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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.

“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.

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).

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.

To analyze the role of sensitive cardiac troponin I (scTnI) and high-sensitive troponin T (hscTnT) in the determination of myocardial injury caused by volume and pressure load due to pulmonary hypertension (PH) and to investigate if these markers may be useful in the management of PH in childhood. Twenty-eight patients with congenital heart disease (CHD) with left to right shunt and PH, 29 patients with CHD with left to right shunt but without PH, and 18 healthy children, in total 75 individuals, were included in the study. All cases were aged between 4 and 36 months. Echocardiographic evaluation was performed in all cases, and invasive hemodynamic investigation was performed in 33 cases. Blood samples were obtained from all cases, for the measurement of brain natriuretic peptide (BNP), pro-brain natriuretic peptide (pro-BNP), sensitive cardiac troponin I (scTnI), and high-sensitive troponin T (hscTnT) levels. The mean BNP, pro-BNP, scTnI, and hsTnT levels were statistically significantly higher in patients with PH than in the patients without PH (p

BackgroundMacitentan 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.AimFew 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.MethodsThis 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.ResultsA 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.ConclusionsOur 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.

Objective This study aims to determine efficacy and safety of rapid left ventricular pacing (RLVP) during balloon aortic valvuloplasty and effect on development of postprocedural aortic insufficiency (AI) in children. Design This is a retrospective comparison of 56 children (mean age 18.3 month; 1 day–15 years of age) who underwent valvuloplasty by using RLVP with standard method (without pacing) during same time period (74 children; mean age 12.6 month; 1 day–18 years of age). Results The systolic valvular gradient decreased from a mean 67 ± 20.4 mm Hg (26–120 mm Hg) before the procedure to a mean 27.6 ± 17.8 mm Hg (0–120 mm Hg) after the procedure. Procedure failed in two of the patients (3.6%) who received the RLVP; only two patients developed severe AI. Among those patients who underwent the standard method procedure failed in 6 patients (8.3%), whereas severe AI was seen in 15 (20.2%). RLVP decreased the incidence of postprocedure severe AI significantly (P .05). RLVP did not change the procedure duration, but decreased the duration of fluoroscopy significantly (P

Summary– Muscular septal aneurysms are extremely rare without a ventricular septal defect and are diagnosed accidentally in most cases. Reported cases generally have rhythm disturbance or electrocardiographic findings of Wolf-Parkinson-White (WPW) syndrome. Presently described are 2 cases of ventricular septal aneurysm associated with WPW syndrome, which presented as dilated cardiomyopathy. Pre-excitation disappeared gradually in first patient. There was also concurrent decrease in degree of bulging of the interventricular septum and improvement of left ventricular systolic function. Second patient had complaints of palpitation and was referred for ablation of accessory pathway. Our findings suggest that presence of pre-excitation may lead to ventricular dyssynchrony and abnormal ventricular septal movement, resulting in appearance of aneurysm.

Background This study focuses on determining concomitant persistent left superior vena cava (SVC) in patients with congenital heart disease (CHD). Methods Between 2005 and 2012, a total of 2.663 patients with CHD, 88 (3.3%) of whom were diagnosed with persistent left SVC, were evaluated retrospectively. The demographic characteristics of patients, clinical and radiographic findings, echocardiography, cardiac catheterization, and angiography results obtained from the patients’ records were reviewed. Results The median age of the patients was 9.5 months, and 46 of the 88 (52.3%) patients were female. The most common concomitant CHD were ventricular septal defect, double outlet right ventricle (DORV), and tetralogy of Fallot (TOF). When the patients were compared according to their CHD, persistent left SVC frequency was significantly higher in those with DORV (P

Spontaneous echocardiographic contrast is defined as a phenomenon of discrete reflections appearing in the cardiac chambers or great vessels. It has been associated with several conditions leading to microbubble formation or low blood flow. This is a report of a neonate with spontaneous echocardiographic contrast related to patent ductus venosus.

Studies have shown that a prolonged P-wave dispersion is a risk factor for the development of atrial fibrillation. The aim of this study was to evaluate P-wave dispersion in adolescents with anorexia nervosa at diagnosis. We evaluated electrocardiographic findings, particularly the P-wave dispersion, at initial assessment in 47 adolescents with anorexia nervosa. Comparison of P-wave dispersion between adolescents with anorexia nervosa and controls showed a statistically significant higher P-wave dispersion in patients with anorexia nervosa (72 ± 16.3 msec) when compared to the control group (43.8 ± 9.5 msec). Percent of body weight lost, lower body mass index, and higher weight loss rate in the patients with anorexia nervosa had no effect on P-wave dispersion. Due to the fact that anorexia nervosa has a high mortality rate we believe that cardiac pathologies such as atrial fibrillation must also be considered in the medical evaluation.

Although radiofrequency ablation is the first line therapy in some children with supraventricular tachycardia, its application in small children is still limited. Herein, we presented a premature newborn diagnosed as multidrug-resistant permanent junctional reciprocal tachycardia, and treated by radiofrequency ablation via the jugular vein approach because of bilateral femoral vein thrombosis. We think that when there is limited vascular access, the transjugular route for radiofrequency ablation might be considered as an alternative treatment in newborns with multidrug-resistant supraventricular tachycardia.

