Predictive Role of Atrial Strain, Aortic Stiffness, and Muscle-Related Factors for Maximal and Submaximal Exercise Capacity in Fontan Patients.

Fontan patients' exercise capacity and associated risks of morbidity and mortality necessitate a comprehensive study. We aim to explore maximal and submaximal exercise capacity, assessing the impact of cardiovascular and peripheral factors, including atrial strain, aortic stiffness, and muscle-related factors. Fontan patients and matched controls were examined. Clinical details were recorded, and atrial strain and aortic stiffness were measured using echocardiography. Hand grip and knee extensor strength were assessed, and muscle oxygenation was recorded. Cardiopulmonary exercise test determined exercise capacity, with peak oxygen uptake/kg (VO2/kg) and oxygen uptake efficiency slope/kg (OUES/kg) as markers for maximal and submaximal capacity, respectively. Thirty-one Fontan patients (median age = 18, range = 7-31 years) and 30 controls (median age = 18, range = 7-32 years) were studied. No significant differences in body composition and muscle strength were observed between Fontan patients and controls (p > 0.05). Fontan patients exhibited lower peak VO2/kg and OUES/kg (p < 0.001 for both), along with decreased atrial strain and increased aortic stiffness compared to controls (p < 0.001). Aortic pulse wave velocity (PWV), left atrial strain contractile phase (LASct), and knee extensor strength independently predicted peak VO2/kg (R2 = 0.514) and OUES/kg (R2 = 0.486) in Fontan patients; age was a predictor for peak VO2/kg. Atrial contractile strain, aortic stiffness, and knee extensor muscle strength are predictors of submaximal exercise capacity; furthermore, age, along with these variables, serves as predictors of maximal exercise capacity in Fontan patients. Evaluating hemodynamic, vascular, and muscular parameters, in conjunction with assessing both maximal and submaximal exercise capacities, is crucial for optimizing disease management in Fontan patients. Clinicaltrials.gov registration: NCT05011565.

The risk of cardiovascular comorbidity in children with Behçet's disease.

OBJECTIVE: Patients with Behcet disease (BD) may experience long term morbidity caused by different forms of cardiovascular diseases. This study aimed to assess the risk for cardiovascular comorbidity in pediatric BD patients with and without vascular involvement, independent of the contribution of traditional risk factors. METHODS: Pediatric patients classified as BD according to the 2015 International Pediatric BD criteria were included in the study. Twenty-four-h ambulatory blood pressure monitoring (ABPM), transthoracic echocardiography, and carotid intima media thickness (cIMT) measurements were performed. Patients with an active disease and have other known risk factors for cardiovascular disease were not included in the study. RESULTS: Thirty-one children and adolescents with pediatric BD (16 female, 51.6%; F/M: 1.06) were enrolled in the study. Among all BD patients 10 patients (34.4%) had abnormal ABPM. Carotid IMT values, mean arterial pressure, systolic and diastolic blood pressure by ABPM and the prevalence of abnormal ABPM, non-dipping, and ambulatory hypertension were similar between patients with and without vascular involvement. The echocardiography measurements showed that BD patients with vascular involvement had significantly higher velocity and velocity time integral of the left ventricle outflow tract which may point out increased stiffness of the aorta. CONCLUSION: Pediatric BD patients with vascular involvement may tend to have more cardiovascular risk factors. However, cardiovascular assessment should be considered in all BD patients regardless of the involved systems. We suggest that ABPM may accurately define hypertension and cardiovascular risk in BD.

Percutaneous Transcatheter Retrieval of Central Venous Port Fragments in Pediatric Patients; A Single-center Experience From the Pediatric Cardiology Department.

