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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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Feixe Acessório Atrioventricular , Ablação por Cateter , Eletrocardiografia , Síndrome de Wolff-Parkinson-White , Humanos , Síndrome de Wolff-Parkinson-White/cirurgia , Síndrome de Wolff-Parkinson-White/fisiopatologia , Masculino , Feminino , Estudos Retrospectivos , Ablação por Cateter/métodos , Feixe Acessório Atrioventricular/cirurgia , Feixe Acessório Atrioventricular/fisiopatologia , Adolescente , Criança , Adulto JovemRESUMO
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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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 Arritmogênica Ventricular Direita , Cardiomiopatias , Taquicardia Ventricular , Feminino , Humanos , Criança , Adolescente , Taquicardia , Cardiomiopatias/complicações , Cardiomiopatias/diagnóstico , Ventrículos do Coração , Displasia Arritmogênica Ventricular Direita/complicações , Displasia Arritmogênica Ventricular Direita/diagnóstico , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiologiaRESUMO
BACKGROUND: We aimed to share our experience of intra-atrial reentrant tachycardia mapping and ablation with a new grid-style multielectrode high-density mapping catheter (Advisor™ HD Grid) in pediatric and young adult patients with operated congenital heart disease. METHODS: All patients with operated congenital heart disease and intra-atrial reentrant tachycardia mapping with the new grid-style catheter between October 2019 and December 2022 were included (group 1), and the results were compared to those patients who operated with conventional catheter methods before this period (group 2). All procedures were performed using the EnSite Precision 3D mapping system (Abbott Laboratories, Abbott Park, Ill, USA) with a limited fluoroscopy approach. Data were evaluated retrospectively. RESULTS: In group 1 (n = 16; 9 male), the median age was 21 years (10-36), compared to 19 years (9-27) in group 2 (n = 10; 5 male). While irrigated radiofrequency ablation was pre-ferred in all patients, the median number of 15 lesions (8-38) in group 1 was significantly less than the median of 30 lesions (8-71) in group 2 (P =.027). The median procedure duration of 159 minutes (110-233) in group 1 was significantly shorter compared to 280 minutes (180-370) in group 2 (P <.05). Acute procedural success was achieved in all patients (16/16; 100%) in group 1 compared to 8/10 patients (80%) in group 2. During the median follow-up of 27 months (11-36), there was only 1 intra-atrial reentrant tachycardia recurrence in group 1 (1/16; 6.2%) and 2 recurrences (2/8; 25%) in group 2 during the median follow-up of 110 months (56-151). No complications related to the mapping catheter itself occurred. CONCLUSION: In the intra-atrial reentrant tachycardia ablation of children with congenital heart disease to increase procedural success and shorten the mapping duration, the utility of Advisor™ HD Grid mapping catheter seems to be a feasible alternative.
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Fibrilação Atrial , Ablação por Cateter , Cardiopatias Congênitas , Taquicardia Supraventricular , Humanos , Masculino , Criança , Adulto Jovem , Adulto , Fibrilação Atrial/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Taquicardia/cirurgia , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/complicações , Ablação por Cateter/efeitos adversos , Catéteres/efeitos adversosRESUMO
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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Ablação por Cateter , Taquicardia Atrial Ectópica , Taquicardia Supraventricular , Masculino , Humanos , Lactente , Ivabradina/uso terapêutico , Taquicardia Atrial Ectópica/diagnóstico , Taquicardia Atrial Ectópica/tratamento farmacológico , Taquicardia Atrial Ectópica/cirurgia , Eletrocardiografia , Taquicardia Supraventricular/cirurgia , Arritmias Cardíacas/tratamento farmacológicoRESUMO
