Microvascular Endothelial Function Assessed Using Peripheral Arterial Tonometry in Adolescents with Repaired Congenital Heart Disease.

Atherosclerosis can develop in adult patients with congenital heart disease (CHD) and should be given attention. Endothelial function is well known as a predictor of the development of atherosclerosis but has not been well investigated in patients with repaired CHD. This study aimed to clarify the endothelial function and its relationship with clinical backgrounds and parameters in adolescents with various types of repaired CHDs. Endothelial function was evaluated using peripheral arterial tonometry (PAT). The reactive hyperemia index (RHI) was evaluated and compared between adolescents with repaired CHD and those in the control group. The relationship between the clinical background and parameters was also investigated in patients with repaired CHD. Forty-eight patients with repaired CHD (age 14.0 ± 3.3 years) and 114 healthy volunteers were included in this study. Patients with repaired CHD comprised 16 with repaired non-cyanotic CHD, 14 with repaired tetralogy of Fallot, and 18 who underwent the Fontan procedure. RHI in the repaired CHD group was significantly lower than in the control group. There was no significant correlation between the RHI and blood biochemical markers, such as uric acid, creatine, and brain natriuretic peptide levels. The RHI was significantly higher in patients taking angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) than in those not taking them. Endothelial function was impaired in adolescents with repaired CHD compared to that in the control group. Microvascular endothelial dysfunction developed even in adolescents with simple non-cyanotic CHD.

Safety and Efficacy of Landiolol Hydrochloride in Children with Tachyarrhythmia of Various Etiologies.

The safety and efficacy of landiolol have not been fully elucidated in pediatric patients. This study aimed to clarify the safety and efficacy of landiolol in a pediatric cohort. We retrospectively assessed the clinical features of 21 pediatric patients who were administered landiolol at our hospital. We also investigated the rates of sinus rhythm conversion and heart rate response. The median patient age was 7 months (interquartile range 1-13 months). The etiology of tachyarrhythmia was junctional ectopic tachycardia in 10 patients (47.6%), atrial tachycardia in 10 patients (47.6%), and ventricular tachycardia in 1 patient (4.8%). Of the 21 children, 18 (85.7%) had congenital heart defects, including 14 (77.8%) in whom a landiolol infusion was performed perioperatively. The landiolol infusion was effective in 18 pediatric patients (85.7%), as measured by the conversion to sinus rhythm or a reduced heart rate. Atrial tachycardia in the perioperative period was terminated in all patients. Of 7 patients with tachyarrhythmias unrelated to the perioperative period, landiolol was effective in 5. No adverse effects were reported in any patient. Landiolol infusion is effective and safe in pediatric patients with tachyarrhythmia of various etiologies, especially those with atrial tachyarrhythmia during the perioperative period.

Acquired and progressive coronary arterial fistulae in patients with single-ventricle physiology and treated with pulmonary vasodilators.

BACKGROUND: Cardiac dysfunction, arrhythmia, and hepatic fibrosis are well-known complications after right heart bypass surgery in patients with single-ventricle physiology. However, little is known about coronary arterial fistulae, and only a few reports have been published. This study aimed to elucidate the clinical characteristics of these rare coronary arterial fistulae that developed as complications in cases of single-ventricle physiology after right heart bypass surgery. METHODS: We retrospectively investigated the clinical features and courses of patients who developed acquired and progressive coronary arterial fistulae after right heart bypass surgery in our hospital. RESULTS: We identified three cases of coronary arterial fistulae out of 21 patients who underwent right heart bypass surgery. All three cases underwent cardiac catheterisation for post-operative evaluation and were administered pulmonary vasodilators of phosphodiesterase type V inhibitors, antiplatelet, anticoagulation, and diuretics. Moreover, they had common clinical features such as right-dominant single ventricle and long-term exposure to chronic hypoxia. Serial angiograms revealed acquired and progressive coronary arterial fistulae. In addition, coronary arterial fistulae contributed to their symptoms of heart failure. CONCLUSION: Patients with chronic hypoxia and dominant right ventricle, who are treated with phosphodiesterase type V inhibitors, should be followed up after right heart bypass surgery to monitor the possible development of coronary arterial fistulae. Moreover, the indication for pulmonary vasodilators in single-ventricle physiology after right heart bypass surgery should be optimised to avoid adverse effects.

