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ABSTRACT Objective This study aimed to provide a long-term follow-up of PRKAG2 syndrome and describe the new phenotypic aspects of the condition. PRKAG2 syndrome is a rare autosomal-dominant glycogen storage disease characterized by cardiac hypertrophy, ventricular pre-excitation, and conduction system disease. Fatal arrhythmias occur frequently. Methods A family cohort of 66 participants was recruited. Clinical and genetic analyses were performed. Results Median age of 36.97±17.28 years, with 69.9% being men. Nineteen subjects carried the deleterious variant p.K290I of the PRKAG2 gene. This group experienced many malignant events, including eight pacemaker implants, three sudden cardiac deaths, five aborted cardiac arrests, four strokes, four premature neonatal deaths, two spontaneous abortions, five forceps deliveries, and 12 cesarean procedures. Extracardiac involvement, such as in neurocognitive and psychiatric disorders, has been observed only in carriers of mutations. Palpitations, Syncope, atrial fibrillation, atrial flutter, sinus pauses, and bradycardia were strongly and significantly associated with major or severe adverse events (sudden cardiac death, aborted cardiac arrest, pacemaker use, stroke, and congestive heart failure). Early diagnosis and intervention through antiarrhythmic drugs, anticoagulation, pacemaker implantation, radiofrequency catheter ablation, and cesarean section surgery improved the symptoms and survival rates. Mutations carriers were advised to avoid pregnancy. Conclusion This study identified that the p.K291I_PRKAG2 mutation is associated with poor prognosis, highlighting the need for early intervention. Further research may uncover the potential connections between intellectual disability, miscarriage, and neonatal death in individuals with this syndrome.
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Abstract Background: In 1996 Iturralde et al. published an algorithm based on the QRS polarity to determine the location of the accessory pathways (AP), this algorithm was developed before the massive practice of invasive electrophysiology. Purpose: To validate the QRS-Polarity algorithm in a modern cohort of subjects submitted to radiofrequency catheter ablation (RFCA). Our objective was to determinate its global accuracy and its accuracy for parahisian AP. Methods: We conducted a retrospective analysis of patients with Wolff-Parkinson-White (WPW) syndrome who underwent an electrophysiological study (EPS) and RFCA. We employed the QRS-Polarity algorithm to predict the AP anatomical location and we compared this result with the real anatomic location determined in the EPS. To determine accuracy, the Cohen's kappa coefficient (k) and the Pearson correlation coefficient were used. Results: A total of 364 patients were included (mean age 30 years, 57% male). The global k score was 0.78 and the Pearson's coefficient was 0.90. The accuracy for each zone was also evaluated, the best correlation was for the left lateral AP (k of 0.97). There were 26 patients with a parahisian AP, who showed a great variability in the ECG features. Employing the QRS-Polarity algorithm, 34.6% patients had a correct anatomical location, 42.3% had an adjacent location and only 23% an incorrect location. Conclusion: The QRS-Polarity algorithm has a good global accuracy; its precision is high, especially for left lateral AP. This algorithm is also useful for the parahisian AP.
Resumen Antecedentes: En 1996 Iturralde y colaboradores publicaron un algoritmo basado en la polaridad del QRS para determinar la ubicación de las vías accesorias (VA), este algoritmo fue desarrollado antes de la práctica masiva de la electrofisiología invasiva. Objetivo: Validar el algoritmo de la polaridad del QRS en una cohorte moderna de sujetos sometidos a ablación con catéter por radiofrecuencia (ACRF). Nuestro objetivo fue determinar su precisión global y su precisión para las VA parahisianas. Métodos: Realizamos un análisis retrospectivo de pacientes con síndrome de Wolff-Parkinson-White (WPW) a los que se les realizó estudio electrofisiológico (EEF) y ACRF. Empleamos el algoritmo de la polaridad del QRS para predecir la ubicación anatómica de la VA y comparamos este resultado con la ubicación anatómica real determinada en el EEF. Para determinar la precisión se utilizaron el coeficiente kappa de Cohen (k) y el coeficiente de correlación de Pearson. Resultados: Se incluyeron un total de 364 pacientes (edad media 30 años, 57 % varones). La puntuación k global fue de 0,78 y el coeficiente de Pearson de 0,90. También se evaluó la precisión para cada zona, la mejor correlación fue para las VA laterales izquierdas (k de 0.97). Hubo 26 pacientes con VA parahisianas, que mostraron una gran variabilidad en las características del ECG. Empleando el algoritmo de la polaridad del QRS, el 34,6 % de los pacientes tenía una ubicación anatómica correcta, el 42,3 % tenía una ubicación adyacente y solo el 23 % una ubicación incorrecta. Conclusión: El algoritmo de la polaridad del QRS tiene una buena precisión global; su precisión es alta, especialmente para VA lateral izquierdo. Este algoritmo también es útil para la VA parahisiana.
