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BACKGROUND: T-wave memory (TWM) is a rare cause of T-wave inversion (TWI). Alterations in ventricular activation due to abnormal depolarization may cause repolarization abnormalities on the ECG, even if myocardial conduction returns to normal. These repolarization changes are defined as TWM. In our study, we aimed to determine the frequency of TWM development and the predictors affecting it in the pediatric population who underwent accessory pathway (AP) ablation due to Wolff-Parkinson-White (WPW) syndrome. METHODS: The data of patients with manifest AP who underwent electrophysiological studies and ablation between 2015 and 2021 were retrospectively analyzed. The study included 180 patients who were under 21 years of age and had at least one year of follow-up after ablation. Patients with structural heart disease, intermittent WPWs, recurrent ablation, other arrhythmia substrates, and those with less than one-year follow-up were excluded from the study. The ECG data of the patients before the procedure, in the first 24 h after the procedure, three months, and in the first year were recorded. The standard ablation technique was used in all patients. RESULTS: Postprocedure TWM was observed in 116 (64.4%) patients. Ninety-three patients (51.7%) had a right-sided AP, and 87 patients (48.3%) had a left-sided AP. The presence of posteroseptal AP was found to be significantly higher in the group that developed TWM. Of these patients, 107 (93.1%) patients showed improvement at the end of the first year. Preprocedural absolute QRS-T angle, postprocedural PR interval, and right posteroseptal pathway location were identified as predictors of TWM. CONCLUSION: The development of TWM is particularly associated with the right-sided pathway location, especially the right posteroseptal pathway location. The predictors of TWM are the preprocedural QRS-T angle, the postprocedural PR interval, and the presence of the right posteroseptal AP.
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Fascículo Atrioventricular Accesorio , Ablación por Catéter , Electrocardiografía , Síndrome de Wolff-Parkinson-White , Humanos , Síndrome de Wolff-Parkinson-White/cirugía , Síndrome de Wolff-Parkinson-White/fisiopatología , Masculino , Femenino , Estudios Retrospectivos , Ablación por Catéter/métodos , Fascículo Atrioventricular Accesorio/cirugía , Fascículo Atrioventricular Accesorio/fisiopatología , Adolescente , Niño , Adulto JovenRESUMEN
Despite the advancements in technology, establishing the optimal implantation technique for pediatric patients with a pacemaker (PM) indication remains challenging. Although the implantation of an epicardial PM is recommended, especially in children weighing less than 10 kg, transventricular placement of endocardial leads can be performed safely, offering a practical substitute for an epicardial pacing system, particularly in situations where a transvenous approach is unfeasible due to patient size, anatomical constraints or epicardial PM leads were not available as in our case.
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Twelve-lead ECG of a child with Wolff-Parkinson-White syndrome and systolic dysfunction was consistent with the anteroseptal accessory pathway. The earliest atrial activation during electrophysiological study was found between the right anteroseptal region near the HIS. Multiple femoral tract right-sided cryotest lesions followed by radiofrequency catheter irrigated through the jugular vein route were unsuccessful. Then, non-coronary aortic cusp mapping and cryoablation were successfully performed with a retroartic approach.
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Ablación por Catéter , Síndrome de Wolff-Parkinson-White , Humanos , Niño , Síndrome de Wolff-Parkinson-White/cirugía , Atrios Cardíacos/cirugía , Electrocardiografía , Aorta/cirugíaRESUMEN
We report on a 12-month-old boy with an ectopic atrial tachycardia successfully treated with the ivabradine that acts on cardiac pacemaker cells by selectively inhibiting the If channel. The patient was diagnosed with supraventricular tachycardia in another centre, and multi-drug therapy was unsuccessful to restore sinus ryhthm, so he was sent to our hospital for catheter ablation. We stopped the medications the patient was taking and started using ivabradine. Sinus rhythm was restored 2 hours after ivabradine treatment was started.
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Ablación por Catéter , Taquicardia Atrial Ectópica , Taquicardia Supraventricular , Masculino , Humanos , Lactante , Ivabradina/uso terapéutico , Taquicardia Atrial Ectópica/diagnóstico , Taquicardia Atrial Ectópica/tratamiento farmacológico , Taquicardia Atrial Ectópica/cirugía , Electrocardiografía , Taquicardia Supraventricular/cirugía , Arritmias Cardíacas/tratamiento farmacológicoRESUMEN
We present two exceptional cases of 14-year-old girls diagnosed with rare cardiomyopathies (left ventricular non-compaction, and arrhythmogenic right ventricular cardiomyopathy), both presenting with the unusual finding of bidirectional ventricular tachycardia.
