Unguarded Tricuspid Valve and Pulmonary Atresia With Intact Ventricular Septum Complicated With Right Coronary Artery Fistula and Advanced Atrioventricular Block in a Fetus: A Case Report.

The unguarded tricuspid valve is a rare and severe condition. When found in the fetus, they mostly undergo abortion or intrauterine death. The details of the fetal course in such cases are poorly understood.

Efficacy of SubcutAneous implantable cardioVErter-defibrillators in ≤18 year-old CHILDREN: SAVE-CHILDREN registry.

Background: In adult patients, subcutaneous implantable cardioverter defibrillators (S-ICDs) have been reported to be non-inferior to transvenous ICDs with respect to the incidence of device-related complications and inappropriate shocks. Only a few reports have investigated the efficacy of S-ICDs in the pediatric field. This study aimed to investigate the utility and safety of S-ICDs in patients ≤18 years old.

Long-term results of percutaneous transluminal coronary balloon angioplasty in Kawasaki disease.

We report the long-term results of percutaneous transluminal coronary balloon angioplasty for early appearing localised stenosis of the left anterior descending artery caused by Kawasaki disease in two 2-year-olds. One patient had 50% restenosis and newly appearing aneurysm at 21-year-old, and the other had no restenosis at 16-year-old. They had no symptoms or cardiac events for 20 years.

Static balloon atrial septostomy in Japan in shortage of standard balloon septostomy catheter.

Background: The supply of Rashkind balloon atrial septostomy (BAS) catheters (Medtronic, Minneapolis, MN, USA) has suddenly been discontinued in the world due to its recall. Consequently, Japan fell into a critical shortage of standard BAS catheters. Although the use of static BAS is off-label in Japan, its importance is increasing in such a situation.

Study design for control of HEART rate in inFant and child tachyarrhythmia with heart failure Using Landiolol (HEARTFUL): A prospective, multicenter, uncontrolled clinical trial.

Background: Persistent tachycardia in pediatric patients after congenital heart surgery further deteriorates their hemodynamic condition, and may become fatal. Therefore, immediate control of the tachycardia is mandatory in these patients. For this purpose, quick-acting, short-acting, titratable intravenous agents are required.

An international multicenter study comparing arrhythmia prevalence between the intracardiac lateral tunnel and the extracardiac conduit type of Fontan operations.

Objective: The study objective was to determine whether the extracardiac conduit Fontan confers an arrhythmia advantage over the intracardiac lateral tunnel Fontan.

Methods: This multicenter study of 1271 patients compared bradyarrhythmia (defined as need for pacing) and tachyarrhythmia (defined as needing antiarrhythmic therapy) between 602 patients undergoing the intracardiac Fontan and 669 patients undergoing the extracardiac Fontan. The median age at the time of the Fontan procedure was 2.

[Revascularization surgery for isolated unilateral absence of the right pulmonary artery].

We describe a baby girl with isolate absence of the right pulmonary artery. She had tachypnea just after birth. Several examinations showed absence of the right pulmonary artery and an aortopulmonary collateral artery.

Plastic bronchitis developing 5 years after Fontan procedure in a girl with Kartagener's syndrome.

Although rare, plastic bronchitis (PB) is an important early complication after Fontan procedure. Kartagener's syndrome is characterized by mucociliary dysfunction of the respiratory tract and has a triad of features, including situs inversus totalis, chronic sinusitis, and bronchiectasia. We experienced PB in a patient with Kartagener's syndrome 5 years after Fontan procedure.

Clinical features of adult patients with Eisenmenger's syndrome in Japan and Korea.

Background: There are few articles on mortality and morbidity of adult patients with Eisenmenger's syndrome (ES) in the current era when disease targeting therapy (DTT) has been available.

Methods And Results: 198 patients (a median age 35 years, 64% female) with ES who visited the 16 participating institutes in Japan and Korea from 1998 to 2009 were enrolled. Clinical data during adulthood were collected from each institutional chart and analyzed centrally.

[Norwood procedure with swing-back technique for double-inlet left ventricle, transposition of great arteries, coarctation of aorta, subaortic stenosis].

Patient is 1 month old, 3.72 kg boy, he was diagnosed as {S, L, L} double-inlet left ventricle (DILV), transposition of the great arteries, coarctation of aorta, rudimentary right ventricle. Patient was undergone Norwood procedure, with "swing-back technique"; end-to end anastomosis of ascending aorta with descending aorta, double-barreled style Damus-Kaye-Stansel (DKS), end-to side anastomosis of neoaorta to aortic arch.

Coil occlusion of PDA in patients younger than 1 year: risk factors for adverse events.

Background: Transcatheter occlusion of infantile patent ductus arteriosus (PDA) challenges the interventionist.

Purpose: To analyze the risk factors for adverse events from this procedure in patients younger than 12 months.

Subjects: We retrospectively analyzed data on 32 patients younger than 12 months in whom transcatheter coil occlusion of a PDA was attempted.

Increased serum high-sensitivity C-reactive protein is related to hypoxia and brain natriuretic peptide in congenital heart disease.

Background: Studies of adults have shown a direct association between increased serum concentrations of high-sensitivity C-reactive protein (hs-CRP) and atherosclerotic cardiovascular disease, diabetes, and chronic heart failure. Some studies have documented elevated hs-CRP in obese children and adolescents, and in patients with a history of Kawasaki disease, but there are few data on its clinical significance in congenital heart disease.