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BACKGROUND: In 1996 Iturralde et al. published an algorithm based on the QRS polarity to determine the location of the accessory pathways (AP), this algorithm was developed before the massive practice of invasive electrophysiology. PURPOSE: To validate the QRS-Polarity algorithm in a modern cohort of subjects submitted to radiofrequency catheter ablation (RFCA). Our objective was to determinate its global accuracy and its accuracy for parahisian AP. METHODS: We conducted a retrospective analysis of patients with Wolff-Parkinson-White (WPW) syndrome who underwent an electrophysiological study (EPS) and RFCA. We employed the QRS-Polarity algorithm to predict the AP anatomical location and we compared this result with the real anatomic location determined in the EPS. To determine accuracy, the Cohen's kappa coefficient (k) and the Pearson correlation coefficient were used. RESULTS: A total of 364 patients were included (mean age 30 years, 57% male). The global k score was 0.78 and the Pearson's coefficient was 0.90. The accuracy for each zone was also evaluated, the best correlation was for the left lateral AP (k of 0.97). There were 26 patients with a parahisian AP, who showed a great variability in the ECG features. Employing the QRS-Polarity algorithm, 34.6% patients had a correct anatomical location, 42.3% had an adjacent location and only 23% an incorrect location. CONCLUSION: The QRS-Polarity algorithm has a good global accuracy; its precision is high, especially for left lateral AP. This algorithm is also useful for the parahisian AP.
ANTECEDENTES: En 1996 Iturralde y colaboradores publicaron un algoritmo basado en la polaridad del QRS para determinar la ubicación de las vías accesorias (VA), este algoritmo fue desarrollado antes de la práctica masiva de la electrofisiología invasiva. OBJETIVO: Validar el algoritmo de la polaridad del QRS en una cohorte moderna de sujetos sometidos a ablación con catéter por radiofrecuencia (ACRF). Nuestro objetivo fue determinar su precisión global y su precisión para las VA parahisianas. MÉTODOS: Realizamos un análisis retrospectivo de pacientes con síndrome de Wolff-Parkinson-White (WPW) a los que se les realizó estudio electrofisiológico (EEF) y ACRF. Empleamos el algoritmo de la polaridad del QRS para predecir la ubicación anatómica de la VA y comparamos este resultado con la ubicación anatómica real determinada en el EEF. Para determinar la precisión se utilizaron el coeficiente kappa de Cohen (k) y el coeficiente de correlación de Pearson. RESULTADOS: Se incluyeron un total de 364 pacientes (edad media 30 años, 57 % varones). La puntuación k global fue de 0,78 y el coeficiente de Pearson de 0,90. También se evaluó la precisión para cada zona, la mejor correlación fue para las VA laterales izquierdas (k de 0.97). Hubo 26 pacientes con VA parahisianas, que mostraron una gran variabilidad en las características del ECG. Empleando el algoritmo de la polaridad del QRS, el 34,6 % de los pacientes tenía una ubicación anatómica correcta, el 42,3 % tenía una ubicación adyacente y solo el 23 % una ubicación incorrecta. CONCLUSIÓN: El algoritmo de la polaridad del QRS tiene una buena precisión global; su precisión es alta, especialmente para VA lateral izquierdo. Este algoritmo también es útil para la VA parahisiana.