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Displasia Ventricular Derecha Arritmogénica , Cardiomiopatías , Taquicardia Ventricular , Femenino , Humanos , Niño , Adolescente , Taquicardia , Cardiomiopatías/complicaciones , Cardiomiopatías/diagnóstico , Ventrículos Cardíacos , Displasia Ventricular Derecha Arritmogénica/complicaciones , Displasia Ventricular Derecha Arritmogénica/diagnóstico , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiologíaRESUMEN
BACKGROUND: Total abnormal pulmonary venous return anomaly is a CHD characterised by abnormal pulmonary venous flow directed to the right atrium. In this study, we aimed to compare the effects of these techniques on early rhythm problems in total abnormal pulmonary venous return anomaly cases operated with conventional or primary sutureless techniques. METHOD: Seventy consecutive cases (median age 1 month, median weight 4 kg) who underwent total abnormal pulmonary venous return anomaly repair with conventional or primary sutureless technique between May 1 2020 and May 1 2022 were evaluated. The rate, diagnosis, and possible risk factors of postoperative arrhythmias were investigated. The results were evaluated statistically. RESULTS: When the total abnormal pulmonary venous return anomaly subgroup of 70 cases was evaluated, 40 cases were supracardiac, 18 cases were infracardiac, 7 cases were cardiac, and 5 cases were mixed type. Twenty-eight (40%) cases had a pulmonary venous obstruction. Primary sutureless technique (57%, supracardiac n = 24, mixed = 3, infracardiac = 13) was used in 40 patients. Median cardiopulmonary bypass time (110 versus 95 minutes) and median aortic clamp time (70 versus 60 minutes), median peak lactate (4.7 versus 4.8 mmol/l) in the first 72 hours, and median peak vasoactive inotropic score in the first 72 hours of the primary sutureless and conventional technique used cases value (8 versus 10) were similar. The total incidence of arrhythmias in the conventional group was significantly higher than in the primary sutureless group (46.7% versus 22.5%, p = 0.04). Supraventricular early beat was observed in 3 (7.5%), sinus tachycardia was seen in 6 (15%), junctional ectopic tachycardia was seen in 1 (2.5%), intra-atrial reentry tachycardia was seen in 1 (2.5%), usual supraventricular tachyarrhythmia was seen in 2 cases (5%) in the primary sutureless group. In the conventional group, supraventricular early beat was observed in six of the cases (20%), sinus tachycardia in five (16.7%), junctional ectopic tachycardia in four (13.3%), intra-atrial reentry tachycardia (10%) in three, and supraventricular tachyarrhythmia in seven cases (23.3%). In the first 30 days, there was a similar mortality rate (10% versus 10%), with four patients in the primary sutureless group and three in the conventional group. The median follow-up period of the cases was 8 months (interquartile range (IQR) 6-10 months). In the follow-up, arrhythmias were detected in two cases (one supraventricular tachyarrhythmia and one intra-atrial reentry tachycardia) in the primary sutureless group and three cases (two supraventricular tachyarrhythmia, one intra-atrial reentry tachycardia) in the conventional technique. All cases were converted to normal sinus rhythm with cardioversion and combined antiarrhythmic therapy. CONCLUSION: Different arrhythmias can be observed in the early period in patients with operated total abnormal pulmonary venous return anomaly. Although a higher rate of rhythm problems was observed in the early period in the conventional method compared to the primary sutureless technique, no significant effect was found on mortality and morbidity between the groups.
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Síndrome de Cimitarra , Taquicardia Ectópica de Unión , Humanos , Lactante , Síndrome de Cimitarra/complicaciones , Síndrome de Cimitarra/cirugía , Taquicardia Sinusal , Arritmias Cardíacas/etiología , Atrios CardíacosRESUMEN
Prolonged pleural effusion is a fairly common condition which has considerable impact on complicated and longer hospital stays after Fontan surgery. Identifying the patient population prone to have pleural effusions is still seeking for an answer. This study is to determine the variables that may predict prolonged pleural effusion according to the data of 69 patients who underwent Fontan operation between June 2018 and December 2020 and survived to date. Prolonged pleural effusion was defined as the need for a chest tube for more than 7 days. Two patient groups, with and without prolonged effusion, were compared in terms of pre-, peri-, and post-operative variables. The patients were subdivided into "high-risk" and "low-risk" groups based on the pre-operative catheterisation data. The most frequent main diagnosis was tricuspid atresia (n: 13, 19%). Among 69 patients, 28 (40%) had prolonged pleural effusion whereas 11 (16%) had effusions that lasted longer than 14 days. Ten patients among prolonged effusion group (35%) had pulmonary atresia coexistent with the main diagnosis. Fontan operation was performed in 6 patients (8.7%) over the age of 10, and 4 of these patients (67%) had prolonged pleural effusion. Among numerous variables, statistical significance between the two groups was achieved in pre-operative mean pulmonary artery pressure, post-operative albumin, C-reactive protein levels, length of hospital stay, duration of chest tube drainage, and amount of effusion per day. Early recognition and treatment strategies with routine medical protocol use remain to be the cornerstone for the management of post-operative prolonged pleural effusions after Fontan surgery.