BACKGROUND: Split/fracture and embolization of central venous/shunt catheters are rare but serious complications in children. Percutaneous retrieval of intravascular foreign bodies is an important minimal invasive treatment. This study is aimed to represent our largest pediatric sample experience till now of 17 years from a single institution. Another aim is to compare the results regarding the removal or leaving in place of embolized or ruptured intravascular or cardiac venous catheter parts in children. PATIENTS AND METHODS: A total of 26 cases were included in this study. Any pediatric patient with normal coagulation parameters and a fractured catheter fragment was included in this study. Other intravascular foreign bodies related to interventional devices and/or pacemaker/implantable cardiac defibrillator leads were excluded from this study. RESULTS: Twenty-six patients, of whom 25 had oncologic diseases and 1 had a ventriculoatrial shunt, were included. The median age was 83.5 months (between 20 mo and 18 y) at treatment.Superior vena cava (9 cases), followed by the right atrium (5 cases), were the most two common sites of embolization for cardiovascular foreign bodies. The success rate of percutaneous retrieval was 92.3% in all patients. There were neither complications nor deaths. The retrieval technique revealed a predisposition for extraction through the femoral vein (96.1%) and using snare techniques (100%). Additional catheters like pigtail, National Institutes of Health, or ablation catheters were used for stabilization in selective cases in which the permanent central venous fragments stuck to the vessels. A tractional maneuver and capturing the ruptured material in the middle were other trick points for successful retrieval. Patients were asymptomatic in 76.9% of cases (20/26). CONCLUSION: Percutaneous retrieval of cardiovascular foreign bodies is a reasonable, safe, and effective way in children when the catheter fragments are free and mobile. It should be considered the preferred treatment option instead of surgery. In patients where catheter fragments are stuck and are adherent to vessels, it could be left, and followed up by anticoagulation. Novel techniques accompanied by an experienced team could be helpful in difficult cases.

Fetal cardiac interventions: First-year experience of a tertiary referral center in Turkey.

AIM: To present the first-year experience of fetal cardiac interventions (FCIs) in a tertiary referral hospital and to evaluate the outcomes. METHODS: This retrospective study consisted of four pregnant women who underwent fetal pulmonary or aortic balloon valvuloplasty between November 2020 and June 2021. The procedures were performed with a percutaneous cardiac puncture under the ultrasonography guidance. Gestational age at intervention, procedural success, complications, and perinatal outcomes were evaluated. Procedural complications defined as fetal bradyarrhythmia requiring treatment, pericardial effusion requiring drainage, balloon rupture, and fetal death. The procedure was considered technically successful if the valve was dilated with a balloon catheter. Ultimately successful procedure was defined as the discharge of infants alive with biventricular circulation. RESULTS: A total of 5 FCIs attempted between 26 + 3 and 28 + 2 gestational weeks. While the procedure was technically successful in 2 cases with pulmonary stenosis, both attempts were unsuccessful in the fetus with pulmonary atresia. Although the procedure was technically successful in the patient with critical aortic stenosis, it ultimately failed. No fetal death occurred in our series and there were no procedure-related significant maternal complications. However, three interventions were complicated by fetal bradycardia and pericardial effusion necessitating treatment, and balloon rupture cropped up in one case. CONCLUSION: FCIs may lead to improving the likelihood of a biventricular outcome for selected fetuses. Careful selection of patients and centralization of experience are essential for obtaining favorable outcomes. Operators should be aware of procedural complications. Improved procedural techniques with a lower complication rate will be achieved through advanced medical technology and special balloon catheters.

Comparison of IVIG resistance predictive models in Kawasaki disease.