BACKGROUND: Isolated complete atrioventricular block is a rare disease often associated with maternal autoantibodies. This study aimed to present the midterm data of patients at our clinic diagnosed with isolated complete atrioventricular block. METHODS: We evaluated 108 patients diagnosed with isolated complete atrioventricular block. Demographic data of the patients, electrocardiography, echocardiography, 24-hour Holter monitoring data, and follow-up and complications of the patients who underwent pacemaker implantation were evaluated retrospectively. RESULTS: The mean age of the patients at diagnosis was 5.51 ± 5.05 years. At the time of diagnosis, 74.8% of the patients had no symptoms associated with complete atrioventricular block. The most common symptom was fatigue. Pacemaker implantation was needed in 88 (81.4%) patients during follow-up. Significant bradycardia was the most common pacemaker implantation indication. The mean battery life was 5.41 ± 2.65 years. The battery replacement-free period of 68 patients who underwent pacemaker implantation and continued their follow-up was 4.18 ± 2.89 (0.1-10) years. Pacemaker-related complications developed in 8 patients during follow-up. Left ventricular dysfunction developed (dyssynchrony induced) in 3 patients at follow-up, and all were paced from the right ventricular anterior wall. Those patients underwent cardiac resynchronization therapy and their left ventricular dysfunction improved. CONCLUSION: Isolated complete atrioventricular block is a rare disease requiring careful clinical follow-up. Patients are often asymptomatic and significant bradycardia is the most common indication for pacemaker implantation. Left ventricular dysfunction is an important cause of morbidity, especially in patients with right ventricular anterior wall pacing. Physicians should be aware of left ventricular dysfunction during follow-up. Cardiac resynchronization therapy should be considered as a treatment option for left ventricular dysfunction.
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Bloqueio Atrioventricular , Terapia de Ressincronização Cardíaca , Cardiopatias , Marca-Passo Artificial , Disfunção Ventricular Esquerda , Humanos , Criança , Lactente , Pré-Escolar , Bloqueio Atrioventricular/terapia , Seguimentos , Estudos Retrospectivos , Bradicardia/terapia , Estimulação Cardíaca Artificial , Doenças Raras/terapiaRESUMO
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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Ablação por Cateter , Síndrome de Wolff-Parkinson-White , Humanos , Criança , Síndrome de Wolff-Parkinson-White/cirurgia , Átrios do Coração/cirurgia , Eletrocardiografia , Aorta/cirurgiaRESUMO
BACKGROUND: In this study, we aimed to evaluate the clinical aspects, electrophysiological studies, and ablation results of permanent junctional reciprocating tachycardia in children. METHODS: 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. RESULTS: 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. CONCLUSION: 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.
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Cardiomiopatias , Ablação por Cateter , Taquicardia Reciprocante , Taquicardia Supraventricular , Feminino , Criança , Humanos , Masculino , Estudos Retrospectivos , Taquicardia Supraventricular/diagnóstico , Ablação por Cateter/métodos , Eletrocardiografia , Taquicardia/cirurgia , Cardiomiopatias/cirurgia , Taquicardia Reciprocante/diagnóstico , Taquicardia Reciprocante/cirurgiaRESUMO
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.
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Cardiomiopatia Dilatada , Disfunção Ventricular Esquerda , Cardiomiopatia Dilatada/genética , Criança , Morte Súbita Cardíaca/etiologia , Humanos , Lamina Tipo A/genética , Masculino , Mutação , Proteínas Nucleares/genéticaRESUMO
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.
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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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Feixe Acessório Atrioventricular , Síndromes de Pré-Excitação , Taquicardia por Reentrada no Nó Atrioventricular , Síndrome de Wolff-Parkinson-White , Adenosina , Adolescente , Criança , Eletrocardiografia , Sistema de Condução Cardíaco , HumanosRESUMO
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: 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.
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Perda Auditiva Bilateral/complicações , Perda Auditiva Neurossensorial/complicações , Síndrome de Jervell-Lange Nielsen/epidemiologia , Adolescente , Criança , Morte Súbita Cardíaca , Eletrocardiografia , Feminino , Perda Auditiva Bilateral/congênito , Perda Auditiva Neurossensorial/congênito , Homozigoto , Humanos , Síndrome de Jervell-Lange Nielsen/diagnóstico , Síndrome de Jervell-Lange Nielsen/genética , Canal de Potássio KCNQ1/genética , Masculino , Mutação , Prevalência , Estudos Prospectivos , Síncope/etiologia , Turquia/epidemiologiaRESUMO
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<0.001), and the overall mortality rate of pediatric sustained VT was observed at 6.9% in this study. CONCLUSION: VTs, which can cause sudden cardiac arrest, are potentially life-threatening arrhythmias. Identifying the heterogeneity of this VT and its peculiar characteristics would facilitate appropriate diagnosis and therapy.