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Fascículo Atrioventricular Accesorio , Ablación por Catéter , Síndrome de Wolff-Parkinson-White , Humanos , Masculino , Adulto , Femenino , Estudios Retrospectivos , Electrocardiografía , Síndrome de Wolff-Parkinson-White/diagnóstico , Síndrome de Wolff-Parkinson-White/cirugía , Fascículo Atrioventricular Accesorio/cirugía , AlgoritmosRESUMEN
Resumo Fundamento A síndrome de Wolff-Parkinson-White (WPW) é uma condição pró-arrítmica que pode exigir restrição de atividades extenuantes e é caracterizada por sinais de ECG, incluindo ondas delta. Observamos casos de padrões intermitentes de WPW apresentando-se como QRS alternante ('WPW alternante') em uma grande coorte de triagem de ECG pré-participação de homens jovens que se candidataram ao recrutamento militar. Objetivos Nosso objetivo foi determinar o padrão de WPW alternante, as características do caso e a prevalência de outros diagnósticos diferenciais relevantes apresentando-se como alternância de QRS em um ambiente de pré-participação. Métodos Cento e vinte e cinco mil cento e cinquenta e oito recrutas militares do sexo masculino prospectivos foram revisados de janeiro de 2016 a dezembro de 2019. Uma revisão de prontuários médicos eletrônicos identificou casos de WPW alternante e padrões ou síndrome de WPW. A revisão de prontuários médicos eletrônicos identificou casos de diagnósticos diferenciais relevantes que podem causar alternância de QRS. Resultados Quatro indivíduos (2,2%) apresentaram WPW alternante em 184 indivíduos com diagnóstico final de padrão ou síndrome de WPW. Dois desses indivíduos manifestaram sintomas ou achados eletrocardiográficos compatíveis com taquicardia supraventricular. A prevalência geral de WPW alternante foi de 0,003%, e a prevalência de WPW foi de 0,147%. As WPW alternantes representaram 8,7% dos indivíduos com QRS alternantes, e QRS alternantes tiveram prevalência de 0,037% em toda a população. Conclusões A WPW alternante é uma variante da WPW intermitente, que compreendeu 2,2% dos casos de WPW em nossa coorte de triagem pré-participação. Não indica necessariamente um baixo risco de taquicardia supraventricular. Deve ser reconhecido na triagem de ECG e distinguido de outras patologias que também apresentam QRS alternantes.
Abstract Background Wolff-Parkinson-White (WPW) syndrome is a proarrhythmic condition that may require restriction from strenuous activities and is characterized by ECG signs, including delta waves. We observed cases of intermittent WPW patterns presenting as QRS alternans ('WPW alternans') in a large pre-participation ECG screening cohort of young men reporting for military conscription. Objectives We aimed to determine the WPW alternans pattern, case characteristics, and the prevalence of other relevant differential diagnoses presenting as QRS alternans in a pre-participation setting. Methods One hundred twenty-five thousand one hundred fifty-eight prospective male military recruits were reviewed from January 2016 to December 2019. A review of electronic medical records identified cases of WPW alternans and WPW patterns or syndrome. Reviewing electronic medical records identified cases of relevant differential diagnoses that might cause QRS alternans. Results Four individuals (2.2%) had WPW alternans out of 184 individuals with a final diagnosis of WPW pattern or syndrome. Two of these individuals manifested symptoms or ECG findings consistent with supraventricular tachycardia. The overall prevalence of WPW alternans was 0.003%, and the prevalence of WPW was 0.147%. WPW alternans represented 8.7% of individuals presenting with QRS alternans, and QRS alternans had a prevalence of 0.037% in the entire population. Conclusions WPW alternans is a variant of intermittent WPW, which comprised 2.2% of WPW cases in our pre-participation screening cohort. It does not necessarily indicate a low risk for supraventricular tachycardia. It must be recognized at ECG screening and distinguished from other pathologies that also present with QRS alternans.
