Staged Norwood operation for patients with hypoplastic left heart syndrome and cor triatriatum: a report of two cases.

Two patients with hypoplastic left heart syndrome (HLHS) concomitant with cor triatriatum underwent the staged Norwood procedure following hybrid palliation. Cor triatriatum was diagnosed after birth in both cases. Case 1 with aortic stenosis and mitral atresia underwent bilateral pulmonary artery banding at 2 days of age.

An Extremely Preterm Infant Born at 23 Weeks' Gestation With an Interrupted Aortic Arch Complex: A Case Report.

We present a case of an infant male born at 23 weeks' gestation with an interrupted aortic arch (IAA) complex. We treated the patient with hypoxic gas ventilation to address developing systemic undercirculation in the acute postnatal phase. As the symptoms of bronchopulmonary dysplasia evolved, hypoxic gas ventilation was no longer required to stabilize the hemodynamics.

Paradoxical Continuous Left-to-Right Ductal Shunt during Circulatory Collapse due to Ductal Closure in an Infant with Duct-Dependent Systemic Circulation.

Duct-dependent systemic circulation is accompanied by a right-to-left ductal shunt, at least during systole. Although observations of paradoxical continuous left-to-right shunts in duct-dependent systemic circulation have been reported, the mechanism remains unclear. We report a continuous left-to-right ductal shunt throughout the cardiac cycle during the initial recovery phase from circulatory collapse and right ventricular (RV) dysfunction due to ductal closure in an infant with hypoplastic left heart and severe aortic coarctation.

Efficacy of a phosphodiesterase type 5 inhibitor on the pulmonary artery index and postoperative hemodynamics after a Fontan operation.

There have been no reports regarding the influence of a change in the pulmonary artery index before or after a Fontan operation. Moreover, the factors that might affect the change in the pulmonary artery index are unclear. This was a retrospective study, which included 94 patients who underwent a Fontan operation.

Efficacy of a Subcutaneous Implantable Cardioverter Defibrillator in a Child with Early Repolarization Syndrome.

An early repolarization (ER) pattern or J waves are considered to be a benign finding observed in the healthy population, however, it has been pointed out that the ER pattern seen in the inferolateral leads could be an independent risk factor for fatal arrhythmias. We present a pediatric case in which early repolarization syndrome (ERS) was suspected due to the presence of ER or J waves in the inferior leads, which eventually disappeared after the administration of pilsicainide. During the follow-up period, several fatal ventricular arrhythmias were recorded after implantation of a subcutaneous implantable cardiac defibrillator (S-ICD).

Successful Percutaneous Thrombectomy of the Left Pulmonary Artery in a Neonate After a Patent Ductus Arteriosus Clipping.

We report a neonate with a successful percutaneous thrombectomy of a total thrombotic occlusion of the left pulmonary artery (LPA) after a surgical clipping for a patent ductus arteriosus (PDA). We suspected the compression of the LPA by the clipping and postoperative hemodynamic instability caused the LPA obstruction. After the surgical removal of the PDA clip and division of the PDA, we could safely retrieve the LPA thrombus with a non-hydrodynamic thrombectomy catheter for coronary arteries.

Efficacy and safety of tolvaptan after pediatric congenital heart disease surgery.

It is not clear whether tolvaptan is safe and effective irrespective of various underlying clinical conditions including the functional ventricle morphology, chromosomal abnormalities, and renal function after complex pediatric congenital heart disease surgery. Also, the appropriate dose of tolvaptan in these patients has not been previously identified. We retrospectively assessed the urine volume, body weight, patient clinical characteristics, laboratory data, and vital signs before and on days 1 and 7 of the tolvaptan administration after congenital heart disease surgery.

Late recovery of the cardiopulmonary exercise capacity after transcatheter amplatzer device closures for atrial septal defects in adults.

Transcatheter atrial septal defect (ASD) closures using an Amplatzer Septal Occluder (ASO) have been widely performed. Compared to children, we sometimes experience late recovery of exercise performance in adult patients. Our study aimed to evaluate the change in the cardiopulmonary exercise capacity in asymptomatic or mildly symptomatic adult patients after a transcatheter ASD closure using an ASO.

Relapsing 6q24-related transient neonatal diabetes mellitus with insulin resistance: A case report.

The overexpression of imprinted genes on chromosome 6q24 causes 6q24-related transient neonatal diabetes mellitus (6q24-TNDM). Most cases of 6q24-TNDM show transient diabetes mellitus (DM) during the neonatal period, followed by relapse after puberty. These two courses of DM are both characterized by insulin insufficiency.

Hepatocyte growth factor predicts failure of Fontan circulation.

Aims: This study aimed to assess the value of the hepatocyte growth factor (HGF) as an independent predictor of a Fontan circulation failure.

Methods And Results: This retrospective case-control study included 34 consecutive patients (19 men and 15 women) who underwent a post-operative cardiac catheterization after a Fontan operation at the Saitama Medical University International Medical Center between April 2017 and December 2019. We divided the patients into two groups according to the HGF level: HGF < 0.

Comparison of transcatheter patent ductus arteriosus closure between children and adults.

The data comparing the characteristics and effect of transcatheter patent ductus arteriosus (PDA) closure between children and adults is scarce. We analyzed 54 consecutive patients who underwent transcatheter PDA closures. We divided the patients into 2 groups of < 18 years and ≥ 18 years and compared the hemodynamic changes before and after the PDA closure.

Predictors of Early Postoperative Supraventricular Tachyarrhythmias in Children After the Fontan Procedure.