BACKGROUND: We aimed to compare the ten different scores (by Kobayashi, Egami, Harada, Formosa, Sano, Piram et al., Wu et al., Yang et al., Tan et al., and Kanai et al.) to assess their performance in predicting IVIG resistance in Turkish children. METHODS: Complete and incomplete KD patients diagnosed with KD at Hacettepe University between June 2007 and September 2019 were evaluated retrospectively. RESULTS: A total of 129 patients, 79 boys (61.2%), with a median age 36 (IQR 19.5-57.0) months were evaluated. Sixteen patients (12.4%) had IVIG resistance. Sensitivity was low for all the ten scores. Tan, Sano, and Egami predictive models had the highest specificity (97.3, 89.4, 86.7%, respectively). Almost all scoring systems distinguished the group of patients with low risk for IVIG resistance but could not differentiate IVIG-resistant patients. Multivariate analysis for the laboratory features showed that platelet count <300 × 109/L and GGT serum levels were independent risk factors for IVIG resistance (OR: 3.896; 95% CI: 1.054-14.404; p = 0.042 and OR: 1.008; 95% CI: 1.001-1.015; p = 0.050). CONCLUSIONS: The current scoring systems had a low sensitivity for predicting the risk for IVIG resistance in Turkish children. On the other hand, increased serum GGT levels and low platelet count were risk factors for predicting IVIG resistance. IMPACT: Intravenous immunoglobulin (IVIG) resistance may be observed in 10-20% of patients diagnosed with Kawasaki disease. Coronary artery involvement is more frequent in IVIG-resistant patients. It is important to predict the patients who might develop IVIG resistance to improve prognosis. The performance of the IVIG resistance predictive models in Kawasaki disease in our population is limited due to the low sensitivity.

Differences and similarities of multisystem inflammatory syndrome in children, Kawasaki disease and macrophage activating syndrome due to systemic juvenile idiopathic arthritis: a comparative study.

To compare the clinical and laboratory findings of multisystem inflammatory syndrome in children (MIS-C), patients with Kawasaki disease (KD) and with macrophage activating syndrome due to systemic juvenile idiopathic arthritis (sJIA-MAS) on real-life data. Patients diagnosed with MIS-C, KD, and sJIA-MAS from 12 different centers in Turkey who were followed for at least 6 months were included in the study. Demographic, clinical, and laboratory findings of all patients were analyzed. A total of 154 MIS-C, 59 KD, and 31 sJIA-MAS patients were included. The median age of patients with MIS-C were higher than those with KD while lower than those with sJIA-MAS (8.2, 3, 12 years, respectively). Myalgia (39.6%), cardiac (50.6%), gastrointestinal (72.7%), and neurological (22.1%) involvements were more common in patients with MIS-C compared to others. MIS-C patients had lower levels of lymphocyte (950 vs 1700 cells/µl) and thrombocyte (173,000 vs 355,000 cells/µl) counts and higher pro-BNP (1108 vs 55 pg/ml) levels than KD. Ferritin levels were higher in patients with MIS-C compared to patients with KD while they were lower than patients with sJIA-MAS (440, 170, 10,442 ng/ml, respectively). Patients with MIS-C had a shorter duration of hospitalization than sJIA-MAS (p = 0.02) while they required intensive care unit admission more frequently (55 vs 8 patients, p < 0.001). The median MAS/sJIA score of MIS-C patients was - 1.64 (- 5.23 to 9.68) and the median MAS/sJIA score of sJIA-MAS patients was -2.81 ([- 3.79] to [- 1.27]). MIS-C patients displayed certain differences in clinical and laboratory features when compared to KD and sJIA-MAS. Definition of the differences and similarities between MIS-C and the other intense inflammatory syndromes of childhood such as KD and MAS will help the clinicians while making timely diagnosis.

Two decades of experience on ablation in children with Ebstein's anomaly.

INTRODUCTION: Accessory pathways are commonly seen due to delamination of tricuspid valve leaflets. In addition to accessory pathways, an enlarged right atrium due to tricuspid regurgitation and incisional scars creates substrates for atrial re-entries and ectopic tachycardia. We sought to describe our experience with catheter ablation in children with Ebstein's anomaly. METHODS AND RESULTS: During the study period, of 89 patients diagnosed with Ebstein's anomaly, 26 (30.9%) of them who underwent 33 ablation procedures were included in the study. Accessory pathways were observed in the majority of procedures (n = 27), whereas atrial flutter was observed in five, atrioventricular nodal reentrant tachycardia in five, and atrial tachycardia in two procedures. Accessory pathways were commonly localised in the right posteroseptal (n = 10 patients), right posterolateral (n = 14 patients), septal (n = two patients), and left posteroseptal (n = one patient) areas. Multiple accessory pathways and coexistent arrhythmia were observed in six procedures. All ablation attempts related to the accessory pathways were successful, but recurrence was observed in five (19%) of the ablations. Ablation for atrial flutter was performed in five patients; two of them were ablated successfully. One of the atrial tachycardia cases was ablated successfully. CONCLUSIONS: Ablation in patients with Ebstein's anomaly is challenging, and due to nature of the disease, it is not a rare occasion in this group of patients. Ablation of accessory pathways has high success, but also relatively high recurrence rates, whereas ablation of atrial arrhythmias has lower success rates, especially in operated patients.