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Case report of a 49-year-old patient with Wolff-Parkinson-White syndrome, very symptomatic, with apparent parahisian pathway who, during an electrophysiological study, presented orthodromic atrioventricular tachycardia, featuring two accessory pathways, retrogradely, the parahisian pathway and a hidden left posterolateral pathway, during the same tachycardia, alternating the retrograde pathway of tachycardia without interruption.
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Síndrome de Wolff-Parkinson-White , Ablación por Catéter , Fascículo Atrioventricular AccesorioRESUMEN
INTRODUCTION: Catheter ablation of the parahisian accessory pathways (PHAP) has been established as the definitive therapy for this type of arrhythmia. However, the PHAP proximity to the normal atrioventricular conduction system makes the procedure technically challenging. Here, we have reported a case series of 20 patients with PHAP who underwent aortic access ablation to evaluate the safety and efficacy of this approach in the PHAP ablation. METHODS AND RESULTS: The ablation through the aortic cusps was the successful approach in 13 of 20 (65%) of the cases. In 11 patients, the aortic approach was the initial strategy for ablation, and the accessory pathway was eliminated in seven (63.6%) of them. The aortic approach followed a failed right-sided attempt in nine patients. In six (66.7%) patients, the ablation was successful with the aortic approach. The only independent predictor for the successful ablation with each approach was the earliest ventricular activation before delta wave (predelta time) and a right-sided earliest ventricular activation of more than 23 ms had high sensitivity and specificity for right-sided success. Systematically using the two strategies (right and left approaches), the ablation of the PHAP was successful in 18 (90%) patients. CONCLUSION: The aortic approach seems to be a safe and effective strategy for the ablation of PHAP. It can be used when the right-sided approach fails or even considered as an initial strategy when the predelta time is less than 23 ms in the right septal region. When combining the right- and left-sided approaches, the success rate is high. We believe that the retrograde aortic approach remains a key tool for this challenging ablation.
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Fascículo Atrioventricular Accesorio/cirugía , Arritmias Cardíacas/cirugía , Ablación por Catéter , Técnicas Electrofisiológicas Cardíacas , Fascículo Atrioventricular Accesorio/fisiopatología , Potenciales de Acción , Adulto , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/fisiopatología , Ablación por Catéter/efectos adversos , Electrocardiografía , Femenino , Frecuencia Cardíaca , Humanos , Masculino , Valor Predictivo de las Pruebas , Recurrencia , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
RESUMEN El síndrome de Wolff-Parkinson-White es una cardiopatía no estructural poco frecuente que pertenece a los denominados síndromes de preexcitación ventricular. Está asociada al desarrollo de muerte súbita, pues puede inducir a la aparición de arritmias malignas y su diagnóstico puede establecerse a través de la realización de un electrocardiograma. En caso de presentarse en personas vinculadas a la práctica deportiva, con el desarrollo de ejercicio físico intenso, puede incrementar el riesgo de muerte súbita. Se describe el caso de un atleta de alto rendimiento con diagnóstico de síndrome de Wolf Parkinson White y se presentan las pautas a seguir, según los criterios internacionales para la interpretación del electrocardiograma en deportistas.
ABSTRACT Wolff-Parkinson-White syndrome is a rare and non-structural heart disease, which belongs to the ventricular preexcitation syndromes. It is associated with the development of sudden death, as it can induce the appearance of malignant arrhythmias in the patient and its diagnosis can be established through electrocardiogram. If it takes place in people linked to sport, with the development of intense physical exercise, it can increase the risk of sudden death. The case of a high-performance athlete with a diagnosis of Wolff-Parkinson-White syndrome is described, and the guidelines to be followed according to international criteria for the interpretation of the electrocardiogram in sportsmen and women are presented.
