Portosystemic shunt with hyperammonemia and high cardiac output as a complication after Fontan surgery.

In the late phase after Fontan surgery, organ dysfunction due to high central venous pressure (CVP) is a major clinical problem. We have described the cases of two patients with portosystemic shunts who exhibited hyperammonemia and high cardiac output associated with peripheral vasodilatation after Fontan surgery. A high CVP in these patients may have resulted in the formation of a portosystemic shunt.

Dynamic QT response to cold-water face immersion in long-QT syndrome type 3.

Background: Abnormal dynamics of QT intervals in response to sympathetic nervous system stimulation are used to diagnose long-QT syndrome (LQTS). We hypothesized that parasympathetic stimulation with cold-water face immersion following exercise would influence QT dynamics in patients with LQTS type 3 (LQT3).

Methods: Study participants (n = 42; mean age = 11.

A novel homozygous missense mutation in three CACT-deficient patients: clinical and autopsy data.

Carnitine-acylcarnitine translocase (CACT) deficiency is a fatty acid ß-oxidation disorder of the carnitine shuttle in mitochondria, with a high mortality rate in childhood. We evaluated three patients, including two siblings, with neonatal-onset CACT deficiency and revealed identical homozygous missense mutations of p.Arg275Gln within the gene.

A novel nonsense mutation in a patient with intractable epilepsy and cardiac malformation.

Cornelia de Lange syndrome (CdLS) is a cohesinopathy caused by genetic variations. We present a female with -associated CdLS with a novel truncation mutation (p. Arg499Ter), transposition of the great arteries, and periodic intractable seizures from 40 months of age.

Elimination of arrhythmogenesis after subtotal resection of congenital cardiac fibroma: a case report.

Subtotal tumour resection is used to treat infants with congenital cardiac fibroma and medication-resistant ventricular arrhythmias; however, complete elimination of arrhythmogenic substrates has been unclear. A 4-month-old male infant with congenital cardiac fibroma and ventricular fibrillation underwent subtotal tumour resection and implantable cardioverter-defibrillator implantation. Five years later, angiography revealed impending compression of the left coronary artery.

Efficacy of primary treatment with immunoglobulin plus ciclosporin for prevention of coronary artery abnormalities in patients with Kawasaki disease predicted to be at increased risk of non-response to intravenous immunoglobulin (KAICA): a randomised controlled, open-label, blinded-endpoints, phase 3 trial.

Background: Genetic studies have indicated possible involvement of the upregulated calcium-nuclear factor of activated T cells pathway in the pathogenesis of Kawasaki disease. We aimed to assess safety and efficacy of ciclosporin, an immunosuppressant targeting this pathway, for protection of patients with Kawasaki disease against coronary artery abnormalities.

Methods: We did a randomised, open-label, blinded endpoints trial involving 22 hospitals in Japan between May 29, 2014, and Dec 27, 2016.

Extracorporeal membrane oxygenation in a low-weight infant after cardiac surgery.

A neonate was diagnosed with ventricular septal defect and aortic coarctation. Bilateral pulmonary artery banding was performed at 9 days of age (weight 1472 g). Because of ductal shock, emergency extracorporeal membrane oxygenation was initiated after surgery.

Group A streptococcus endocarditis in children: 2 cases and a review of the literature.

Background: Infective endocarditis (IE) is defined as endocarditis caused by microorganisms (bacteria or fungi) involving either the heart or great vessels. The clinical course of IE can be complicated by cardiac dysfunction and bacterial embolization to virtually any organ. Staphylococcus aureus and viridans group streptococci are the most common causative organisms, whereas group A Streptococcus (GAS) is less common.

Elimination of arrhythmogenesis after subtotal resection of congenital cardiac fibroma: a case report.

Subtotal tumour resection is used to treat infants with congenital cardiac fibroma and medication-resistant ventricular arrhythmias; however, complete elimination of arrhythmogenic substrates has been unclear. A 4-month-old male infant with congenital cardiac fibroma and ventricular fibrillation underwent subtotal tumour resection and implantable cardioverter-defibrillator implantation. Five years later, angiography revealed impending compression of the left coronary artery.

Anatomical correction of atrioventricular discordance using three-dimensional replica.

Surgical experience with {S,L,D} segmental anatomy of atrioventricular discordance with double-outlet right ventricle is extremely rare. In addition to ordinary cardiac examination, we reviewed electrophysiological studies and a three-dimensional cardiac replica (crossMedical, Inc., Kyoto, Japan).

