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Emery-Dreifuss muscular dystrophy is one of a group of muscular dystrophies caused by a deficiency in genes encoding nuclear proteins (emerin, lamin A/C, nesprin). It progresses with joint contractures, muscular dystrophy, and cardiac involvement. Cardiac findings include dilated cardiomyopathy, conduction defects, and an associated increased risk of sudden cardiac death. We report the case of a young boy, aged 16, with lamin A/C gene mutation and dilated cardiomyopathy. From the patient's history, it was learned that his father and sister also had dilated cardiomyopathy and both died of heart failure. Cardiac resynchronization therapy implantation was planned in the follow-up of the patient due to progressive left ventricular dysfunction and left ventricular dyssynchrony. But the family did not accept this treatment option. The patient was placed on the heart transplant list. While waiting for a suitable donor, he died as a result of sudden cardiac arrest while he was being treated in the intensive care unit.

The most common pediatric arrhythmias are tachycardias, and the most common type is supraventricular tachycardia, originating from or above the atrioventricular node and HIS bundle. Ventricular tachycardias are less common but more dangerous. Supraventricular tachycardias usually cause a narrow complex tachycardia unless there is a basal bundle branch block or rate-dependent aberration. A wide QRS tachycardia should be treated as ventricular tachycardias unless proven to be an supraventricular tachycardia with aberration. Diagnosis of both tachyarrhythmia types depends mainly on 12-lead electrocardiography. The most common supraventricular tachycardia type in newborns and infants is atrioventricular reentry tachycardia, related to manifest or concealed accessory pathways and in adolescent atrioventricular nodal reentry tachycardia, whereas focal atrial tachycardias consist of 10%-15% of supraventricular tachycardias during all ages. Supraventricular tachycardias have a low risk of morbidity, and ablation therapy is successful in most types with success rates over 90%. Ventricular tachycardias can be monomorphic or polymorphic, nonsustained or sustained, and can cause more hemodynamic instability than supraventricular tachycardias, requiring more close monitoring and urgent therapies. If hemodynamically unstable, synchronized cardioversion must be performed. Polymorphic ventricular tachycardias are very dangerous and often associated with primary ion channel defects (channelopathies), which can cause sudden cardiac death.

In this study, we aimed to evaluate the clinical aspects, electrophysiological studies, and ablation results of permanent junctional reciprocating tachycardia in children.The study comprised 29 pediatric patients diagnosed with permanent junctional reciprocating tachycardia between 2011 and 2021 in 2 pediatric electrophysiology centers. From the file records, the basic demographic characteristics of the patients, as well as electrocardiographic and echocardiographic findings, were acquired retrospectively. The medical treatment and responses of the patients throughout follow-up, as well as the electrophysiological study and ablation data of the patients who had electrophysiological study, were assessed.The mean age at diagnosis of the patients was 3.13 ± 4.43 (0-18) years and the mean weight was 18.22 ± 19.68 (3.8-94) kg. Eighteen patients (62.1%) were girls. Eleven patients (38%) developed tachycardia-induced cardiomyopathy. Tachycardia was incessant in 15 patients (51.7%). In total, 22 patients required 26 ablation procedures. Tachycardia-induced cardiomyopathy and multidrug-resistant tachycardia were the most prevalent indications for ablation. The right posteroseptal pathway was detected in 18 patients (81.8%). The acute procedure success rate was 100% (22/22). The recurrence rate was 18% (4/22) and 3 of them underwent successful ablation again. The overall success percentage was 95.4% (21/22). None of the patients had any complications. The mean follow-up period was 4.39 ± 3.05 years.Although permanent junctional reciprocating tachycardia is uncommon, it is often persistent, resistant to medical treatment, and associated with a substantial risk of tachycardia-induced cardiomyopathy. Catheter ablation can be performed on these patients at any age, with minimal risk of complications and a high success rate. It is crucial to keep monitor of the patients' recurrence.