Empiric slow-pathway ablation results for presumed atrioventricular nodal reentrant tachycardia in pediatric patients.

BACKGROUND: In pediatric patients with documented narrow QRS tachycardia that is suggestive of atrioventricular nodal reentrant tachycardia (AVNRT) and not inducible in electrophysiological study (EPS), empiric slowpathway ablation (ESPA) may be considered. There is limited data in children about this topic. METHODS: Seventy-nine patients who underwent cryoablation and/or radiofrequency ablation (RFA) for presumed AVNRT between January 2010 and January 2020, with no inducible tachycardia and no other tachycardia mechanisms during EPS, were included in this study. RESULTS: The age was between 6 and 18 years. All patients had no structural heart disease. Preablation exhibited sustained SP conduction for all patients. In all cases, the ablation end points were prolongation in wenckebach cycle length (WBCL) with loss of cross and/or jump, and/or echo beat. The end points were not achieved in two patients. Overall, the mean basal WBCL increased to 351 ms (240-500 ms) from 301.3 ms (180-420 ms), evident in the non-recurrence group. Nine patients had a transient AV block that improved. We followed the patients without medication for about 46.9 months (8 months to 10 years). Palpitations occurred again in 9 of 77 patients (clinical recurrence rate 9/79 - 11.3%). The documented ECG recurrence rate was 1.2% (1/79). In the non-recurrence group, WBCL prolongation was higher and mean age was lower than in the recurrence group (13.075 vs. 15.33 years). CONCLUSION: In cases with presumed AVNRT, ESPA seems to be a reasonable and safe way. In our study, we found our procedural success rate as 97.4% and follow-up recurrence rate as 12.6% (9+1/79).

Multiple Ablation Targets in Children: Multiple Accessory Pathways and Coexistent Arrhythmia.

The coexistence of different mechanisms of arrhythmia and multiple accessory pathways (MAPs) leading to multiple ablation targets is rarely seen in children, and data regarding these patients in the literature are limited. Herein, we aimed to evaluate patients who required multiple ablation applications, focusing on different targets during the procedures in children, and evaluating the characteristics of coexistent arrhythmia and MAPs, and the results of these procedures in children. Ablation procedures conducted between March 2009 and December 2018 were evaluated retrospectively, and patients with MAPs and/or coexistent arrhythmia who had undergone ablation procedures were included in the study. Among the 1210 patients who underwent ablation procedures, 52 patients (26 male, 26 female) were ablated for multiple targets. Of the 456 patients with APs, 21 had MAPs (4.6%) and of the 1210 patients who underwent ablation procedures, 31 patients had coexistent arrhythmia (2.5%). The patients had a mean age of 12.24 ± 3.4 (4-18) years and mean body weight of 45.17 ± 14.12 (17-74) kg. A total of 110 APs or foci were identified as quaternary in one patient, while it was triple in four patients. The procedures were unsuccessful in six targets of six patients. Although recurrence was observed in four patients, none were ablated for MAPs. Two complications were encountered, comprising ST segment depression that developed in one patient with Wolf-Parkinson-White syndrome, atrioventricular nodal re-entry tachycardia, and a temporary atrioventricular block during atrioventricular nodal re-entrant tachycardia ablation. The overall success rate according to the pathway/foci number was 94.5% (104/110), with a recurrence rate of 4.5% (5/110), and a complication rate of 1.8% (2/110). The patient success, recurrence, and complication rates were 88.4% (46/52), 7.6% (4/52), and 3.8% (2/52), respectively. In conclusion, the incidence of multiple arrhythmogenic foci and MAPs were not as low as expected in children. A structured and stepwise approach is mandatory for the diagnosis of the different mechanisms of tachycardia, even after successful ablation procedures. The success, recurrence, and complication rates were comparable with those of patients who had a solitary arrhythmogenic focus or solitary AP.