Methods: Measurements of hs-CRP, brain natriuretic peptide (BNP), hemoglobin, and percutaneous oxygen saturation (SpO2) were done in the following 70 patients: 18 controls; 11 with hypoxia (SpO2 /= 40 pg/mL and SpO2 > 85%); and 10 patients with hypoxia-BNP (SpO2 /= 40 pg/mL).

Genesis stent implantation without using a long sheath in two children.

A Genesis stent was implanted in two children, one with superior vena caval (SVC) stenosis and one with pulmonary artery branch stenosis. Case 1 was a 2-month-old baby with SVC stenosis following intracardiac repair for total anomalous pulmonary venous connection (TAPVC) and case 2 was a 2-year-old child with left lower pulmonary artery stenosis following one-stage unifocalization for dextrocardia, double outlet right ventricle, ventricular septal defect, pulmonary atresia and major aortopulmonary collateral arteries. Both procedures resulted in immediate clinical and hemodynamic improvement.

Possible role of adrenomedullin in the regulation of Fontan circulation: mature form of plasma adrenomedullin is extracted in the lung in patients with Fontan procedure.

Objective: We investigated the pathophysiological significance of molecular forms of adrenomedullin (AM) in patients after the Fontan procedure.

Methods: Plasma concentrations of mature AM (AM-m), an active form, glycine-extended AM (AM-Gly), an inactive form, and total AM (AM-T: AM-m+AM-Gly) were measured by specific immunoradiometric assay in the femoral vein, pulmonary artery and femoral artery of 29 consecutive patients after the Fontan procedure. The eleven patients who had history of Kawasaki disease and have normal coronary and hemodynamics served as control.

Development of a reexpandable covered stent for children.

The use of covered stents in children is limited by the need for a large long sheath for delivery and the impossibility of redilation once implanted. The authors developed a reexpandable covered stent implantable in children through a small sheath and evaluated its clinical feasibility in mini piglets. An original Palmaz stent was covered with a polyurethane membrane that could be stretched up to 700%.

Percutaneous transluminal coronary rotational atherectomy for localized stenosis caused by Kawasaki disease.

We report the results of percutaneous transluminal coronary rotational atherectomy (PTCRA) for localized stenosis caused by Kawasaki disease (KD) in children. Five males and a females, aged 5-15 years old (median, 9), underwent PTCRA. The interval from the onset of KD to PTCRA ranged from 5 to 12 years (median, 9).

Coil occlusion of patent ductus arteriosus.

Background: Coils are the only devices available for transcatheter occlusion of patent ductus arteriosus (PDA) in Japan. Since April 1999, we have introduced a 0.052-inch Gianturco coil (0.

Coil occlusion for patent ductus arteriosus larger than 3 mm.

Background: Coil occlusion of patent ductus arteriosus (PDA) is now widely accepted as the first-line treatment, but there are few reports of age-dependent differences in the complications associated with this technique.

Methods And Results: Sixteen patients (11 adults, 5 children) with a PDA larger than 3 mm, who underwent coil occlusion at Sapporo Medical University Hospital between September 1995 and August 2004, were enrolled. Immediate and intermediate outcomes and complications were analyzed.

Relapse of T-cell all after stem cell transplant presenting as hypertrophic cardiomyopathy: the value of non-invasive diagnostic imaging in detecting cardiac leukemia.

We describe a 14-year-old female with acute lymphoblastic leukemia (ALL) with a mediastinal mass at diagnosis who developed hypertrophic cardiomyopathy (HC) after stem cell transplantation (SCT). During refractory relapse after SCT using bone marrow from her HLA-matched sibling, she underwent whole thorax irradiation because of pleural effusion and a recurrent mediastinal mass. After a second SCT using peripheral blood from the same donor, she developed HC suspected to be related to tacrolimus.

Late distortion of the original Palmaz stent implanted in postoperative lesions associated with congenital heart disease.

The objective of this study was to report late distortion of a Palmaz stent. Late distortion of an original Palmaz stent, implanted in an extracardiac lesion, is rare. We completed a 1-year follow-up of 54 patients who had been implanted with 80 Palmaz stents in extracardiac lesions.

Usefulness of electron beam computed tomography for quantitative estimation of myocardial ischemia in patients.

Background: The electron beam computed tomography (EBT) was used as non-invasive methods for estimating mycardial characteristics in patients after onset of Kawasaki disease.

Methods: All of the 22 coronary branches with localized stenosis (LS), and 22 branches without coronary artery lesion after Kawasaki disease were examined for changes of myocardial CT number. In this study, each myocardial region was divided into three parts (endocardial, central, epicardial parts) and the myocardium/lumen (M/L) ratio was calculated.

The throat flora and its mitogenic activity in patients with Kawasaki disease.

The etiology of Kawasaki disease (KD) remains unknown, although some infectious organism has been suggested as the cause. Recent studies suggest that some bacterial toxins with superantigen activity are involved in its pathogenesis, but no specific bacterial toxin has yet been identified. Throat swabs for bacterial culture were obtained from 21 patients with KD and 20 with other febrile illnesses as controls.

Pulse wave velocity and ankle brachial index in patients with Kawasaki disease.

Background: Pulse wave velocity (PWV) and ankle brachial index (ABI) are a useful method to estimate atherosclerosis in adults, while a history of Kawasaki disease (KD) may be a risk factor for later atherosclerosis of systemic arteries. PWV and ABI in patients with a history of KD have not been reported.

Method: In total, 90 patients ranging in age from 4 to 25 years who had a history of KD previously, any time from 1 month to 19 years ago, were studied.