Objective Long QT syndrome (LQTS) is an inherited cardiac ion channel disorder (channelopathy) that is characterized by prolonged QT intervals on the electrocardiography (ECG) and possess the risk of sudden cardiac death (SCD). Jervell-Lange Nielsen syndrome (JLNS) is a specific subtype of LQTS that is accompanied by congenital sensorineural hearing loss, inherited autosomal recessively, and higher risk of SCD. In this study, we aimed to investigate JLNS prevalence in deaf children attending special schools for hearing loss, located in our province. Methods An ECG screening program was conducted in 6 special schools for children with hearing loss in Istanbul and a total of 440 students between 6 and 18 years old were included. Corrected QT interval (QTc) was calculated using the Bazett formula. Notably, 51 students, detected with any abnormal finding on ECG, were invited to our center for a comprehensive examination. Results A total of 8 patients were found with a prolonged QT interval. JLNS was diagnosed in 4 (0.9%) patients. In addition, 2 students had already been diagnosed with JLNS at another center earlier. The other 2 students, being siblings, were newly diagnosed with JLNS; and appropriate treatment was initiated. Genetic testing revealed a pathological homozygous mutation in KCNQ1 gene. The younger sibling (Case 1), who possessed a QTc of greater than 500 ms and a history of syncope, which was very suspicious for SCD, was implanted an implantable cardioverter-defibrillator. Propranolol treatment was initiated for both siblings. Conclusion JLNS should be carefully considered and screened, especially in patients with a history of congenital deafness.

Background This study aims to evaluate early postoperative arrhythmias in children undergoing congenital cardiac surgery. Methods A total of 670 pediatric patients (355 males, 315 females; median age: 4 months; range, 1 day to 18 years) who underwent cardiac surgery due to congenital heart defects between December 2018 and November 2019 were included. The rate of postoperative arrhythmias, diagnosis, potential risk factors, and management strategies were evaluated. Multivariate regression analysis was used to identify significant factors of development of postoperative arrhythmias. Results Tachyarrhythmia was detected in 54 patients (8.1%), and the most common tachyarrhythmia was junctional ectopic tachycardia. Medical treatment was required in 25/38 (66%) of junctional ectopic tachycardia patients. Amiodarone was initiated in 18, dexmedetomidine in five, and flecainide + amiodarone in two of the patients. Different degrees of atrioventricular block were observed in 30 patients (4.5%). In 12 patients, permanent pacemakers were implanted during hospitalization. Age at the time of surgery under one-year-old, high inotropic scores, prolonged operation time, and high Aristotele"s scores were independent risk factors associated with early postoperative arrhythmia (p

Objectives:In recent years, cardiac resynchronization therapy (CRT) has also started to be performed in the paediatric and CHD population. This study aimed to evaluate the efficacy of CRT in children with CHD.Patients and methods:Patients with CHD who underwent CRT treatment in our paediatric cardiology clinic between January, 2010 and January, 2020 were included in the study. Demographic findings, 12-lead electrocardiograms, echocardiograms, clinical characteristics, management strategies, and outcomes were reviewed systematically.Results:The study population consisted of 18 CHD patients who had been treated with CRT for 10 years in our institution. The median age was 11 years (2.2–18 years) and the median weight was 39 kg (10–81 kg). Systemic ventricle was left ventricle in 13 patients, right ventricle in 4 patients, and 1 patient had single-ventricle physiology. CRT implantation indications were as follows: dysfunction after permanent pacemaker in 11 patients, dysfunction after left bundle branch block in 4 patients, and systemic ventricular dysfunction in 3 patients. CRT implantation techniques were epicardial (n = 13), hybrid (n = 4), and transvenous (n = 1) methods. QRS duration significantly decreased after CRT implantation (160 versus 124 m/second, p < 0.05). Median systemic ventricle ejection fraction (EF) significantly increased after the procedure (30 versus 50%, p < 0.05). Fourteen patients (78%) were responders, two patients (11%) were superresponders, and two patients (11%) were non-responders after the CRT treatment. One patient deceased during follow-up. Median follow-up duration was 40 months (6–117 months).Conclusion:When electromechanical dyssynchrony occurs in paediatric cases with CHD and developing heart failure, patients should be evaluated in terms of CRT to improve ventricular function. Alternative CRT therapy will be beneficial in these cases that do not improve clinically despite optimal medical treatment.

