatzer® duct occluder I; The first case report in Japan.

We report the first case of transcatheter perimembranous ventricular septal defect (pmVSD) closure in Japan where none of existing devices for VSD closure has been approved. The pmVSD was successfully closed with first generation Amplatzer® duct occluder (ADO-I; St Jude Medical, St Paul, MN, USA). The procedure was performed under general anesthesia with transesophageal echocardiographic and fluoroscopic guidance.

Efficacy of bubble contrast echocardiography in detecting pulmonary arteriovenous fistulas in children with univentricular heart after total cavopulmonary connection.

Background: Development of pulmonary arteriovenous fistulas in patients with cavopulmonary anastomosis may result in a significant morbidity. Although the use of bubble contrast echocardiography with selective injection into both the branch pulmonary arteries in identifying pulmonary arteriovenous fistulas has been increasing, the actual efficacy of this diagnostic modality has not been properly evaluated. Thus, this study aimed to assess the efficacy of bubble contrast echocardiography in detecting pulmonary arteriovenous fistulas in children with total cavopulmonary connection.

Successful everolimus-eluting stent implantation into the left main trunk stenosis in the anomalous coronary artery after neo-aortic valve replacement in a 6-year-old boy.

CHDs occasionally have coronary complications; however, stent implantation is technically difficult in small children. We reported a successful drug-eluting stent implantation into the congenital anomalous coronary artery in a 6-year-old boy. This treatment is useful for rescuing coronary stenosis, and dual antiplatelet therapy is important to prevent stent restenosis.

Percutaneous pulmonary debanding for an infant complicated by spontaneously closing muscular ventricular septal defect: A case report and in vitro study.

Pulmonary artery banding (PAB) is a standard operation for various congenital heart defects complicated by pulmonary hypertension (PH) and judged unsuitable for primary intracardiac repair. We report successful percutaneous pulmonary artery debanding in a baby complicated by muscular ventricular septal defect (VSD), that was initially large and multiple, but closed spontaneously later. The 5-month-old boy was referred to our hospital on day 3, diagnosed as having aortic coarctation (CoA), with multiple muscular VSDs and severe PH.

Static balloon atrial septostomy for the purpose of left heart intervention in postoperative adult CHD.

Unlabelled: Background and purposeStatic balloon atrial septostomy is a widely accepted intervention for children with CHD. Successful surgical palliation is creating increasing numbers of adult CHD patients who need subsequent left heart intervention requiring transseptal access. In these patients, the interatrial septum is usually thick and fibrotic because of a previous open heart surgery or catheter intervention, and conventional transseptal puncture may be unsuccessful.

Successful simultaneous transcatheter treatment for a secundum atrial septal defect complicated by valvular pulmonary stenosis in an infant.

Only few reports have described successful simultaneous transcatheter intervention for CHD in infants. We present an infant with secundum atrial septal defect complicated by valvular pulmonary stenosis. Percutaneous transcatheter pulmonary valvuloplasty was performed first, followed by transcatheter closure of the secundum atrial septal defect uneventfully.

Tissue Motion Annular Displacement of the Mitral Valve Can Be a Useful Index for the Evaluation of Left Ventricular Systolic Function by Echocardiography in Normal Children.

As the important role of longitudinal shortening in ventricular function has been well recognized over the past decade, evaluation of longitudinal systolic function of the left ventricle has become a subject of growing interest. Tissue motion annular displacement of the mitral valve (TMAD) is a new parameter of longitudinal systolic function. Although some studies have reported that this new parameter correlates with left ventricular ejection fraction (LVEF) in adults, little is known about TMAD in normal children.

Successful replacement of the systemic tricuspid valve with a mechanical valve in a 3-month-old boy with congenitally corrected transposition of the great arteries having a dysplastic tricuspid valve.

There are a few reports of successful replacement of the left-sided systemic tricuspid valve with a mechanical valve in small infants with congenitally corrected transposition of the great arteries having Ebstein's anomaly. Tricuspid valve replacement is the preferred option when pulmonary artery banding, performed as a prelude to performing the double-switch operation, is not feasible because of severe heart failure caused by tricuspid regurgitation.

Tolerance to ischemia reperfusion injury in a congenital heart disease model.

Background: Open heart surgery-associated ischemia/reperfusion (I/R) injury affects postoperative outcome, and a leading cause of this is lipid peroxidation. Congenital heart disease (CHD) patients, however, are less sensitive to I/R injury. Although little is known about the underlying molecular mechanisms, CHD-associated hypoxia alters the polyunsaturated fatty acid (PUFA) composition of membranes, which are the preferential targets for reactive oxygen species (ROS) generated during I/R.

Asymptomatic spinal arteriovenous fistula presenting only as continuous murmur.

Spinal arteriovenous fistula is extremely rare in children. Weakness and sensory disturbance in the lower extremities are the specific clinical presentations. Children, however, commonly have no subjective symptoms; in rare cases, a continuous murmur is the only physical finding.

Combined Alport syndrome and Klinefelter syndrome.

To date, there have been a very limited number of case reports on combined Alport syndrome (AS) and Klinefelter syndrome (KS). We herein describe the case of a 9-month-old boy diagnosed with concomitant AS and KS. KS was detected on chromosomal analysis of the amniotic fluid, and hematuria/proteinuria was identified in urinary screening at 6 months of age.

Combination of double aortic arch and interruption of aortic arch in pulmonary atresia with ventricular septal defect.

No reports on pulmonary atresia with ventricular septal defect with the combination of double aortic arch and interruption between left and right carotid arteries have been published so far.

Acute renal failure after high-dose methotrexate therapy in a patient with ileostomy.

High-dose methotrexate (HD-MTX) is an important treatment for Burkitt lymphoma, but can cause hepatic and renal toxicity when its clearance is delayed. We report a case of acute renal failure after HD-MTX therapy in a patient with ileostomy, The patient was a 3-year-old boy who had received a living-related liver transplantation for congenital biliary atresia. At day 833 after the transplantation, he was diagnosed with PTLD (post-transplantation lymphoproliferative disorder, Burkitt-type malignant lymphoma).