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Síndrome de Wolff-Parkinson-White , Muerte Súbita , Electrocardiografía , AtletasRESUMEN
Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy characterized by prominent left ventricular (LV) trabeculae, deep intertrabecular recesses, and the thin compacted layer. The disease is potentially associated with sudden cardiac death due to LV dysfunction and ventricular arrhythmias. The presence of accessory pathway and Wolff-Parkinson-White syndrome is particularly rare in adults. Here we describe the rare association of LVNC and ventricular pre-excitation in an 18-year-old female with neonatal hypoxic brain injury (AU)
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Humanos , Femenino , Adolescente , No Compactación Aislada del Miocardio Ventricular/diagnóstico , Síndrome de Wolff-Parkinson-White/diagnóstico , Cardiomiopatías/diagnóstico , Cardiopatías/diagnóstico , Ventrículos Cardíacos/fisiopatología , No Compactación Aislada del Miocardio Ventricular/fisiopatologíaRESUMEN
Abstract Background: There are currently several electrocardiographic algorithms to locate the accessory pathway (AP) in patients with Wolff-Parkinson-White (WPW) syndrome. Objective: To compare the ability of electrocardiographic algorithms in identifying the location of the AP in patients with WPW pattern referred for ablation. Methods: Observational, cross-sectional, retrospective study with 111 patients with WPW syndrome referred for AP ablation. The electrocardiogram (ECG) obtained prior to the ablation was analyzed by an experienced observer who consecutively applied seven algorithms to identify non-invasively the AP. We then compared the location estimated with this assessment with that obtained in the electrophysiological study and calculated the agreement rates. Results: Among the APs, 59 (53.15%) were distributed around the mitral annulus and the remaining 52 (46.85%) were located around the tricuspid annulus. The overall absolute accuracy of the algorithms evaluated varied between 27% and 47%, increasing to between 40% and 76% when we included adjacent locations. The absolute agreement rate by AP location was 2.00-52.20% for septal APs (n = 51), increasing to 5.90-90.20% when considering adjacent locations; 7.70-69.20% for right APs (n = 13), increasing to 42.90-100% when considering adjacent locations; and 21.70-54.50% for left APs (n = 47), increasing to 50-87% when considering adjacent locations. Conclusion: The agreement rates observed for the analyzed scores indicated a low discriminative ability of the ECG in locating the AP in patients with WPW.
Resumo Fundamento: Existem atualmente vários algoritmos eletrocardiográficos para localizar a via acessória (VA) em pacientes com síndrome de Wolff-Parkinson-White (WPW). Objetivo: Comparar a capacidade discriminativa dos algoritmos eletrocardiográficos na localização da VA no padrão de WPW em pacientes encaminhados para ablação. Métodos: Estudo observacional, transversal e retrospectivo, incluindo 111 pacientes com síndrome de WPW encaminhados para ablação da VA. O eletrocardiograma (ECG) prévio à ablação foi analisado por um observador experiente que aplicou consecutivamente sete algoritmos para identificar a VA de forma não invasiva. A localização estimada com esta avaliação foi comparada à obtida no estudo eletrofisiológico e as taxas de acerto foram calculadas. Resultados: Entre as VAs, 59 (53,15%) estavam distribuídas ao redor do anel mitral e as restantes 52 (46,85%) em torno do anel tricúspide. O acerto global absoluto dos algoritmos em estudo variou entre 27% e 47%, aumentando para 40% a 76% quando incluímos localizações adjacentes. O acerto absoluto em função da localização da VA foi o seguinte: para as VAs septais (n = 51) variou entre 2% e 52,20% (5,90% e 90,20% incluindo localizações adjacentes), para as VAs direitas (n = 13) variou entre 7,70% e 69,20% (42,90% e 100%, incluindo localizações adjacentes), para as VAs esquerdas (n = 47) variou entre 21,70% e 54,50% (50% a 87%, incluindo as localizações adjacentes). Conclusões: Os índices de acerto observados para os escores analisados indicaram uma reduzida capacidade discriminativa do ECG na localização da VA em pacientes com WPW.
