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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
As the life expectancy improves in cystic fibrosis, cardiac dysfunction is becoming an important risk factor for morbidity and mortality. Here, the association of cardiac dysfunction with proinflammatory markers and neurohormones between cystic fibrosis patients and healthy children was investigated. Echocardiographic measurements of right and left ventricular morphology and functions together with levels of proinflammatory markers and neurohormones (renin, angiotensin-II, and aldosterone) were obtained and analysed in a study group of 21 cystic fibrosis children aged 5-18 years and compared with age- and gender-matched healthy children. It was shown that patients had significantly higher interleukin-6, C-reactive protein, renin and aldosterone levels (p < 0.05), dilated right ventricles, decreased left ventricle sizes, as well as both right and left ventricular dysfunction. These echocardiographic changes correlated with hypoxia, interleukin-1 α, interleukin-6, C-reactive protein, and aldosterone (p < 0.05) levels. The current study revealed that hypoxia, proinflammatory markers, and neurohormones are major determinants of subclinical changes in ventricular morphology and function. While the right ventricle anatomy was affected by cardiac remodeling, the left ventricle changes were induced by right ventricle dilation and hypoxia. A significant but subclinical systolic and diastolic right ventricle dysfunction in our patients was associated with hypoxia and inflammatory markers. Systolic left ventricle function was affected by hypoxia and neurohormones. Echocardiography is a reliable and non-invasive method that is used safely in cystic fibrosis children for screening and detection of cardiac anatomical and functional changes. Extensive studies are needed to determine the time and frequency of screening and treatment suggestions for such changes.
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Fibrose Cística , Disfunção Ventricular Esquerda , Criança , Humanos , Fibrose Cística/complicações , Proteína C-Reativa , Aldosterona , Interleucina-6 , Renina , Ecocardiografia/métodos , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/etiologia , Ventrículos do Coração/diagnóstico por imagem , Neurotransmissores , HipóxiaRESUMO
Hypertension is an increasing disease in children and the risk of endothelial damage and target organ damage increases in the presence of additional risk factors such as obesity. In our study, the effect of hypertension on early atherosclerotic changes and target organ damage in children was investigated. Twenty four-hour ambulatory pulse wave analysis was performed by oscillometric method in 71 children aged 8-18 years, 17 of whom were diagnosed with primary hypertension without obesity, 18 had both primary hypertension and obesity, and 16 had renal hypertension. Twenty healthy normotensive children were included as the control group. Carotid intima-media thickness (CIMT) and Left Ventricular Mass Index were measured. Central systolic blood pressure (cSBP), central diastolic blood pressure (cDBP), systolic blood pressure (SBP) and diastolic blood pressure (DBP) were higher in the primary hypertension group compared to controls (p = 0.001, p = 0.005, p = 0.001, p = 0.009, respectively), cSBP was higher in the renal hypertension group than the control group (p = 0.018). There was no difference between the groups in terms of pulse wave analysis parameters, CIMT, or left ventricular mass index (p > 0.05). Pulse wave velocity was positively correlated with SBP, DBP, cSBP, cDBP (p < 0.001). Augmentation index was positively correlated with DBP and cDBP (p = 0.01, p = 0.002, respectively). Our findings show that high blood pressure is associated with arterial stiffness and target organ damage beginning in childhood. The detection of early atherosclerotic vascular changes using pulse wave analysis allows to take necessary precautions such as lifestyle changes to prevent target organ damage in hypertensive children.
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Aterosclerose , Hipertensão Renal , Hipertensão , Rigidez Vascular , Humanos , Criança , Espessura Intima-Media Carotídea , Análise de Onda de Pulso , Hipertensão/complicações , Pressão Sanguínea/fisiologia , Hipertensão Renal/complicações , Obesidade/complicações , Aterosclerose/diagnóstico por imagem , Aterosclerose/complicações , Hipertensão EssencialRESUMO
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
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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BACKGROUND: Transcatheter closure of a patent ductus arteriosus (PDA) has always been considered risky for infants weighing <6 kg and preterms. We present our findings regarding transcatheter closures of PDA. METHODS: The inclusion criteria were a weight of <6 kg and the presence of PDA symptoms. The study subjects were divided into two groups: <6 kg and premature infants. RESULTS: A total of 69 infants were included. The mean ages and weights of the <6 kg and the preterms were 5.4 ± 2.7 months and 30.3 ± 19.9 days, and 4.6 ± 0.8 and 1.7 ± 0.3 kg, respectively. Type C PDAs were most frequently observed in the premature group, and type A was in <6 kg. Sixteen of the patients were premature infants, and 81.2% of them had an extremely low birth weight. All of the premature infants had comorbidities, and had been receiving respiratory support therapy. Transcatheter closure was successfully completed in 81.2% of the premature infants and 94.3% of the <6-kg infants. Major complications occurred in 4 patients (one death and three device embolizations). The patient's age was found to be the main risk factor. The most frequently used device was the Amplatzer duct occluder II in additional sizes (84.6%) in the preterms and the Amplatzer duct occluder I (34%) and II (34%) in the <6-kg group. CONCLUSION: The transcatheter closure of PDA is relatively safe and effective in preterms and in infants <6 kg. The selection of a suitable device based on the type of PDA is critical to the success of the procedure.