Background Hypertrophic cardiomyopathy (HCM) is the second most common pediatric cardiomyopathy. Although there is a large body of literature about HCM in adults, there is limited information on HCM in childhood. We evaluated various aspects of pediatric HCM patients treated at our center. Methods We identified 152 pediatric patients with HCM between October 2011 and October 2019. Clinical history, invasive (ICD, pacemaker, electrophysiologic study, catheter ablation therapy) and non-invasive (ECG, holter moniterization, echocardiography, cardiac MR, genetic study, medicam treatment) data were collected and evaluated. Results The mean ± standard deviation age of patients was 8.9 ± 5.7 years (1 month-18 years) and 67.8% were male. The most frequent clinical symptoms were murmur and palpitations. Three cases (2%) had aborted sudden death as the first manifestation of HCM. Of these patients, 120 (78.9%) had non-syndromic HCM and 32 (27.2%) had syndromic HCM. Asymmetric septal hypertrophy was common (48.3%) in the non-syndromic group, whereas concentric hypertrophy was common (56.2%) in syndromic group. Left ventricular outflow tract obstruction (LVOTO) occurred in 39 (25.6%) patients. Nine (5.9%) patients underwent electrophysiologic study and/or ablation and 16 patients underwent surgical intervention. Implantable cardioverter defibrillator (ICD) insertion was performed in 38 patients (26 transvenous, 12 epicardial). ICDs were inserted in three (7.9%) patients for secondary prevention; in the remaining patients (92.1%) the devices were placed for primary prevention. Mean SD follow-up time was 27.1 ± 22 months. Five (3.3%) patients died during the follow-up. No patient had heart transplantation or a long-term assistive device. Conclusion The etiology of HCM is heterogeneous and present at any age. It is important to determine the timing of surgery and potential risks for sudden cardiac arrest. As most cases of HCM are familial, evaluation of family members at risk should be a routine component of clinical management.

BACKGROUND Permanent pacemaker (PM) implantation is performed for various indications and by different techniques in children; however, many problems with lead performance are encountered during follow-up. This study aims to evaluate the possible effects of different lead types and implantation techniques on pacing at early and midterm in children with a permanent PM. PATIENTS AND METHODS Pediatric patients who underwent permanent PM system implantation at our tertiary cardiac surgery center between January 1, 2010 and January 1, 2020 were evaluated retrospectively. Patients were categorized in the epicardial pacing lead (EP), transvenous pacing lead (TP), and transvenous bipolar lumenless (Select Secure [SS]) lead groups according to the lead implantation technique and lead type with the same manufacturer. Groups were evaluated statistically for demographic features, pacing type and indication for implantation, lead electrical performance, lead failure, complications, and outcome. RESULTS Over 10 years, 323 lead implantations were performed on 167 patients (96 males, median age 68 months [5 days-18 years]). Of 323 leads, 213 (66%) were EP, 64 (20%) were TP, and 46 (14%) were SS. Of the total, 136 of the leads were implanted in atria, and 187 were implanted in ventricles. Primary pacing indications were postoperative complete atrioventricular (AV) block (n = 95), congenital AV block (n = 71), sinus node dysfunction (n = 13), and acquired complete AV block (n = 1). Additional cardiac diseases were present in 115 patients (69%). No statistically significant difference was observed in gender, syndrome, or pacing indication (P > .05). Atrial and ventricular capture, threshold, sensing, and lead impedance measurements were not significantly different at the initial and follow-up periods (P > .05). The median follow-up duration was 3.3 years (6 months-10 years). Twenty lead failures were determined in 15 patients (EP: 14 lead failures in 10 patients; TP: two lead failures in two patients; and SS: four lead failures in three patients) during follow-up, and no statistically significant difference was found between groups (P = .466). The 5-year lead survival was 98% for TP, 95% for EP, and 90% for SS; the 10-year lead survival was 90% for TP, 70% for EP, and 70% for SS. There was no mortality related to chronic pacing or due to the procedure of implantation. CONCLUSIONS Despite improvements in technology, lead failure is still one of the most critical problems during these patients' follow-up. Early to midterm lead survival rates of all three lead types were satisfactory.