Growing potential of small aortic valve with aortic coarctation or interrupted aortic arch after bilateral pulmonary artery banding.

Objectives: Bilateral pulmonary artery banding (bPAB) is utilized for some patients with a ventricular septal defect (VSD) and aortic coarctation (CoA) or interrupted aortic arch (IAA). We evaluated aortic valve (AoV) diameter and patient outcomes following bPAB.

Methods: Between August 2010 and September 2015, 10 consecutive patients with VSD and patent ductus arteriosus-dependent CoA or IAA underwent bPAB because of an AoV diameter of approximately <50% of the normal value (n = 6), severe subaortic stenosis and poor patient condition (n = 1, respectively), or low birthweight (n = 2).

QT Dynamics During Exercise in Asymptomatic Children with Long QT Syndrome Type 3.

Sympathetic provocative testing is commonly used to detect the abnormal QT dynamics in long QT syndrome (LQTS) patients, particularly LQTS type 1 and type 2. However, little is known about LQTS type 3 (LQT3). We investigated QT dynamics during exercise testing in LQTS patients, particularly LQT3.

QT dynamics evaluated on fully automated QT measurement in children.

Background: The association between QT interval and heart rate (HR), QT dynamics, has been reported to vary greatly between individuals. We investigated QT dynamics using fully automated QT interval measurement with a commercially available multifunctional electrocardiogram (ECG) recorder.

Methods: The study group included 17 otherwise healthy subjects (mean age, 12.

Phrenic nerve protection via packing of gauze into the pericardial space during ablation of cristal atrial tachycardia in a child.

The success of catheter ablation of focal atrial tachycardia is limited by possible collateral damage to the phrenic nerve. Protection of the phrenic nerve is required. Here we present a case of a 9-year-old girl having a history of an unsuccessful catheter ablation of a focal atrial tachycardia near the crista terminalis (because of proximity of the phrenic nerve) who underwent a successful ablation by means of a novel technique for phrenic nerve protection: packing of gauze into the pericardial space.

Hypoplastic left heart syndrome in PAGOD syndrome.

Chromosomal abnormalities as well as non-cardiac anomalies have been identified as independent risk factors for surgical morbidity and mortality in Fontan palliation. The combination of malformations consisting of pulmonary hypoplasia, agonadism (sex reversal), omphalocele, and diaphragmatic defect is compatible with pulmonary artery and lung hypoplasia, agonadism, omphalocele, and diaphragmatic defect (PAGOD). Most cases have been associated with cardiac disease, particularly hypoplastic left heart syndrome (HLHS) that is potentially destined for Fontan palliation.

High prevalence of the SCN5A E1784K mutation in school children with long QT syndrome living on the Okinawa islands.

Background: Genetic testing for long QT syndrome (LQTS) is now in clinical practice. We conducted molecular genetic analyses to definitively diagnose LQTS and to determine its subtypes for gene-specific treatment. We conducted a retrospective study to determine the characteristics of schoolchildren with LQTS living on the Okinawa Islands.

Automated external defibrillator documented degeneration of pre-excited atrial fibrillation into ventricular fibrillation.

Wolff-Parkinson-White (WPW) syndrome can be the cause of syncope or sudden cardiac death, which results from ventricular fibrillation (VF) degenerated from rapid anterograde conduction of atrial fibrillation (AF) to the ventricles through the accessory pathway. We present a case of WPW syndrome in which recording the actual moment of onset of the degeneration of pre-excited AF into VF. This was fortuitous and also lucky for this patient.

Dual atrioventricular nodal non-reentrant tachycardia in a child undergoing repair of ventricular septal defect.

Paroxysmal non-reentrant supraventricular tachycardia due to double ventricular response through antegrade dual atrioventricular nodal pathways by a single atrial excitation has been reported in limited adult cases but not in pediatric patients with structurally normal hearts or with congenital heart defects. We report the case of a 5-year-old boy with non-reentrant double-ventricular response (DVR) supraventricular tachycardia (SVT) after repair of ventricular septal defect. To the best of our knowledge, this is the first pediatric report about an electrophysiologic study and successful selective radiofrequency (RF) catheter ablation for the slow pathway leading to tachyarrhythmia that is difficult to manage medically.