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Ablação por Cateter , Taquicardia Ventricular , Criança , Seguimentos , Humanos , Masculino , Estudos Retrospectivos , Taquicardia Ventricular/cirurgia , Resultado do TratamentoRESUMO
OBJECTIVE: Long QT syndrome (LQTS) is an inherited primary arrhythmia syndrome associated with life-threatening ventricular arrhythmias and sudden death. This study aimed to report the clinical and genetic characteristics and outcomes of children diagnosed as having LQTS in a tertiary pediatric cardiology center in Turkey. METHODS: This was a retrospective review of pediatric patients diagnosed as having LQTS at our center from January 2011 to April 2020. RESULTS: A total of 145 patients (76 males) were included, with a mean age of 9.2±4.5 years and a mean weight of 35.7±18.5 kg; 38 (26.2%) were identified as having LQTS during family screening, whereas a significant proportion of patients were asymptomatic at presentation, 15 patients (10.3%) were diagnosed after previous cardiac arrest, and 26 patients (18%) had syncope. The mean Schwartz score was 4.5 points (range, 3-7.5 points). Furthermore, 107 patients (82%) were confirmed to have a pathogenic mutation for LQTS genes. All patients received beta-blockers. Implantable cardioverter-defibrillator insertion was performed in 34 patients (23.4%). Left or bilateral cardiac sympathetic denervation was performed in 9 patients (6.2%). Median follow-up time was 35.6±25.8 months. Five (3.4%) patients died during the follow-up. Statistical analyses of risk factors for major cardiac events revealed that the QTc was >500 ms and that T wave alternans, high Schwartz score, and Jervell and Lange-Nielsen syndrome were strong and significant predictors of cardiac events. CONCLUSION: LQTS has a variety of clinical manifestations. Patients' symptoms ranged between asymptomatic and sudden cardiac death (SCD). By raising the awareness of physicians regarding the disease, SCD might be prevented in the early period.
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Desfibriladores Implantáveis , Síndrome do QT Longo , Adolescente , Criança , Pré-Escolar , Morte Súbita Cardíaca/etiologia , Eletrocardiografia , Humanos , Síndrome do QT Longo/complicações , Síndrome do QT Longo/genética , Masculino , Estudos RetrospectivosRESUMO
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<0.05). The most common operations associated with early postoperative arrhythmia were left ventricular outflow tract, (6/20, 30%), complete atrioventricular septal defect (13/53, 24%), and tetralogy of Fallot (20/134, 14%) surgeries. CONCLUSION: Cardiac arrhythmias are common in the early period after congenital heart surgery in children. The diagnosis and frequency of arrhythmias may vary according to different surgical procedures.
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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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Falha de Equipamento , Hipertensão Pulmonar/complicações , Marca-Passo Artificial , Pré-Escolar , Feminino , HumanosRESUMO
PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.
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Ablação por Cateter , Criocirurgia , Taquicardia Ventricular , Complexos Ventriculares Prematuros , Adolescente , Criança , Eletrocardiografia , Ventrículos do Coração/cirurgia , Humanos , Taquicardia Ventricular/diagnóstico por imagem , Taquicardia Ventricular/cirurgia , Complexos Ventriculares Prematuros/diagnóstico por imagem , Complexos Ventriculares Prematuros/cirurgiaRESUMO
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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Cardiomiopatia Hipertrófica , Adolescente , Arritmias Cardíacas , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/cirurgia , Cardiomiopatia Hipertrófica Familiar , Criança , Pré-Escolar , Morte Súbita Cardíaca , Desfibriladores Implantáveis , Feminino , Humanos , Lactente , MasculinoRESUMO
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.