Objective: The aim of this study is to present electrophysiologic characteristics and catheter ablation results of tachycardia-induced cardiomyopathy (TIC) in children with structurally normal heart. Methods: We performed a single-center retrospective review of all pediatric patients with TIC, who underwent an electrophysiology study and ablation procedure in our clinic between November 2013 and January 2019. Results: A total of 26 patients, 24 patients with single tachyarrhythmia substrates and two patients each with two tachyarrhythmia substrates, resulting with a total of 28 tachyarrhythmia substrates, underwent ablation for TIC. The median age was 60 months (2–214 months). Final diagnoses were supraventricular tachycardia (SVT) in 24 patients and ventricular tachycardia (VT) in two patients. The most common SVT mechanisms were focal atrial tachycardia (31%), atrioventricular reentrant tachycardia (27%), and permanent junctional reciprocating tachycardia (15%). Radiofrequency ablation (RFA) was performed in 15 tachyarrhythmia substrates, and cryoablation was performed in 13 tachyarrhythmia substrates, as the initial ablation method. Acute success in ablation was achieved in 24 out of 26 patients (92%). Tachycardia recurrence was observed in two patients (8%) on follow-up, who were treated successfully with repeated RFA later on. Overall success rates were 92% (24 out of 26) in patients and 93% (26 out of 28) in substrates. On echocardiography controls, the median left ventricular recovery time was 3 months (1–24 months), and median reversible remodeling time was 6 months (3–36 months). Conclusion: TIC should be kept in mind during differential diagnosis of dilated cardiomyopathy. Pediatric TIC patients can be treated successfully and safely with RFA or cryoablation. With an early diagnosis of TIC and quick restoration of the normal sinus rythm, left ventricular recovery, and remodeling may be facilitated. (Anatol J Cardiol 2020; 24: 370-6)

Radiofrequency catheter ablation (RFCA) procedure is performed for many tachyarrhythmias. We performed successful RFCA in a 5-year-old child for supraventricular tachyarrhythmia and Wolff-Parkinson-White syndrome. Acute circumflex artery (CxA) occlusion occurred due to RFCA. After percutaneous balloon angioplasty was performed into the CxA, the patient was treated with systemic steroid to resolve myocardial edema. To the best of our knowledge, systemic steroid was used first time for acute coronary artery injury related myocardial ischemia.

We report a patient with long QT syndrome who received an inappropriate implantable cardioverter-defibrillator shock due to electrical interference from a refrigerator. This electrical interference was mistakenly detected as an episode of ventricular fibrillation and ended with an inappropriate delivery of shock without any warning symptoms before.

Objective:This study demonstrates the clinical and electrophysiological details of catheter ablation conducted in children with focal atrial tachycardia using three-dimensional electroanatomic mapping systems.Patients and methods:Electrophysiological procedures were performed using the EnSite™ system.Results:Between 2014 and 2020, 60 children (median age 12.01 years [16 days–18 years]; median weight 41.5 kg [3–98 kg]) with focal atrial tachycardia and treated with catheter ablation were evaluated retrospectively. Tachycardia-induced cardiomyopathy was developed in 15 patients (25%). Most of the focal atrial tachycardia foci were right-sided (75%), and more than one focus was found in four patients. Radiofrequency ablation was performed in 47 patients (irrigated radiofrequency ablation in seven cases), cryoablation in 9, and radiofrequency ablation and cryoablation in the same session in 4 patients. The median procedural time was 163.5 minutes (82–473 minutes). Fluoroscopy was used in 29 of (48.3%) patients (especially for left-side substrate) with a mean time of 8.6 ± 6.2 minutes. The acute success rate was 95%. The procedure failed in three patients, and recurrence was observed in 3.5% of patients (2/57) during a median follow-up of 17 months (2–69 months). The second ablation was performed in four cases, of which three were successful. Overall success rate was 96.6% with no major complications observed, except in one patient with minimal pericardial effusion.Conclusion:Catheter ablation seems to be an effective and safe treatment in focal atrial tachycardia. Electroanatomic mapping system can facilitate the ablation procedure and minimise radiation exposure.

Cardiac pacemakers have improved patient survival and quality of life, though malfunctions can be seen. We present the case of a girl with Seckel syndrome and congenital complete heart block. She had a single chamber permanent pacemaker in the right ventricle. When she referred us with a pulmonary hypertensive crisis (PHC), it was seen that the device was not pacing even in maximum treshold and pulse width values. After new epicardial lead implantation into the left ventricular apex, capture could be established again. For the cases presenting with capture failure, after eliminating lead-related problems and biochemical abnormalities, PHC should be kept in mind as a reason. This article is protected by copyright. All rights reserved.