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Humanos , Masculino , Femenino , Persona de Mediana Edad , Adulto Joven , Síndrome de Wolff-Parkinson-White/diagnóstico , Algoritmos , Electrocardiografía/métodos , Fascículo Atrioventricular Accesorio/diagnóstico , Estándares de Referencia , Valores de Referencia , Síndrome de Wolff-Parkinson-White/fisiopatología , Estudios Transversales , Reproducibilidad de los Resultados , Estudios Retrospectivos , Ablación por Catéter , Estadísticas no Paramétricas , Fascículo Atrioventricular Accesorio/fisiopatologíaRESUMEN
BACKGROUND: Epicardial mapping and ablation of accessory pathways through a subxiphoid approach can be an alternative when endocardial or epicardial transvenous mapping has failed. METHODS AND RESULTS: We reviewed acute and long-term follow-up of 21 patients (14 males) referred for percutaneous epicardial accessory pathway ablation. There was a median of 2 previous failed procedures. All patients were highly symptomatic, 8 had atrial fibrillation (3 with cardiac arrest) and 13 had frequent symptomatic episodes of atrioventricular reentrant tachycardia. Six patients (28.5%) had a successful epicardial ablation. Five patients (23.8%) underwent a successful repeated endocardial mapping, and ablation after epicardial mapping yielded no early activation site. Epicardial mapping was helpful in guiding endocardial ablation in 2 patients (9.5%), showing that the earliest activation was simultaneous at the epicardium and endocardium. Four patients (19%) underwent successful open-chest surgery after failing epicardial/endocardial ablation. Two patients (9.5%) remained controlled under antiarrhythmic drugs after unsuccessful endocardial/epicardial ablation. Two patients had a coronary sinus diverticulum and one a right atrium to right ventricle diverticulum. Three patients acquired postablation coronary sinus stenosis. There was no major complication related to pericardial access. CONCLUSIONS: Percutaneous epicardial approach is an alternative when conventional endocardial or transvenous epicardial ablation fails in the elimination of the accessory pathway. A new attempt by endocardial approach was successful in a significant number of patients. Open-chest surgery may be required in symptomatic cases refractory to endocardial-epicardial approach.
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Fascículo Atrioventricular Accesorio/cirugía , Arritmias Cardíacas/cirugía , Ablación por Catéter/métodos , Sistema de Conducción Cardíaco/cirugía , Pericardio/cirugía , Fascículo Atrioventricular Accesorio/diagnóstico , Fascículo Atrioventricular Accesorio/fisiopatología , Adolescente , Adulto , Anciano , Antiarrítmicos/uso terapéutico , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/fisiopatología , Brasil , Ablación por Catéter/efectos adversos , Mapeo Epicárdico , Femenino , Sistema de Conducción Cardíaco/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Pericardio/fisiopatología , Valor Predictivo de las Pruebas , Reoperación , Insuficiencia del Tratamiento , Adulto JovenRESUMEN
The association between Brugada syndrome (BS) and ventricular preexcitation is a rare condition, with sporadic cases already reported. We report the case of a 29-year-old man, with palpitation unrelated to physical or emotional stress. The electrocardiogram of the first visit revealed a ventricular preexcitation pattern and an end-conduction delay, with negative T wave in V1 and intraventricular conduction disturbance in V2 (atypical for BS). The typical aspect of BS occurred after introduction of propafenone for the prevention of atrioventricular tachycardia. We discuss the recognition of this rare association, the proarrhythmic effects of some drugs, treatment options, and prognosis.