Bilateral thoracoscopic sympathectomy for cardiac denervation in pediatric population: Does Kuntz nerve cauterization have an impact on success?

INTRODUCTION: Cardiac sympathetic denervation (CSD) is an effective procedure for the treatment of inherited channelopathies. Left CSD has traditionally been recommended as neuromodulation to prevent arrhythmia episodes; however, it is thought that bilateral sympathectomy in combination with Kuntz ablation may have additional effects effective due to the anatomical variability of preganglionic sympathetic fibers. The aim of the study was to share our single-center clinical experience with bilateral thoracoscopic sympathectomy for cardiac denervation in different groups of pediatric patients with malignant arrhythmias. METHODS: Fourteen patients (seven with CPVT, five with LQTS, one with resistant ventricular tachycardia as a sequela of myocarditis, and one with cardiomyopathy and atrial tachycardia) underwent bilateral thoracoscopic sympathectomy for cardiac denervation. RESULTS: In all patients, arrhythmia episodes persisted despite medical therapy, and patients with implantable cardioverter-defibrillator received appropriate therapies. The rate of appropriate therapies was 3.25 per year (1-5 per year) in the year before sympathectomy. No major complications related to the procedure were observed in any of the patients. The QTc interval of LQTS patients decreased from 506.2 ± 16.9 ms before the procedure to 476 ± 28.8 ms after the procedure. The mean duration of follow-up after the procedure was 23.3 months (11-47 months). Only two patients received single episodes of therapy 12 and 22 months after CSD, and one patient had arrhythmic events due to noncompliance to medical therapy. CONCLUSION: Due to the anatomical variability of preganglionic fibers bilateral CSD with Kuntz nerve ablation effective treatment and is a safe option.

Fluconazole-associated QT interval prolongation and Torsades de Pointes in a paediatric patient.

"Torsades de pointes", a life-threatening rhythm disorder, is a polymorphic ventricular tachycardia that usually develops in association with a prolonged QT interval. Fluconazole, an anti-fungal drug, may also induce QT prolongation, in some cases subsequent torsades de pointes. Herein, we report a 16-year-old female presenting "torsades de pointes" after administration of fluconazole and rapidly improved upon cessation of the drug.

Giant diverticulum of coronary sinus with multiple accessory pathways treated with catheter ablation.

Coronary sinus abnormalities are usually associated with arrhythmia disorders when symptomatic. We report a 5-year-old 14 kg patient with a giant diverticulum of coronary sinus and Wolff-Parkinson-White syndrome. Catheter ablation therapy was decided during follow-up due to inadequate response to multidrug therapy. Posteroseptal and left posterolateral accessory pathways were established and radiofrequency ablation was performed successfully through coronary sinus.

Transcatheter closure of patent vertical vein after repair of total anomalous pulmonary venous connection: a case series.

BACKGROUND AND OBJECTIVES: Total anomalous pulmonary venous connection is a rare cyanotic CHD that requires surgical repair. An unligated vertical vein after total anomalous pulmonary venous connection surgery may help to decrease the episodes of post-operative pulmonary hypertensive crises, low cardiac output syndrome, and mortality. The aim was to assess long-term outcome and our post-operative transcatheter vertical vein closure experiences in five patients with repaired total anomalous pulmonary venous connection patients. METHODS: A retrospective study was conducted in five cases with an unligated vertical vein following repair of supra-cardiac total anomalous pulmonary venous connection at our hospital from 2011 through 2018. Patients characteristics, cardiac catheterisation findings, surgical, and transcatheter procedural details were retrospectively analysed. RESULTS: Transcatheter closure of the unligated vertical vein was technically successful in all the patients. Procedure-related complications were not observed in any of the patients. No long-term complication was found. CONCLUSIONS: We suggest that transcatheter closure of the patent vertical vein is an effective and well-tolerated alternative to the surgical approach.