Postoperative arrhythmias are a frequent and fatal complication after the Fontan operation. However, clinical evidence demonstrating early postoperative arrhythmias in children undergoing the Fontan operation is limited. This study aimed to evaluate the prevalence of arrhythmias and identify the predictors of early postoperative supraventricular tachyarrhythmias (SVTs) after the Fontan procedure.

Early assessment of cardiac troponin I predicts the postoperative cardiac status and clinical course after congenital heart disease surgery.

Cardiac troponin I (cTnI) is a regulatory protein with a high sensitivity and specificity for cardiac injury. Preoperative and postoperative elevations of cTnI are usually considered predictors of the mortality and morbidity. However, little is known about the relationship between the cTnI and postoperative course after the congenital heart disease (CHD) operation.

Usefulness of Red Blood Cell Distribution Width in the Assessment of Hemodynamics After Tetralogy of Fallot Repair.

Background: There are no reports on the effect of red blood cell distribution width (RDW) in surgical repair of tetralogy of Fallot (ToF).

Methods and results: A total of 50 patients who underwent cardiac catheterization after surgical repair of ToF were retrospectively assessed. RDW was positively correlated with the ratio of right ventricular pressure to left ventricular pressure (RVP/LVP; P<0.

Successful radiofrequency catheter ablation of life-threatening atrial tachycardia in an infant with asplenia syndrome.

A 1-year-old infant with asplenia syndrome and congenital heart disease consisting of common atrium, common inlet left ventricle with a common atrio-ventricular (AV) valve, pulmonary atresia, and total anomalous pulmonary venous connection was admitted to our hospital for radiofrequency catheter ablation (RFCA) of supraventricular tachycardia (SVT) before total cavo-pulmonary connection. After antiarrhythmic medications were discontinued for RFCA, she suffered from SVT that resulted in the rapid deterioration of hemodynamic status. Antiarrhythmic medications and cardioversion were not effective in terminating SVT.

The necessity of implantable cardioverter defibrillators in patients with Kearns-Sayre syndrome - systematic review of the articles.

The most common cardiac feature of Kearns-Sayre syndrome (KSS) is atrioventricular block (AVB), and pacemaker implantations (PMIs) are recommended for KSS patients with advanced AVB. However, some KSS patients develop fatal arrhythmias such as polymorphic ventricular tachycardia (PMVT) and ventricular fibrillation (VF) and die suddenly even after PMIs. We report a patient with KSS who developed PMVT, VF, and QT prolongation, and was treated with mexiletine and successfully managed with an implantable cardioverter defibrillator (ICD).

Efficacy of catheter interventions in the early and very early postoperative period after CHD operation.

Background: Catheter interventions for residual lesions in the early postoperative period after CHD operations are still not established as a reliable treatment option.

Methods: We retrospectively reviewed our institutional experience of cardiac catheterisations and catheter interventions performed in the early postoperative period. We classified our patients into two groups.

Pathogenesis and Bone Resorption in Acquired Cholesteatoma: Current Knowledge and Future Prospectives.

Cholesteatoma is a cystic non tumorous lesion of the temporal bone that has the ability to destroy nearby structures by its power to cause bone resorption and as a result, fatal complications prevail. We aimed to conduct a comprehensive review for pathogenesis of acquired cholesteatoma, bone resorption mechanisms, and offer a future vision of this serious disease. We have reviewed different theories for pathogenesis of acquired cholesteatoma including the most relevant and updated ones with special emphasis on the mechanisms of bone resorption through Medline/PubMed research using the keywords 'aetiopathogenesis, bone resorption, acquired cholesteatoma, temporal bone, and cytokines.

Successful emergent coil embolization of an inferior epigastric artery perforation in a neonate.

A 28-day-old neonate with a postoperative ventricular septal defect and coarctation of aorta suffered from a right inferior epigastric artery perforation at the time of a central venous catheter placement. It resulted in a rapid and extreme hemoglobin decrease and decrease in the systolic blood pressure. The contrast computed tomography scan revealed a large amount of retroperitoneal hemorrhaging and a hematoma.

Volumes of Velopharyngeal and Glossopharyngeal Airway Were Not Changed after Uvulopalatopharyngoplasty: Report of Three Cases.

Objective. The aim of this study was to investigate the changes in velopharyngeal and glossopharyngeal airway morphology and volume after uvulopalatopharyngoplasty in three adult obstructive sleep apnea syndrome patients who had bilateral large tonsils using three-dimensional computed tomography. Case Report.

Usefulness of the Red Blood Cell Distribution Width to Predict Heart Failure in Patients With a Fontan Circulation.

The red blood cell distribution width (RDW) is a quantitative measure of the variability in the size of circulating erythrocytes. We aimed to study whether higher levels of the RDW were associated with heart failure in a Fontan circulation and to analyze its clinical value compared to brain natriuretic peptide. This retrospective study included 38 consecutive pediatric patients with a Fontan circulation who underwent routine cardiac catheterization at the International Medical Center, Saitama Medical University from October 2010 to July 2014.

Aortic root dilatation and aortic stiffness in patients with single ventricular circulation.

Background: This study tested the hypothesis that aortic stiffness is increased more in patients with single ventricular (SiV) circulation and dilated aorta than in those without aortic dilatation, and that aortic stiffness is an independent determinant of aortic dilatation in this type of patient.

Methods And Results: Pulse wave velocity (PWV) and aortic size were measured during catheterization in 56 consecutive patients with SiV circulation (aortic dilatation, n=31 [observed/expected aortic root diameter >1.5]; without aortic dilatation, n=25).