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Cateterismo Cardíaco , Permeabilidade do Canal Arterial/cirurgia , Dispositivo para Oclusão Septal , Peso Corporal , Feminino , Humanos , Lactente , Recém-Nascido de Baixo Peso , Recém-Nascido , Recém-Nascido Prematuro , Masculino , Estudos Retrospectivos , Fatores de Risco , Resultado do TratamentoRESUMO
A special deformity associated with the use of left atrial disc hubless atrial septal occluders has been described in 3 children. This chalice-shaped deformity that occurred during the use of Occlutech and Lifetech septal occluders was given the name "Holy Grail Configuration". Despite being easily reversible, reduction of the metal load on the left atrium has been suggested to have some negative effects.
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Átrios do Coração , Comunicação Interatrial , Ajuste de Prótese/métodos , Implantação de Prótese , Dispositivo para Oclusão Septal , Adolescente , Cateterismo Cardíaco/métodos , Criança , Pré-Escolar , Ecocardiografia , Feminino , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/cirurgia , Comunicação Interatrial/diagnóstico , Comunicação Interatrial/cirurgia , Humanos , Masculino , Desenho de Prótese , Implantação de Prótese/instrumentação , Implantação de Prótese/métodos , Resultado do TratamentoRESUMO
INTRODUCTION: The suppression of tumorigenicity 2 (ST2) is a receptor member belonging to the interleukin-1 (IL-1) family. The ligand and soluble versions are its two isoforms. The IL-33-ST2L ligand complex's development provides protection against heart fibrosis and hypertrophy. Investigations on heart failure in adults have demonstrated that it does not change by age, body mass index (BMI), creatinine, hemoglobin, and albumin levels, in contrast to NT pro BNP. In adult heart failure patients, it has been demonstrated to be an independent predictor of mortality and cardiovascular events. The most recent guideline recommends using it as class 2b in the diagnosis of adult heart failure. Studies on ST2 in children are rare. The purpose of this study is to assess the prognostic value of ST2 for cardiovascular events in young individuals suffering from heart failure. METHOD: This study included pediatric patients (0-18 years old) with congenital heart disease or cardiomyopathy who needed medical care, as well as surgical or interventional treatment. Height, weight, gender, saturation, heart failure classification (Ross or NYHA), medications, the electrocardiogram, echocardiography, pro BNP, and sST2 values of the patients, as well as any hospitalization, lower respiratory tract infection, organ dysfunction, or need for angiography or surgery during follow-up data on arrhythmia and death were gathered during a 1-year follow-up. The SPSS software version 25 application was used to carry out the statistical analysis. RESULTS: This study included 59 patients, of whom 27 (46.6%) were male. The average age of the patients was 55.5 months (1-228 months) and the average body weight was 16 kg (2.6-90 kg). Major cardiovascular events occurred in 45 of 59 patients (76.3%). Twenty-four patients experienced one MACE, while twenty-one patients experienced multiple MACEs. Pro BNP and sST2 levels were similar in the groups that developed MACE compared to those that did not. Pro BNP was discovered to be significantly higher in patients with hospitalization, growth retardation, lower respiratory tract infection, and organ failure, however, when assessing each situation (p = 0.001, p = 0.011, p = 0.001, p = 0.007, respectively). Soluble ST2 was found to be higher in patients with growth retardation than in those without (p = 0.037). Although the soluble ST2 level failed to demonstrate a correlation with pro BNP, it did show a positive correlation (r = 0.437) with the Ross score. When compared to other groups, it was discovered to be higher in patients with valvular insufficiency type heart disease. CONCLUSIONS: In this study, higher sST2 levels were discovered, particularly in the group with valve insufficiency and children with growth retardation. It was associated with the Ross score, but not with the pro BNP level. Although it increases in correlation with clinical heart failure, its predictive value for MACE is low. Similarly, pro BNP is not proven to be predictive; nonetheless, its high levels in patients with hospitalization, growth retardation, lower respiratory tract infection, and organ failure demonstrate that pro BNP may increase for a variety of causes. Long-term studies with more patients are needed for ST2 to be suitable for clinical use in pediatric patients.