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Procedimiento de Fontan , Derrame Pleural , Atresia Tricúspide , Humanos , Procedimiento de Fontan/efectos adversos , Procedimiento de Fontan/métodos , Estudios Retrospectivos , Resultado del Tratamiento , Derrame Pleural/diagnóstico , Derrame Pleural/etiología , Derrame Pleural/epidemiología , Atresia Tricúspide/complicaciones , Atresia Tricúspide/cirugíaRESUMEN
INTRODUCTION: Long QT syndrome (LQTS) is of great importance as it is the most common cause of sudden cardiac death in childhood. The diagnosis is made by the prolongation of the QTc interval on the electrocardiography. However, clinical heterogeneity and nondiagnostic QTc intervals may cause a delay in the diagnosis. In such cases, genetic tests such as next-generation sequencing (NGS) panel analysis enable a definitive diagnosis. We present the first study that aimed to expand the LQTS's mutational spectrum by NGS panel analysis from Turkey. METHODS: Fifty-seven unrelated patients with clinically diagnosed LQTS were investigated using an NGS panel that includes six LQTS-related genes. Clinical aspects, outcome, and molecular analysis results were reviewed. RESULTS: Pathogenic (53%)/likely pathogenic (23%)/variant of unknown significance (4%) variants were detected in any of the genes examined in 79% of the patients. Among all detected variants, KCNQ1(71%) was the most common gene, followed by SCN5A (11%), KCNH2 (10%), CALM1 (5%), and CACNA1C (3%). Twelve novel variants were detected. Among the variants in KCNQ1, the c.1097G>A variant was present in 42% of patients. This variant also composed 31% of the variants detected in all of the genes. CONCLUSION: Our study expands the spectrum of the variations associated with LQTS with twelve novel variants in five genes. And also it draws attention to the frequency of the KCNQ1 c.1097G>A variant and forms the basis for new studies to determine the possible founder effect in the Turkish population. Furthermore, identifying new variants and clinical findings has importance in elaborating the roles of related genes in pathophysiology and determining the variable expression and incomplete penetration rates in this syndrome.
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Síndrome de QT Prolongado , Canales de Potasio de Rectificación Interna , Canales de Calcio Tipo L/genética , Canal de Potasio ERG1/genética , Humanos , Canal de Potasio KCNQ1/genética , Síndrome de QT Prolongado/diagnóstico , Síndrome de QT Prolongado/genética , Mutación , Canal de Sodio Activado por Voltaje NAV1.5 , Canales de Potasio de Rectificación Interna/genética , TurquíaRESUMEN
OBJECTIVES: Fasciculoventricular fiber (FVF) that does not cause tachyarrhythmia is a rare form of ventricular preexcitation, which is important to distinguish from Kent fibers. Although, adenosine and some electrocardiographic features are important in the differentiation of Wolff Parkinson White (WPW) than FVF, a clear distinction may not always be possible without an electrophysiological study (EPS). In this study, we aimed to present the clinical and electrophysiological features of our pediatric patients with fasciculoventricular fiber. PATIENTS AND METHOD: Between October 2013 and September 2021, 565 patients who underwent electrophysiological studies due to ventricular preexcitation in our clinic were screened in the study, and 27 (4.7%) patients with fasciculoventricular fiber were included. The data of the patients were obtained from the file records using the electronic internet database system Filemaker® . Electrophysiological study age, weight, gender, symptom, and presence of congenital heart disease of the patients were obtained from the file records. Accessory pathway localization was evaluated according to the modified Arruda algorithm in pre-procedural electrocardiography. In addition, delta wave amplitudes were measured in the first 40 ms from the surface ECG. PR interval, QRS interval, and delta wave amplitude were recorded before and after ablation in patients with additional accessory pathways. Post-procedure