Impact of Pacemakers and Implantable Cardioverter Defibrillators on the Psychosocial Functioning of Paediatric Patients.

Although implanted cardiac devices improve patients' physical health, long-term psychosocial effects especially in the paediatric population are still unknown. The aim of this study was to evaluate the psychosocial effects of cardiac devices in a paediatric population.Pediatric Quality of Life Questionnaire (PedsQoL) was used to evaluate life quality, Connor-Davidson Resilience Scale was used to evaluate resilience and Brief Symptom Inventory was used to evaluate psychiatric symptoms in a paediatric population with cardiac devices.Seventy-one patients were enrolled in the study. Fifty of them (70.5%) had a cardiac pacemaker and 21 of them (29.5%) had implantable cardioverter defibrillator. When compared to the control group both implantable cardioverter defibrillator and pacemaker using patients had lower quality of life (79.5 ± 12.4 versus 86.7 ± 12.1, p = 0.001) but no difference was observed in resilience and psychological pathologies. Age, gender, family size, and education level had no effect on quality of life. Implantable cardioverter defibrillator bearing patients had higher levels of anxiety than pacemaker patients (0.58 versus 0.30 p = 0.045), and implantable cardioverter defibrillator patients who had received shock in the last year had higher levels of somatisation than the group that did not receive device shock (0.17 versus 0.44 p = 0.022).In conclusion study showed that cardiac devices have negative effects on the psychosocial health of children. Cardiologist working with these patients should be aware of these pathologies and monitor not only physical health but also psychosocial health too.

Levels of Physical Activity and Physical Fitness in Pediatric Pacemaker Patients: A Cross-Sectional Study.

Children with heart diseases have reduced physical activity (PA) levels relative to their peers, which in turn increases cardiovascular risk. To the best of our knowledge, physical fitness and objectively measured PA levels have not been previously studied in children with pacemakers. We evaluated PA levels and physical fitness in pediatric pacemaker patients compared to their healthy peers. Twenty-eight pediatric patients with pacemakers (15 female, 13 male; mean age 13.43 ± 3.68 years) and 24 healthy subjects (14 female, 10 male; mean age 13.08 ± 3.67 years) were included. Physical fitness was assessed using the Munich Fitness Test (MFT). SenseWear Armband metabolic Holter device was used to record the PA for 7 consecutive days. MFT total and sub-parameter scores were significantly lower in the patient group (p < 0.05). Patients' total and active energy expenditure, PA level, total distance, number of steps, and vigorous PA were significantly lower than those of healthy children (p < 0.05). Sedentary activity and light, moderate, and very vigorous PA durations were similar in both groups (p > 0.05). Duration of mean moderate to vigorous PA was higher than 60 min/day recommended in PA guidelines in both patients and healthy subjects. These results provide initial data on PA and fitness in children with pacemakers and suggest that physical fitness and activity levels in children with pacemakers are lower than in healthy peers. Appropriate exercise programs may improve PA levels in pediatric pacemaker patients. Awareness of the importance of PA should be raised among the parents and families of these children.

Clinical efficacy and safety of switch from bosentan to macitentan in children and young adults with pulmonary arterial hypertension: extended study results.