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BACKGROUND: Intracoronary sinus ablations have been performed for various arrhythmical substrates. The aim of this study is to report our experience on pediatric patients of the safety and efficacy of ablations in the coronary sinus. METHODS: This is a retrospective study of all patients who underwent ablations in the coronary sinus from October 2013 to October 2021 at a single center. Clinical presentation, type of arrhythmia causing tachycardia, ablation procedure, and outcome were recorded. RESULTS: A total of 27 patients were included in the study. Nineteen (69%) of those followed up received a diagnosis of Wolff-Parkinson-White syndrome (WPW), 4 (15%) were cases of supraventricular tachycardia with concealed accessory pathway (AP), 2 (8%) were cases of focal atrial tachycardia, and 2 (8%) were cases of permanent junctional reciprocating tachycardia. Negative delta wave was noteworthy especially in lead II in 11/19 (58%) cases and coronary sinus diverticulum was detected in the WPW cases. Of those with manifest AP (19 cases), 15 (79%) had a high-risk AP and the AP in all WPW cases was adenosine unresponsive. Radiofrequency (RF) catheter ablation was performed in 25/27 (93%) cases during the procedure, and 16/25 (64%) of these were irrigated RF catheters. No complications were observed in the follow-up, including coronary artery injury. CONCLUSIONS: Catheter ablation of supraventricular tachyarrhythmias can be accomplished effectively and potentially safely within the coronary sinus. Coronary sinus diverticula should be suspected in patients with manifest posteroseptal APs who have a previous failed ablation and typical electrocardiographic signs.
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Feixe Acessório Atrioventricular , Ablação por Cateter , Seio Coronário , Taquicardia Supraventricular , Síndrome de Wolff-Parkinson-White , Humanos , Criança , Seio Coronário/diagnóstico por imagem , Seio Coronário/cirurgia , Estudos Retrospectivos , Síndrome de Wolff-Parkinson-White/cirurgia , Feixe Acessório Atrioventricular/cirurgia , Taquicardia Supraventricular/cirurgia , Taquicardia/cirurgia , EletrocardiografiaRESUMO
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
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
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
OBJECTIVE: The aim of this study was to use tissue Doppler imaging to evaluate the left atrial systolic and the left ventricular (LV) diastolic function as well as the left atrial ejection force in children who underwent transcatheter closure of a secundum atrial septal defect (ASD). METHODS: Tissue Doppler measurements of the left atrial ejection force, the mitral valve, and left atrial volume were performed before the ASD closure procedure, and on the 1st day, 10th day, and 1st and 3rd months after the procedure in 56 patients and in 28 healthy controls. RESULTS: There was a significant decrease in the septal and lateral a' velocities on the first day (p<0.05). There was a statistically significant increase in the septal e'/a' parameters at the third month compared with the initial measurements. The left atrial ejection force was lower in patients with an ASD than in the healthy group (10.69±4.94 kdyn, 12.31±4.05 kdyn, respectively), but there was no significant difference (p=0.053). The left atrial ejection force was significantly greater in the patient group 3 months after the procedure, and there was no significant difference compared with the control group. CONCLUSION: Improvement in the LV diastolic and left atrial systolic functions was observed in children who underwent transcatheter closure of an ASD. There was no negative effect related to the devices used.
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Função do Átrio Esquerdo/fisiologia , Cateterismo Cardíaco/efeitos adversos , Comunicação Interatrial/fisiopatologia , Função Ventricular Esquerda/fisiologia , Velocidade do Fluxo Sanguíneo/fisiologia , Cateterismo Cardíaco/instrumentação , Estudos de Casos e Controles , Criança , Pré-Escolar , Diástole , Feminino , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/fisiopatologia , Comunicação Interatrial/diagnóstico , Comunicação Interatrial/cirurgia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Estudos Prospectivos , Dispositivo para Oclusão Septal/efeitos adversos , Sístole , Ultrassonografia Doppler/métodosRESUMO
Cutis laxa tip1b is a rare autosomal recessive disorder caused by FBLN 4 mutation and primarily characterized by vascular anomalies. Herein, we present five patients who are the members of the same family. The primary cardiac findings of these patients were giant aortic aneurysms. One 2.5-year-old patient with a massive aneurysm of the ascending aorta died as a result of compression to the heart chambers, trachea, and bronchi. The bentall procedure was performed in three of our patients who are under follow-up. One patient is still under clinical follow-up without surgery. After the diagnosis of the first patient, a genetic study was performed in which FBLN 4 mutations were investigated. Four new patients were detected during genetic screening of the family. Other 29 family members were screened bur were negative in physical examinations and echocardiography. Pedigree is important for early diagnosis of genetic diseases in asymptomatic individuals.