values were included in the FVF group. RESULTS: The mean age of the patients was 11.47 ± 4.25 years. All 70.4% of the reasons for admission were symptoms such as palpitations and syncope. Two patients had hypertrophic cardiomyopathy and 1 patient had ccTGA. In the electrophysiological study, additional manifest WPW was found in 9 (33%) patients (3 patients with high risk, 6 patients with orthodromic supraventricular tachycardia), focal atrial tachycardia in a patient, and atrioventricular nodal reentry tachycardia in a patient. While the delta wave amplitude was found to be 2.56 ± 1.38(1-5.5) mm in the first 40 ms in surface electrocardiography in 9 patients with additional accessory pathway, it was found to be 1.64 ± 0.67(0.5-3) mm in the FVF group. There was no statistically significant difference between the 2 groups (p = .398). Delta wave amplitude > 3.5 mm was not detected in any patient with isolated FVF. Interestingly, delta wave amplitude was < 3.5 mm in 7 (78%) of 9 patients who were identified and ablated with an additional accessory pathway. Total 19 of the patients (59.3%) were adenosine-responsive (18 isolated FVF, 1 manifest AP+FVF adenosine-responsive. 8 patients with other manifest AP + FVF had no pre-procedural adenosine-asystole response, and all of them QRS were expanded). CONCLUSION: Although, the fasciculoventricular fibers themselves are not the cause of tachyarrhythmia, the accessory pathway and other tachyarrhythmia substrate frequency accompanying these cases are quite high (approximately 40%) in EPS. The delta wave characteristics of ablated patients are very similar to FVF patients. While all patients with isolated FVF were adenosine responsive, most of those with additional manifest WPW were unresponsive. Therefore, performing EPS in patients with suspected FVF based on surface ECG features seems to be important for the detection of additional tachyarrhythmias and risky accessory pathways.
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Fascículo Atrioventricular Accesorio , Síndromes de Preexcitación , Taquicardia por Reentrada en el Nodo Atrioventricular , Síndrome de Wolff-Parkinson-White , Adenosina , Adolescente , Niño , Electrocardiografía , Sistema de Conducción Cardíaco , HumanosRESUMEN
BACKGROUND: Mitochondrial fatty acid oxidation disorders (FAODs) cause impairment in energy metabolism and can lead to a spectrum of cardiac pathologies including cardiomyopathy and arrhythmias. The frequency of underlying cardiac pathologies and the response to recommended treatment in FAODs was investigated. METHODS: Sixty-eight children (35 males, 33 females) with the diagnosis of a FAOD were included in the study. Cardiac function was evaluated with 12-lead standard electrocardiography, echocardiography, and 24 h Holter monitoring. RESULTS: Forty-five patients (66%) were diagnosed after disease symptoms developed and 23 patients (34%) were diagnosed in the pre-symptomatic period. Among symptomatic patients (n: 45), cardiovascular findings were detected in 18 (40%) patients, including cardiomyopathy in 14 (31.1%) and conduction abnormalities in 4 (8.8%) patients. Cardiac symptoms were more frequently detected in primary systemic carnitine deficiency (57.1%). Patients with multiple acyl-CoA dehydrogenase, long-chain 3-hydroxyacyl-CoA dehydrogenase, and mitochondrial trifunctional protein deficiencies also had an increased frequency of cardiac symptoms. Patients with medium-chain acyl-CoA dehydrogenase, very long-chain acyl-CoA dehydrogenase, and carnitine palmitoyltransferase I deficiencies had a lower prevalence of cardiac symptoms both during admission and during clinical follow up. Cardiomyopathy resolved completely in 8/14 (57%) patients and partially in 2/14 (14.3%) patients with treatment. Two patients with cardiomyopathy died in the newborn period; cardiomyopathy persisted in 1 (7.1%) patient with very long-chain acyl-CoA dehydrogenase deficiency. CONCLUSION: Early diagnosis, treatment and follow up made a significant contribution to the improvement of cardiac symptoms of patients with FAODs.