Background Radiofrequency ablation (RFCA) became a treatment of choice in patients with recurrent ventricular tachycardia, ventricular fibrillation, and appropriate interventions of implanted cardioverter-defibrillator (ICD), however, electrical storm (ES) ablation in a pregnant woman has not yet been reported. Case presentation We describe a case of a successful rescue ablation of recurrent ES in a 26-year-old Caucasian woman during her first pregnancy (23rd week). The arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) was diagnosed 3 years earlier and several drugs as well as 2 ablations failed to control recurrences of ventricular tachycardia. RFCA was performed on the day of the third electric storm. The use of electroanatomic mapping allowed very low X-ray exposure, and after applications in the right ventricular outflow tract, arrhythmia disappeared. Three months after ablation, a healthy girl was delivered without any complications. During twelve-month follow-up there was no recurrence of ventricular tachycardia or ICD interventions. Conclusions This case documents the first successful RFCA during ES due to recurrent unstable ventricular arrhythmias in a patient with ARVD/C in pregnancy. Current guidelines recommend metoprolol, sotalol and intravenous amiodarone for prevention of recurrent ventricular tachycardia in pregnancy, however, RFCA should be considered as a therapeutic option in selected cases. The use of 3D navigating system and near zero X-ray approach is associated with minimal radiation exposure for mother and fetus as well as low risk of procedural complication.

Pericardial Effusion in Childhood: Experience from Tertiary Care Center in Ankara. Tamer Yoldas, Ozkan Kaya, Seyma Kayali, Ilker Ertu grul, Vehbi Do gan, Senem Ozgur, Utku Arman Orun, Selmin Karademir, Murat Koc. Department of Pediatric Cardiology, Dr. Sami Ulus Maternity and Children Research and Training Hospital; Department of Pediatric Cardiovascular Surgery, Dr. Sami Ulus Maternity and Children Research and Training

Effects of Supraventricular Tachycardia Ablation on Anxiety and Depression. Fatma Yigit, Ebru Altintas. Department of Cardiology, Baskent University Medical Faculty Adana Teaching and Reseach Center, Adana, Turkey; Department of Psychiatry, Baskent University Medical Faculty Adana Teaching and Reseach Center, Adana, Turkey. Backgrounds: Supraventricular tachycardia is a common rhythm disturbance. Radiofrequency catheter ablation is an effective and safety treatment choice in this arrhythmia. Depression and anxiety are often today. Palpitations may be symptom of the many mental and the phsycal disorders. The aim of this study is investigate to effects of radiofrequency catheter ablation on anxiety and depression in supraventricular tachycardia patients. Methods: Twenty-eight patients with palpitation that cardiac electrophysiology (EPS) was that planned were included to the study. Patients were evaluated by the same psychiatrist both just before EPS and after 1 month. Groups were compared with regard to socio-demographic characteristics, co morbid disorders, Beck Anxiety Inventory (BAI) and Beck Depression Inventory (BDI). Results: There were no statistically significant differences between the groups in terms of age, gender, marital status, occupation, family history for psychiatric and cardiac diseases, and concomitant physical diseases. After EPS, in 60.7% of patients was detected typical atrioventricular nodal reentrant tachycardia. Ablation success was in 91% of the patients. The most common mental disorder was anxiety disorder (41%, 14 patients). In 3 patients (10.7%) had depressive disorder. After EPS -1 month laterBAI score was significantly lower as compared with baseline BAI score. Whilst there was no significant difference in BDI score after EPS compared with baseline BDI score (table). Conclusions: The present study revealed that the anxiety level is decreasing in patients with palpitation after EPS with RF ablation, but not depression. Table. The Anxiety and Depression Scores Before and After Electrophysiology Study Score median (minimum-maximum) p BAI-1 20 (0-42) BAI-2 13.5 (1-36) 0.049 BDI-1 14 (1-25) BDI-2 12 (0-25) 0.23 BDI-1: Baseline Beck Depression Scale score; BDI-2: post-EPS Beck Depression Inventory score; BAI-1: Baseline Beck Anxiety Inventory score; BAI-2: post-EPS Beck Anxiety Inventory score. p1⁄4 Wilcoxson test (group comparison results of repeated measurements). Bold indicates statistically significant p-values.

The American Journal of Cardiology MARCH 26e29, 2015 11 IN AND C was attempted in 7 of the patients (2 teratoma, 1 papillerfibroelastoma, 1 fetal type rhabdomyoma, 1 TBS associated rhabdomyoma producing left ventricular outflow tract obstruction, 1 cystic tumor with granulomatous inflammation and 1 undiagnosed solid mass). Of the patients 3 died in perioperative period. Conclusion: Most of cardiac tumours during childhood are benign. Spontaneus regression is most probably possible in cases of TBS associated rhabdomyoma. Surgical intervention is only required for children who develop clinical symptoms due to hemodynamic instability.