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Síndrome de Brugada/complicaciones , Síndrome de Wolff-Parkinson-White/complicaciones , Adulto , Síndrome de Brugada/fisiopatología , Síndrome de Brugada/cirugía , Ablación por Catéter , Electrocardiografía , Humanos , Masculino , Pronóstico , Síndrome de Wolff-Parkinson-White/fisiopatología , Síndrome de Wolff-Parkinson-White/cirugíaRESUMEN
BACKGROUND: Bundle branch block (BBB) is a difficult diagnosis in the Wolff-Parkinson-White syndrome (WPW). We investigated the clinical implications of BBB that appears after performing an accessory pathway (AP) ablation. METHODS: We studied 199 patients with WPW who were submitted to AP ablation. Thirty (15%) exhibited BBB after the ablation. Twenty-two patients had right BBB and 8 had left BBB. Thirteen patients had right-sided AP and 17 had left-sided AP. They were compared with 82 similar patients without BBB after the AP ablation. RESULTS: Among the patients with BBB, 86.66% showed delays in the middle part of the QRS in the ECG recorded before ablation vs. 18.29% of the patients without BBB (p<0.05) (sensitivity 86%, specificity 81%, positive predictive value 67% and negative predictive value 93%). Forty-four percent of the patients with BBB had BBB morphology during orthodromic tachycardia vs. 10% of the patients without BBB (p<0.05) (sensitivity 44%, specificity 89%, positive predictive value 57% and negative predictive value 82%). No relationship was found between AP location and the site of the BBB. Ejection fraction was normal before (0.61 ± 0.03) and upon completion of follow-up (0.61 ± 0.07). BBB disappeared in 95.3% of the patients. CONCLUSIONS: Delays in the middle portion of the QRS may predict BBB after AP ablation. BBB after performing AP ablation is frequent, transient, benign, and not related to either the ablation lesion location or progression to structural heart disease. BBB after AP ablation may be related to cardiac memory.
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Bloqueo de Rama/diagnóstico , Bloqueo de Rama/fisiopatología , Ablación por Catéter/tendencias , Síndrome de Wolff-Parkinson-White/fisiopatología , Síndrome de Wolff-Parkinson-White/cirugía , Adolescente , Adulto , Anciano , Ablación por Catéter/efectos adversos , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Adulto JovenRESUMEN
Primeiramente relatada por Wilhelm Ebstein em 1866,a anomalia de Ebstein corresponde a uma doença cardíacacongênita incomum, levando a uma má formaçãoda valva tricúspide. Pode se apresentar com dispnéia,cianose, arritmias, cardiomegalia e insuficiência ventriculardireita. O ecocardiograma confirma o diagnóstico.Descrevemos o caso de uma paciente feminina, 38 anos,com hipertensão arterial sistêmica e fibromialgia, apresentandoSíndrome de Wolff-Parkinson-White associadaà anomalia de Ebstein.
First reported by Wilhelm Ebstein in 1866, Ebstein´sAnomaly is an unusual congenital cardiac disease, leadingto a tricuspid valve bad formation. It can presentsdyspnea, cyanosis, arrythmia, cardiomegaly and rightventricular failure. Echocardiography firms the diagnosis.We describe a female patient, 38 years old, withsystemic arterial hypertension and fibromyalgia, presentingWolff-Parkinson-White Syndrome associated withEbstein´s Anomaly.
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Relata-se o caso de uma criança de nove anos de idade, com quadro de síncope havia dois anos, com eletrocardiograma compatível com síndrome de Wolff-Parkinson-White e história familiar de uma irmã com síncope vasovagal. A paciente foi submetida ao estudo eletrofisiológico para estratificação de risco e ablação da via anômala. Após ablação,permaneceu com os mesmos sintomas, o que a fizeram procurar atendimento médico. Foi então solicitado teste de inclinação (tilt test), o qual foi positivo para síncope vasovagal do tipo mista. Portanto, conclui-se ser a causa dos desmaios a síncope vasovagal, e as alterações eletrocardiográficas compatíveis com Wolff-Parkinson-White seremapenas um achado casual. Após dois anos da primeira consulta, ela se encontra com aumento dos intervalos entre os episódios de síncope.
The authors report a case of a nine years old child presented with syncope for about two years, with electrocardiogram compatible with Wolff-Parkinson-White and a family history revealing a sister with vasovagal syncope. The patient underwent electrophysiological study for risk stratification and ablation of the anomalous pathway. Afterthe ablation procedure, she remained with the same symptoms that caused her to initially seek medical attention, and then performed the tilt test, which was positive for vasovagal syncope of the mixed type. Therefore, the vasovagal syncope was concluded to be the syncope´s cause, and the electrocardiographic changes compatible withWolff Parkinson White were just an incidental finding. Two years after the first consultation, she finds herself with an increased interval between syncopal episodes.