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Cardiomiopatías , Errores Innatos del Metabolismo Lipídico , Enfermedades Mitocondriales , Niño , Recién Nacido , Masculino , Femenino , Humanos , Errores Innatos del Metabolismo Lipídico/diagnóstico , Acil-CoA Deshidrogenasa de Cadena Larga/metabolismo , Acil-CoA Deshidrogenasa , Enfermedades Mitocondriales/diagnóstico , Cardiomiopatías/diagnóstico , Ácidos Grasos , Carnitina , Oxidación-ReducciónRESUMEN
Triadin knockout syndrome has been defined as a disease with transient long QT, T wave abnormalities, and extremely severe fatal cardiac arrhythmias in young children. In this report, we wanted to share the characteristics of our two cases who presented with sudden cardiac arrest and were diagnosed with triadin knockout syndrome. CASE 1: A 7.5-year-old male patient was referred to our clinic with a history of recurrent syncope and aborted cardiac arrest. There was no family history of sudden death, syncope, or arrhythmia. Physical examination, electrocardiography, echocardiography, and 24-hour rhythm Holter monitoring were normal, and bidirectional ventricular tachycardiaT was detected during the exercise stress test. Genetic analysis revealed a homozygous mutation of c.531_533delinsGG, p.(Lys179Asnfs * 44) frameshift variant in TRDN(NM_006073) gene. CASE 2: A 4.5-year-old male was admitted due to syncope during exertion and underwent cardiopulmonary resuscitation due to sudden cardiac arrest. He had family history about sudden cardiac death. Physical examination was normal, and there was borderline QTc prolongation. Bidirectional non-sustained polymorphic ventricular tachycardia was observed at adrenaline provocation test. In genetic analysis, c.568dupA, pII190Asnfs * 2 frameshift variant homozygous mutation was detected in TRDN(NM_006073) gene. Intracardiac defibrillator implantation were performed for both cases. There has not been any event under propranolol and flecainide combination treatment. CONCLUSION: Triadin knockout syndrome (TCOS) is a rare overlap syndrome characterized by highly malignant arrhythmias, and it is a deadly combination of long QT syndrome and catecholaminergic polymorphic ventricular tachycardia that affects primarily young children. Since lethal arrhythmias are frequently described, genetic testing is very important in these patients. Because, identification of a genetic mutation may be a guide in treatment.
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A young child presented with syncope attacks. Late-onset post-operative complete atrioventricular block and Torsades de Pointes were diagnosed. She was treated with surgical epicardial pacemaker implantation. This report is the description of Torsades de Pointes due to late-onset post-operative complete atrioventricular block followed by R on T phenomenon in a child.
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Bloqueo Atrioventricular , Síndrome de QT Prolongado , Marcapaso Artificial , Torsades de Pointes , Femenino , Niño , Humanos , Torsades de Pointes/diagnóstico , Torsades de Pointes/etiología , Bloqueo Atrioventricular/diagnóstico , Bloqueo Atrioventricular/etiología , Bloqueo Atrioventricular/terapia , Síndrome de QT Prolongado/diagnóstico , Marcapaso Artificial/efectos adversos , ElectrocardiografíaRESUMEN
A young child presented with hepatomegaly, ascites and bradycardia in the setting of COVID-19. Permanent complete atrioventricular block and severe right heart failure were diagnosed. He was treated with surgical epicardial pacemaker implantation. This report is the first description of COVID-19-induced permanent complete atrioventricular block in a child.
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Bloqueo Atrioventricular , COVID-19 , Marcapaso Artificial , Disfunción Ventricular Derecha , Bloqueo Atrioventricular/diagnóstico , Bloqueo Atrioventricular/etiología , Bloqueo Atrioventricular/terapia , Bradicardia/diagnóstico , Bradicardia/etiología , COVID-19/complicaciones , Niño , Humanos , Masculino , Disfunción Ventricular Derecha/etiologíaRESUMEN
Cardiac pacemakers have improved patient survival and quality of life, although 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 threshold 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.
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Falla de Equipo , Hipertensión Pulmonar/complicaciones , Marcapaso Artificial , Preescolar , Femenino , HumanosRESUMEN
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.
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Estimulación Cardíaca Artificial/métodos , Electrodos Implantados , Cardiopatías Congénitas/terapia , Marcapaso Artificial , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Estudios RetrospectivosRESUMEN
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.
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Cardiomiopatía Hipertrófica , Adolescente , Arritmias Cardíacas , Cardiomiopatía Hipertrófica/diagnóstico , Cardiomiopatía Hipertrófica/cirugía , Cardiomiopatía Hipertrófica Familiar , Niño , Preescolar , Muerte Súbita Cardíaca , Desfibriladores Implantables , Femenino , Humanos , Lactante , MasculinoRESUMEN
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.
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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.
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Ablación por Catéter , Criocirugía , Taquicardia Supraventricular , Niño , Humanos , Estudios Retrospectivos , Taquicardia Supraventricular/diagnóstico , Taquicardia Supraventricular/cirugía , Resultado del TratamientoRESUMEN
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.
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Terapia de Resincronización Cardíaca , Cardiopatías Congénitas , Insuficiencia Cardíaca , Bloqueo de Rama , Niño , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/terapia , Insuficiencia Cardíaca/terapia , Humanos , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
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.