"Cutis laxa" tip1B ender görülen FBLN 4 mutasyonunun neden oldugu, ön planda damar anomalileri ile seyreden otozomal çekinik bir hastaliktir. Burada ayni ailenin üyesi olan bes olgumuzu sunduk. Olgularimizin asil kalp bulgusu çikan aort anevrizmasi idi. Bir olgumuz 2,5 yasinda çikan aorttaki dev anevrizmanin trakea, bronslar ve kalp odaciklarina basisi nedeni ile kaybedildi. Üç olgumuza Bentall operasyonu yapildi ve izlemleri devam etmektedir. Bir olgumuz halen klinik olarak izlemdedir. Ilk olgunun tani almasindan sonra genetik çalismasi yapildi ve FBLN 4 mutasyonu saptandi. Aile taramasi sirasinda belirtisiz dört olgu daha saptandi. Yogun akraba evliliklerinin oldugu ailenin taranabilen 29 üyesinde fizik baki ve ekokardiyografik incelemelerde sorun saptanmadi. Aile agacinin çikarilmasi, belirtisi olmayan bireylerde genetik hastaliklarin erken tanisi açisindan önemlidir.
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Right atrial aneurysms were first described in 1955, and subsequently, only a few cases have been reported. The etiology of this condition is unknown. Its comorbidity with cholestasis has not previously been reported in the literature. An 11-month-old baby female, who was an offspring from a first-cousin marriage, was referred to our hospital for investigation of jaundice. She underwent echocardiography because of a heart murmur, and this revealed a giant right atrial aneurysm. In addition, her liver biopsy confirmed the diagnosis of progressive familial intrahepatic cholestasis (PFIC) type 3. Although both conditions are rare, we found their comorbidity interesting and are thus reporting the case.
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OBJECTIVE: We prospectively investigated the neoendothelialization of transcatheter secundum atrial septal defect (ASD) closure in children receiving one of three different occluders. METHODS: Transcatheter ASD closure was performed for 44 children. The patients were divided into three groups: group I: Amplatzer, group II: Lifetech CeraFlex, and group III: Occlutech Figulla Flex II septal occluder. The data were prospectively analyzed. Markers of the three phases of wound healing were studied in all patients before and on the 1st and 10th days and 1st month post intervention. RESULTS: The mean age of children was 7.08±3.51 years, and the mean weight was 26.07±15.07 kg. The mean ASD diameter was 12.65±3.50 mm. Groups I, II, and III comprised 34.1%, 31.8%, and 34.1% patients, respectively. No significant differences were observed between the groups regarding patient number, age, defect size, device diameter, or total septum/device ratio (p>0.05). Inflammatory and proliferative phase marker levels increased following the procedure (p<0.05). However, scar formation markers did not change after 1 month. No significant differences in neoendothelializaton were observed among the different occluders (p>0.05). CONCLUSION: All three devices were composed of nitinol with different surface coating techniques. Although the different manufacturing features were claimed to facilitate of neoendothelialization, no differences were observed among the three devices 1 month following the procedure.
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Comunicação Interatrial/cirurgia , Dispositivo para Oclusão Septal , Cateterismo Cardíaco , Criança , Desenho de Equipamento , Feminino , Humanos , Masculino , Estudos Prospectivos , Resultado do Tratamento , CicatrizaçãoRESUMO
One of the most rare and dangerous complications of a modified Blalock-Taussig shunt involves the formation of a pseudoaneurysm. A pseudoaneurysm may rupture or may result in other adverse events including compression of mediastinal structures or collapse of the underlying lung parenchyma. Shunt-related pseudoaneurysm may be associated with shunt occlusion, bacteremia, or pulmonary hemorrhage. We describe two cases complicated by large pseudoaneurysm formation, following systemic-to-pulmonary artery shunt operations. The patients in question had recurrent pulmonary hemorrhage. Both underwent successful placement of covered coronary artery stents.
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Falso Aneurisma/cirurgia , Procedimento de Blalock-Taussig/efeitos adversos , Vasos Coronários/cirurgia , Stents , Falso Aneurisma/etiologia , Pré-Escolar , Feminino , Cardiopatias Congênitas/cirurgia , Humanos , Lactente , Masculino , Artéria Pulmonar/cirurgia , Resultado do TratamentoRESUMO
Embolization of a port catheter is a dangerous and serious complication. In this paper, we present two cases of children, aged 4.5 months and 6 years, in whom port catheters had embolized to the right ventricle one month and 1.5 years priorly, respectively; the port catheters were retrieved via snaring.