BACKGROUND: Macitentan is an orally active, potent, dual endothelin receptor antagonist and is the only registered treatment for pulmonary arterial hypertension that significantly reduced morbidity and mortality in a long-term study. AIM: We have recently reported that switch from bosentan to macitentan significantly improved exercise capacity in children and young adults with pulmonary arterial hypertension in a 24-week prospective study and well tolerated without adverse events. We now aimed to evaluate clinical efficacy, safety of switch in a larger patient population, in a 24-month prospective study. METHODS: This is a single-institution, 24-month prospective study. Patients ≥12 years with idiopathic/heritable, pulmonary arterial hypertension, or related to CHD or residual pulmonary arterial hypertension due to repaired congenital systemic-to-pulmonary shunts and on bosentan treatment were included. Concomitant treatment with oral phosphodiesterase type 5 inhibitors/inhaled prostanoids was allowed. Outcome measures included change from baseline to 24 months, in the 6-minute walk distance, functional class, oxygen saturation at rest/after walk distance test, and natriuretic peptide levels. Safety end points included adverse events, laboratory abnormalities. RESULTS: Twenty-seven patients (19 adults/8 children, mean age: 21.1 ± 6.3 years (12-36), weight: 53.1 ± 15.7 kgs (26-87)) were included. Mean duration of macitentan treatment: 22.3 ± 3.9 months (9-24). Six-minute walk distance significantly improved from baseline (mean: 458 ± 79 m (300-620)) at 6 months (mean: 501 ± 73 m (325-616) + 43 m) (p < 0.05), at 12 months (mean: 514 ± 82 m (330-626) + 56 m) (p < 0.05), and at 24 months (mean: 532 ± 85 m (330-682) + 74 m) (p < 0.05). We observed a significant improvement during the first 6 months but no incremental improvement after 6 months (p > 0.05). Macitentan did not significantly change functional class, oxygen saturation, and natriuretic levels (p > 0.05). None of the patients had anaemia, hepatotoxicity, and peripheral edema. CONCLUSIONS: Our study is the first study which showed that switch from bosentan to macitentan improved exercise capacity in children and young adults with pulmonary arterial hypertension significantly in the first 6 months and compared to baseline in 24 months and well tolerated without adverse events.

Palivizumab compliance in congenital heart disease patients: factors related to compliance and altered lower respiratory tract infection viruses after palivizumab prophylaxis.

BACKGROUND: Lower respiratory tract infections caused by respiratory syncytial virus can be severe during infancy, which requires admission to the hospital. These infections may be more severe especially in patients with congenital heart disease. Passive immunisation with palivizumab, a monoclonal antibody, is recommended in high-risk infants. We tried to determine the compliance rates, factors affecting compliance, and also other microorganisms responsible for lower respiratory tract infections after palivizumab prophylaxis in these patients. METHODS: We evaluated patients' compliance to prophylaxis with palivizumab in two consecutive respiratory syncytial virus seasons from pharmacy records. We also investigated factors affecting compliance and the frequency of hospitalisations for lower respiratory tract infections. We investigated the causative microorganisms detected in hospitalised patients. RESULTS: In this study, 86.7% of the desired number of injections was achieved in 176 patients in two seasons. Out of these, 117 patients (66.4%) received all the doses they were prescribed. Although not statistically significant, compliance to prophylaxis was higher in male patients, cyanotic patients, those who started under 1 year old, and who lived in the city centre. Human metapneumovirus, parainfluenza type 3, and bocavirus were detected in the hospitalised patients. CONCLUSION: Patients with congenital heart disease can survive the period of infancy with less problem by making palivizumab prophylaxis more effective, and awareness about non- respiratory syncytial virus factors may be a guide for the development of new treatments.

Clinical efficacy and safety of switch from bosentan to macitentan in children and young adults with pulmonary arterial hypertension.