Multiple brain abscesses with good prognosis in an infant with cyanotic congenital heart disease: a case report.

BACKGROUND: Brain abscesses are relatively rare, but they are a potentially life-threatening condition. Predictive factors for poor outcome are a young age and the presence of multiple abscesses. We report a case of a 15-month-old girl with cyanotic congenital heart disease who developed multiple brain abscesses caused by Streptococcus intermedius. The patient was treated with a combination of surgical aspiration and antimicrobial therapy without apparent neurological sequelae. To the best of our knowledge, this is the youngest such patient to have been reported in the literature. We explore the possible causes of her good outcome. CASE PRESENTATION: At the age of 15 months, the Japanese patient initially was presented to our hospital with transient eye deviation to the left and vomiting. In a blood examination, her white blood cell count (12,720 per mm3 with a left shift) and C-reactive protein level (1.23 mg/ml) were slightly elevated. Magnetic resonance imaging of the brain showed three mass lesions. These were 1.5-cm, 1.9-cm, and 1.2-cm rim-enhancing lesions with extensive surrounding edema. Brain abscesses were diagnosed, and vancomycin (50 mg every 12 hours) and meropenem (40 mg every 8 hours) were started empirically. However, because each brain abscess was enlarged at 8 days after admission, surgical aspiration was performed at 10 days after admission, and cultures of the aspirated pus grew S. intermedius. Penicillin G (0.7 million units every 4 hours) and ceftriaxone (280 mg every 12 hours), to which this isolate is susceptible, were then administered, and the brain abscesses reduced in size. After 1 month of ceftriaxone and 3 months of penicillin G treatment, all of the brain abscesses disappeared. Apparent neurological sequelae were not observed at 6 months after onset. CONCLUSIONS: A good outcome can be obtained if multiple brain abscesses develop in infancy or early childhood in cases without unconsciousness at admission, meningitis, or sepsis. Appropriate antimicrobial therapy should be started immediately after diagnosis, with surgical aspiration performed to identify the causative pathogen and avoid intraventricular rupture of the brain abscesses.

Catheter ablation in children and patients with congenital heart disease: Review of 1021 procedures at a high-volume single center in Japan.

BACKGROUND: Although pediatric catheter ablation therapy has often been described, few reports on outcomes in a large series of patients at a single center are available. OBJECTIVE: The purpose of this study was to evaluate arrhythmia substrates, outcomes, and complications of catheter ablation in children and patients with congenital heart disease (CHD) performed at a single center. METHODS: We retrospectively analyzed all pediatric patients <18 years and patients of all ages with CHD who underwent ablation therapy between June 2006 and May 2018. RESULTS: A total of 1021 ablation procedures were performed in 877 patients (median age 12.5 years; range 2 months to 67 years). This cohort included 152 CHD patients, 90 small patients (<15 kg), and 14 infants (<1 year). The most frequent indication was Wolff-Parkinson-White pattern (WPW) (n = 287 [32.7%]). Of the 55 patients with asymptomatic WPW, 40 patients (72.7%) had retrograde accessory pathway conduction. Overall success and recurrence rates were 93.5% and 17.3%, respectively. Small patients and CHD patients had lower success rates. No deaths occurred. Serious complications occurred in 5 patients. CONCLUSION: Catheter ablation is safe and effective for treatment of arrhythmia in pediatric and CHD patients. However, ablation was less successful in small patients and CHD patients. The risk of complications was similar to those previously reported for catheter ablation in pediatric, CHD, and adult patients.

Safety and Efficacy of Radiofrequency Catheter Ablation for Tachyarrhythmia in Children Weighing Less Than 10 kg.