Aortic cusps might be the source of supraventricular or ventricular arrhythmias. For many years, aortic cusp ablation has been widely used to treat premature ventricular contractions (PVCs) and ventricular tachycardia (VT). However, the data on the outcomes of this procedure in children are limited. The study aimed to convey or describe our own aortic cusp ablation experiences in children and, thus, contribute to the literature. The focus was pediatric cases of ventricular arrhythmia in which the origin of the PVCs was ablated above the Valsalva. The sample comprised patients who underwent aortic cusp ablation between 2013 and 2018. The demographic characteristics, noninvasive test results, procedure details, and follow-up results for the patients were noted. The 3D EnSite Precision cardiac mapping system and limited fluoroscopy were used. A total of 26 procedures were performed on 22 patients. The mean age was 14.4 ± 3.0 (9–19) years, and the mean weight was 57.3 ± 17.5 (27–99) kg. The mean follow-up period after the first presentation was 38.6 ± 22.9 (3–72) months. There were significant differences in the values of the transition index, V2S/V3R, IIR/IIIR, aVRS/aVLS ratio, and QRS polarity in I at various locations. The most common ablation site was the left coronary cusp (LCC). Radio frequency (RF) ablation, cryoablation, and irrigated RF ablation were found to be effective energy sources in 15, 4, and 3 patients, respectively. Patients who underwent ablation at the LCC–right coronary cusp (RCC) commissure were more likely to present with only VT and to experience worsening symptoms. Twelve patients had previously undergone ablation of the right ventricular outflow tract (RVOT). Ablation in the RVOT had been previously performed in all the patients who eventually underwent ablation at the RCC and the LCC–RCC commissure. Aortic cusp ablation can be safely performed in children. The careful evaluation of previous noninvasive tests provides important data for determining the location. There might be significant differences in the signs and requirements on the basis of the locations during mapping and ablation.

OBJECTIVE Pediatric ventricular tachycardias (VTs) have heterogeneous etiology and different clinical features. This study aimed to evaluate the clinical spectrum and long-term course of pediatric sustained VTs. METHODS Patients diagnosed as having sustained VT between 2010 and 2020 were evaluated retrospectively. RESULTS A total of 129 patients with VT were evaluated; 74 patients were male, and the median age was 12.5 years (0.25-18 years). Patients were grouped as having idiopathic VT (IVT) [n=85 (65.9%)], cardiomyopathy-associated VT (CMP-VT) [n=24 (18.6%)], catecholaminergic polymorphic VT [n=17 (13.2%)], and myocarditis-associated VT [n=3, (2.3%)]. Palpitations (n=61) and syncope (n=24) were the most common symptoms. VT originated from the right ventricle in 53.6% of the patients. Half of the patients underwent electrophysiological study, 64 patients received radiofrequency ablation therapy, and 29 patients had implantable cardiac defibrillators. During the follow-up, 70.4% of all patients had complete resolution, whereas 19 patients had a partial resolution and 23 patients (19.5%) had stable disease. Monomorphic VTs and VTs with left bundle bunch block were more thriving controlled (p=0.02 vs. p=0.04). In terms of long-term results, no statistical difference was found among the VT groups (p=0.39). Deaths were observed only in IVT (n=1) and CMP-VT (n=8) groups (p