BACKGROUND: Macitentan is an orally active, potent, dual endothelin receptor antagonist and is the only registered treatment for pulmonary arterial hypertension that significantly reduced morbidity and mortality in a long-term event-driven study. Aim Few studies compared the clinical efficacy and safety of switch from bosentan to macitentan only in adult patients with pulmonary arterial hypertension. We aimed to evaluate the clinical efficacy and safety of switch from bosentan to macitentan in children and young adults. METHODS: This is a single-institution, 24-week prospective study. Patients ⩾12 years of age with idiopathic/heritable pulmonary arterial hypertension or related to CHD or residual pulmonary arterial hypertension due to repaired congenital systemic-to-pulmonary shunts and on bosentan therapy were included. Concomitant treatment with oral phosphodiesterase type 5 inhibitors and inhaled prostanoids was allowed. Outcome measures included change from baseline to week 24, in the 6-minute walk distance, functional class, oxygen saturation at rest/after 6-minute walk distance test, systolic pulmonary artery pressure estimated by echocardiography, and brain natriuretic peptide levels. Safety end points included adverse events laboratory abnormalities. RESULTS: A total of 13 patients - 5 male and 8 female - completed the study. The mean age was 20.3±6.5 years (12-35) and weight was 54.0±14.5 kg (27-75). Five patients were ⩽18 years of age. Macitentan improved 6-minute walk distance from baseline (mean: 466±35 m (300-590)), at 12 weeks (mean: 494±78 m (325-590), +28 m) (p0.05). None of the patients had anaemia, hepatotoxicity, and peripheral oedema. CONCLUSIONS: Our study is the first study that showed that switch from bosentan to macitentan significantly improved exercise capacity in children and young adults with pulmonary arterial hypertension and is well tolerated without any adverse events.

Transcatheter closure of the patent foramen ovale in children: intermediate-term follow-up results.

The patent foramen ovale is almost a normal anatomical hole between the atria with ~30% incidence in the general population. It has been suggested that the patent foramen ovale is the cause of some neurological events, which is explained by paradoxical embolism. Transcatheter closure of the patent foramen ovale is a common procedure in adult patients with cerebral ischaemic events, but there are limited data investigating the results in children. Between January, 2005 and February, 2014, 17 patients' patent foramen ovales were closed by the transcatheter approach in our department. The indications for closure were transient ischaemic attack in 10 patients, stroke in four patients, and migraine in three patients. The mean age and mean weight at the time of the procedure were 11.1±3.7 years and 42.1±15.4 kg, respectively. We asked our patients whether their previous ailments continued. All patients responded to the study survey. In 15 patients, ailments did not continue after patent foramen ovale closure and they significantly decreased in two of them. We suggest that under the right conditions device closure of the patent foramen ovale is a safe solution for these cryptogenic ischaemic events and migraine.

Midterm Results of Implantable Cardioverter Defibrillators in Children and Young Adults from a Single Center in Turkey.

BACKGROUND: Despite concerns about complications with the implantable cardioverter defibrillator (ICD), it is effective for the prevention of sudden cardiac death (SCD). We aimed to analyze our midterm experience with ICD in children and young adults. METHODS: This retrospective study included patients who were implanted with an ICD between 2001 and 2014. Demographic characteristics, clinical information, shock features, and complications for all patients with ICD were analyzed. The study population was divided into two groups: early-era patients implanted before 2008, and late-era patients implanted after 2008. RESULTS: Sixty-nine patients (median age: 12 years, median follow-up: 52 months) were implanted with an ICD. Diagnostic categories were channelopathy (56.6%), cardiomyopathy (36.2%), congenital heart disease (5.8%), and other (1.4%). We performed implantation for primary prevention in 66.6% (39.3% in early-era patients and 85.4% in late-era patients). Thirty-one (44.9%) received 139 appropriate shocks (66% of total shocks) while 14 (20.2%) received 71 inappropriate shocks. However, there was no statistically significant difference in the use of appropriate shocks in the primary (66.7%) versus the secondary (72.2%) prevention groups. The incidence of appropriate and inappropriate shock was 66.7% and 33.3% in the primary prevention group, and 72.2% and 27.8% in the secondary prevention group, respectively. Two patients died, although only one death was the result of a lead problem. CONCLUSIONS: Although lead integrity problems, inappropriate shocks, and infections are significant issues, ICD therapy appears to be a safe, effective, and necessary option for the prevention of SCD in both children and young adults.