An increasing number of children are undergoing radiofrequency catheter ablation (RFCA) for tachyarrhythmia. However, infants and toddlers undergoing RFCA are often resistant to medication or need to eliminate arrhythmia substrate, and the risks of RFCA complications are still high in infants and toddlers. From April 2008 and December 2016, 285 children who underwent radiofrequency catheter ablation (RFCA) were stratified according to body weight (group A, less than 10 kg, n = 22; group B, over 10 kg, n = 263) and the clinical features of RFCA were retrospectively reviewed in these groups. Indications for RFCA included drug-refractory tachyarrhythmia or symptomatic tachycardia and tachycardia-induced cardiomyopathy. The acute success rate in this group was 90.9%, with a relatively low recurrence rate (15.0%) after 7.0 ± 1.6 years follow-up. We performed RFCA using only 2-4 catheters in all cases. Major complications included complete right bundle branch block in one patient. No significant differences in rates of success, recurrence, or complications were noted between children weighing less and more than 10 kg. RFCA is safe and efficacious for tachyarrhythmia even in patients weighing less than 10 kg.

Microvascular endothelial function in Japanese early adolescents.

Endothelial dysfunction is the early predictive factor for the development of atherosclerosis and future cardiovascular diseases in adulthood. The prevalence of endothelial dysfunction in children and early adolescents is increasing worldwide. Peripheral arterial tonometry is a noninvasive technique for assessing peripheral microvascular function and is used as a validated marker of endothelial function. We assessed anthropometric parameters, blood pressure, arterial stiffness, and peripheral endothelial function in 157 Japanese early adolescents (75 boys and 82 girls). We measured peripheral endothelial function by using peripheral arterial tonometry to determine the reactive hyperemia index, and assessed the association of reactive hyperemia index with parameters of anthropometry and arterial stiffness. The mean reactive hyperemia index of all subjects was 1.85 ± 0.6, and there was no difference of reactive hyperemia index according to sex. Reactive hyperemia index was significantly associated with systolic and diastolic blood pressures, and had no correlation with anthropometric parameters and arterial stiffness markers. The reactive hyperemia index values among Japanese early adolescents were similar to those reported in previous studies on children and early adolescents. This noninvasive technique may be useful for the assessment of microvascular endothelial function among children and early adolescents.

Fatal cardiac anomaly of unguarded mitral orifice with asplenia syndrome.

We report the case of a newborn baby with an unguarded mitral orifice associated with asplenia syndrome, double-outlet right ventricle, dysplastic tricuspid valve, and pulmonary stenosis. This case was accompanied by severe tricuspid regurgitation and severe right ventricular hypertrophy. The patient had a fatal clinical course due to severe hypoxia and congestive heart failure. Unguarded mitral orifice is a rare disease in which there has been no previous report of lethal clinical course during the neonatal period. Prior reports stated that unguarded mitral orifice was a new constellation of defects and that its etiology and embryology could be classified in the same category because of similar associated malformations of double-outlet right ventricle and pulmonary stenosis or atresia. However, the present case was diagnosed on autopsy as also having asplenia syndrome. Therefore, it is possible that the genetic etiology of unguarded mitral orifice in this case was different from cases of non-heterotaxy. .

Persistent high fever for more than 10 days during acute phase is a risk factor for endothelial dysfunction in children with a history of Kawasaki disease.

BACKGROUND: Endothelial dysfunction has previously been reported in children with a history of Kawasaki disease, but the determinants of endothelial function in Kawasaki disease patients are still unknown. In this study, we investigated endothelial function in Kawasaki disease patients and attempted to identify risk factors for persistent endothelial dysfunction. METHODS: Using high-resolution ultrasound, we measured the percent flow-mediated dilatation, an arterial response to reactive hyperemia, to evaluate endothelial function in 67 patients with a history of Kawasaki disease and 28 age- and sex-matched control subjects. We divided the Kawasaki disease patients into a group with impaired endothelial function (the percent flow-mediated dilatation below -2 standard deviations of the control group) and a group with normal endothelial function (the percent flow-mediated dilatation more than -2 standard deviations of control). Logistic multiple regression analysis was performed to identify independent predictors of impaired endothelial function. RESULTS: In Kawasaki disease patients, the percent flow-mediated dilatation was significantly lower than in the control subjects (9.8±3.6%, compared with 13.1±3.4%, p<0.01). In 13 Kawasaki disease patients (3 patients with coronary artery lesions and 10 patients without coronary artery lesions), the percent flow-mediated dilatation was below -2 standard deviations of control. Logistic multiple regression analysis showed that a febrile period of longer than 10 days during the acute phase was the significant risk factor for endothelial dysfunction (odds ratio: 8.562; 95% confidence interval: 1.366-53.68). Presence of coronary artery lesions was not a determinant of endothelial dysfunction. CONCLUSIONS: Systemic endothelial dysfunction exists in children with a history of Kawasaki disease, and a febrile period of longer than 10 days during the acute phase is an independent predictor of endothelial dysfunction irrespective of coronary artery involvement.