Multidisipliner bir alan olan adli bilimler, adaletin toplum ve toplumu olusturan bireyler arasinda esit olarak saglanabilirligine hizmet eden saglik, fen ve sosyal bilimler gibi egitsel, teknolojik ve pozitif bilimler toplulugunu kapsar. Bu bilimsel alanda teorik ve uygulamanin birbirleriyle ortusen ve ortusmeyen bircok noktasi bulunmaktadir. Teorik bilgilerin havada ucustugu cagimizda bu bilgilerin adli bilimlerin uygulama alanina yansimasi cok onemlidir. Adli tip, hukuk, psikoloji, sosyal hizmet, cocuk gelisimi, kriminoloji, antropoloji, ogretmenlik, medya, polis ve jandarma gibi guvenlik birimlerini de iceren toplumun cok genis bir alaninda birlikte calismaya ve uretmeye gereksinim bulunuyor. Egitim ve ogretimin en onemli parcasini deneyimlerimiz ve uygulama alanlari yani “olgu temelli” yaklasim belirliyor. Olgu, bilgiyi dogru kullanim, gelisim ve degisimin temeli olma yaninda egitim ve ogretimin ayrilmaz bir parcasidir. Bu paradigma ile elinizde tutugunuz “Olgularla Adli Tip & Adli Bilimler” kitabinin tip fakultesi ogrencileri, adli tip asistanlari, adli tip uzmanlari, adli bilimlerin farkli alanlarinda lisans, yuksek lisans ve doktora ogrencileri basta olmak uzere tip, hukuk, psikoloji, dis hekimligi, kriminoloji, sosyal hizmet, antropolojigibi cok cesitli adli bilimler alaninda calisanlara en son guncel literatur bilgileri yaninda olgularla yeni perspektifler de kazandiracagi kanisindayim. Kitabin okuyucuya multidisipliner bir yaklasim yaninda evrensel etik ilkelere de bagli bir adli tip/adli bilimler yaklasimi kazandirmasini diliyorum. Kitabin yaziminda katkilari olan bolum yazari hocalarimiza, meslektaslarimiza, asistanlarimiza, ogrencilerimize ve destek ve katkilari icin Akademisyen Yayinevi sahibi Yasin Dilmen’e sonsuz tesekkur ediyorum. Bilimselligin ve liyakatin gelecegin adli bilimlerini insa etmede tek gercek oldugu bilinciyle kitabin alanimizin bilimsel niteligini daha da yukseklere tasinmasina aracilik etmesi dilegiyle… Prof. Dr. Halis DOKGOZ Mersin, 2020

Known as a benign arrhythmia and normally requiring no specific treatment, accelerated idioventricular rhythm can rarely degenerate to a life-threatening arrhythmia. Here, we present a child with left coronary cusp-originating accelerated idioventricular rhythm, degenerating into torsades de pointes and resulting in cardiac arrest, which was ablated with a cryocatheter. An 11-year-old boy, followed due to asymptomatic accelerated idioventricular rhythm before, was referred to our department because he had experienced an aborted cardiac arrest during sleep. He had been resuscitated for 5 minutes. Twenty-four-hour Holter-ECG revealed incessant accelerated idioventricular rhythm, consisting up to 90% of the whole record and two torsades de pointes attacks, triggered by accelerated idioventricular rhythm-induced “R on T” phenomenon, and resulting in syncope and cardiac arrest. Transthoracic echocardiography revealed no structural cardiac defect but mild left ventricular systolic dysfunction with an ejection fraction of 45% and shortening fraction 23%. An electrophysiologic study was conducted, and accelerated idioventricular rhythm focus was mapped to left aortic coronary cusp. A cryocatheter with an 8-mm tip was preferred for successful ablation of the accelerated idioventricular rhythm focus, due to close neighbourhood to coronary ostium. The patient was discharged in 3 days without any premature ventricular contractions or accelerated idioventricular rhythm and with normalised cardiac functions. After 9 months on follow-up, he was still asymptomatic, without any premature ventricular contractions or accelerated idioventricular rhythm and with normal cardiac functions. Although the clinical course of accelerated idioventricular rhythm is known as benign, accelerated idioventricular rhythm can rarely degenerate to a life-threatening arrhythmia. In such cases, electrophysiologic study and catheter ablation are a good option in such cases with accelerated idioventricular rhythm for an ultimate cure.

Early repolarization (ER) is a benign electrographic pattern and described as J-point elevation ≥1 mm in two or more adjacent inferior and/or lateral derivations, resulting in QRS slurring or notching (1-3). Some researchs have shown that ER might be one of the arrhythmogenic reasons for idiopathic ventricular fibrillation (VF) (1, 3). Here we present the case of a boy with inferolateral ER complicated by an electrical storm and treated with orciprenaline and radiofrequency ablation of the Purkinje network.