[Juvenile neuronal ceroid-lipofuscinosis with hypertrophic cardiomyopathy and left ventricular noncompaction: a case report].

We report a 17-year-old female with juvenile neuronal ceroid-lipofuscinosis (NCL) accompanied by hypertrophic cardiomyopathy (HCM) and left ventricular noncompaction (LVNC). Within our knowledge, this is the first reported case of juvenile NCL with LVNC, and the youngest case of HCM diagnosed by ultrasound. Juvenile NCL is a progressive hereditary disease involving multi-organ accumulation of ceroid-lipofuscin; its resulting complications require prompt attention. Due to its relative rarity, its cardiac involvement is not well known. Based on findings from this patient and related juvenile NCL cases, the risk of cardiac involvement tends to increase with age; a high frequency of ventricular hypertrophy has been reported in patients aged older than 20 years of age. Medical progress and comprehensive care have led to longer survival in patients with juvenile NCL, which likely increases the incidence of cardiac involvement. In relation to HCM in other metabolic disorders, attention should be paid to arrhythmias, including repolarization disturbances, sinus node dysfunction and ventricular tachycardia. LVNC is a cardiomyopathy characterized by prominent left ventricular trabeculae and deep intratrabecular recesses, which are associated with diastolic or systolic dysfunction, thromboembolic complications and arrhythmias. From ours and other case reports, we recommend regular follow-up of NCL patients as follows: echocardiography to estimate cardiomyopathy, Holter monitoring to identify arrhythmias, and computed tomography to detect thrombosis from both ventricles. The mechanism of the HCM and LVNC associated with juvenile NCL remains unclear. Our case requires careful follow-up. Prospective studies of the cardiac involvement in juvenile NCL are necessary to further elucidate its pathomechanism.

Electrophysiologic studies and radiofrequency catheter ablation of ectopic atrial tachycardia in children.

Ectopic atrial tachycardia (EAT) often resists medical therapy, making radiofrequency catheter ablation (RFCA) the preferred treatment. This study reviewed the records of 35 patients who underwent electrophysiologic studies (EPS) and 39 RFCA procedures for EAT during a 10-year period. Of the 35 patients, 10 (28%) presented with decreased ventricular function and tachycardia-induced cardiomyopathy (TIC). The EAT originated on the right atrial side in 19 patients (54%) and on the left atrial side in the remaining 16 patients (46%). The right atrial sites included the right atrial appendage (RAA) (n = 9, 25%), the tricuspid annulus (n = 7, 20%), and the crista terminalis (n = 3). The left atrial sites included the left atrial appendage (LAA) (n = 6, 17%), the pulmonary veins (n = 5, 14%), the mitral annulus (n = 3), and the posterior wall of the left atrium (n = 2). The mechanism of all EAT probably is automaticity. All EATs could be abolished using RFCA. Follow-up data were available for all patients 2 to 8 years after RFCA. All 35 patients remained recurrence free, and ventricular function improved for all 10 patients with TIC. The origin of EAT in children differed from its origin in adults. The authors conclude that RFCA is a safe and effective treatment option for children with refractory EAT and should be considered early in the course of their illness.

Successful catheter ablation of reentrant junctional tachycardia in a patient with asplenia syndrome before total cavo-pulmonary connection.

Asplenia syndrome is commonly associated with complex structural cardiac malformations, and junctional tachycardia (JT), which may compromise hemodynamic status, has been reported in association with asplenia syndrome.(1) We report successful radiofrequency catheter ablation of reentrant JT in a patient with asplenia syndrome.