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmic disorder characterized by exercise-induced ventricular arrhythmias and risk for sudden cardiac death. An exercise stress test is a standard method for diagnosis of CPVT, characterized by typical bidirectional polymorphic ventricular tachycardia. In patients who are not suitable for an exercise stress test, epinephrine stress testing (epinephrine challenge) can be used instead. There are a few reports in the literature discussing supraventricular tachycardias during exercise testing in pediatric CPVT patients. We present a 6-year-old boy who was admitted with syncopy. Epinehrine stress testing was performed according to the Ackerman protocol and revealed different types of tachycardias., Katekolaminerjik polimorfik ventriküler taşikardi (CPVT), egzersize bağlı ventriküler aritmi ve ani kardiyak ölüm riski ile karakterize kalıtımsal bir aritmik hastalıktır. Egzersiz stres testi, tipik çift yönlü polimorfik ventriküler taşikardilerle sonuçlanan CPVT tanısında standart bir yöntemdir. Egzersiz stres testi için uygun olmayan hastalarda, adrenalin stres testi kullanılabilir. Literatürde pediatrik CPVT hastalarında egzersiz testi sırasında supraventriküler taşikardilerle ilgili az sayıda rapor vardır. Bu olgu sunumunda senkop şikayeti ile başvuran altı yaşında erkek çocuk hastada Ackerman protokolüne göre uygulanan adrenalin stres testiyle ortaya çıkan farklı taşikardi türlerini paylaştık.

In children with typical atrioventricular nodal reentrant tachycardia (tAVNRT), cryoablation is preferred over radiofrequency ablation (RFA) because of its safety profile and acceptable long-term success rates. In this study, we have assessed the utility of 8-mm tip cryocatheters for tAVNRT ablation in our center. All pediatric AVNRT patients who underwent cryoablation with an 8-mm tip cryocatheter in our center between 2013 and 2018 were included. EnSite™ (St. Jude Medical Inc., St. Paul, MN, USA) was used in all patients. A total of 120 patients (64 females, 53%) were included in this study, and the mean age was 13.9 years with a standard deviation of 2.5 years. Eleven patients (9.1%) had structural heart disease, and 12 patients (10%) had additional arrhythmia substrate. The mean number of effective cryolesions was 8 with a standard deviation of 2.3. Fluoroscopy was used in three patients (2.5%). There were minor complications in only four patients (3.3%)—transient first-degree atrioventricular block or transient incomplete right bundle branch block. Acute success rate of cryoablation was 108/120 (90%). In twelve patients, cryoablation was suboptimal, or it failed. The procedure was completed successfully with RFA in the same session in ten patients. Overall acute success rate of ablation (Cryo ± RFA) was 98.5%. During a mean follow-up period of 24.6 months with a standard deviation of 11.3 months, three patients had recurrence (2.5%). Time between the beginning of the effective cryolesion and termination of AVNRT was found associated with acute success of cryoablation (p = 0.013). Cryoablation of AVNRT with an 8-mm tip catheter in children appears to be safe, with an acceptable acute success rate and a low recurrence rate. A faster termination of AVNRT during the cryolesion, slowing down before ending with atrioventricular block, is a good indicator for acute success

Objective Arrhythmogenic right ventricular dysplasia (ARVD) is a myocardial genetic disease that occurs primarily in the right ventricle. Patients with ARVD may present with severe ventricular arrhythmias, syncope, and cardiac arrest. The purpose of this study is to evaluate the clinical features and arrhythmic complications of patients with pediatric-onset ARVD. Methods Patients diagnosed with ARVD between January 2010 and January 2019 were included in this study. Results A total of 19 patients with ARVD were evaluated. Of them, 15 patients were male, and their mean age was 12±4 years. The most common symptoms were palpitations (n=6), syncope (n=4), and heart failure symptoms (n=2). Five patients were asymptomatic. Thirteen patients had an epsilon wave; all patients ≥14 years had a T wave inversion in V1-3. Premature ventricular contractions (PVCs) were observed in 15 patients, and ventricular tachycardia (VT) was observed in 9 patients. All patients underwent cardiac magnetic resonance imaging (MRI). Echocardiography and cardiac MRI of two patients were normal at the time of admission; patients were in the concealed phase, and the diagnosis was made by ECG, Holter monitoring, and genetic findings. We administered a beta-blocker in all patients. Two patients underwent an electrophysiological study and ablation because of PVC/VT. An implantable cardiac defibrillator was implanted in 8 patients. The mean follow-up period was 21.5±11 months. Two patients were deceased with incessant VT and heart failure, and one patient was deceased with multiorgan dysfunction after biventricular assist device implantation (n=3). Conclusion Diagnosis of pediatric-onset ARVD might be much more difficult in children. Sudden cardiac death might be prevented in the early period by raising the awareness of physicians about the disorder. Prevention of sudden death with implantable cardiac defibrillators is crucial in the management of these patients. It should be kept in mind that children with structurally normal hearts may present with an earlier concealed phase and can be diagnosed with ARVD.

Catheter ablation via the femoral vein has been widely used in children. However, in certain conditions, an alternative vascular access is required for a successful ablation. Herein we reported that, accessory pathways (APs) and ectopic foci which reside right anterior and anterolateral to tricuspid valve orifice can be safely and effectively ablated with transjugular venous approach. Eleven procedures performed via the transjugular venous approach were reviewed retrospectively from the 355 electrophysiological procedures performed between March 2016 and November 2017. EnSite 3D electro-anatomic mapping and limited flouroscopy was used in all patients. The mean age of patients was 14.4 ± 2.9 years (12–18) and 6/11 (54.5%) were males. Seven of the patients had previous ablation procedures via the femoral vein approach and due to failure or recurrence the procedures had to be repeated. In these patients mean procedure time shortened from 196 ± 80 (105–280) to 111 ± 13 (96–125) min with the transjugular approach. 10/11 patients had APs, and one patient had focal atrial tachycardia. One of the APs was a Mahaim pathway. Ablation localisations were right anterior and right anterolateral in 8/11 and 3/11 patients respectively. The average fluoroscopy time was 3.38 ± 6.5 (0–15) min. All of the RF ablations via transjugular approach were successful (100%, 11/11). The mean follow-up period was 10.6 ± 1.1 (9–12) months. So far no recurrence has been observed. Right anterior and anterolateral regions are the most challenging areas in terms of catheter stabilization even when long sheaths are used during femoral route ablation. Ablation attempts in these regions have partial success, frequent recurrence, and high complication rates. This study revealed that transjugular approach seems as an effective alternative for those cases where transfemoral approach is unsuccessful for the ablation targets located in the right anterior and antero-lateral regions.

AIM Our aim was to evaluate the findings and the role of intraoperative epicardial echocardiography (IEE) in the management of pediatric cardiac surgery patients. METHODS Patients evaluated with IEE between December 2015 and December 2017 were analyzed retrospectively. Demographic data, preoperative transthoracic echocardiography (TTE), and IEE reports were evaluated. RESULTS A total of 410 patients evaluated by IEE were included in the study. Of these, 52% were women, and 48% were men. The median age was 8.5 months (range: 1 month-7 years), and median body weight was 7.1 kg (range: 3.3-61 kg). The most common diagnoses were tetralogy of Fallot (TOF; n = 148), ventricular septal defect (VSD; n = 117), atrial septal defect (ASD; n = 57), and complete atrioventricular septal defect (AVSD; n = 48). There were minor residual lesions not requiring reestablishment of cardiopulmonary bypass (CPB) in 16.6% (n = 68), while major residual lesions requiring return to CPB were determined in 5.1% (n = 21). Major residual lesions were detected in 7 patients with TOF (4 severe right ventricular outflow tract obstructions, 2 pulmonary artery stenosis, 1 residual VSD shunt), 6 patients with VSD (hemodynamically significant residual shunts), and 5 patients with complete AVSD (3 left atrioventricular valve regurgitations, 1 right atrioventricular valve regurgitation, 1 left ventricular outflow tract obstruction). Transient bradycardia was observed in 5 patients. CONCLUSION Intraoperative epicardial echocardiography provides good guidance during congenital heart surgery. IEE helps to clarify the surgical planning and decreases morbidity and mortality due to unnecessary invasive procedures, especially for pathologies involving the pulmonary artery and its branches, as well as for apical ventricular septal defects.