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BACKGROUND: Chagas cardiomyopathy (CCM) is increasingly prevalent in developed countries due to migration from endemic areas. Accurate risk stratification is crucial due to the variable clinical course of CCM. OBJECTIVE: To analyze the association between Rassi score progression and electrophysiology study (EPS) changes in CCM patients. METHODS: This prospective, observational cohort study involved CCM patients from two tertiary hospitals. Patients were classified as low, intermediate, or high risk based on the Rassi score. Data collected included demographics, clinical history, and diagnostic tests. EPS assessed AH, HH, and HV intervals, and inducibility of ventricular arrhythmias. Follow-ups were at 30 days and six-month intervals, with individualized discussions for cardiac implantable electric devices (CIED) based on EPS results. RESULTS: Of 67 screened CCM patients, 59 underwent EPS. The mean Rassi score was 8.7 ± 4.5 points, with 33.8 % low, 38.9 % intermediate, and 27.1 % high risk. EPS abnormalities were found in 57.6 % of patients, mainly VT/VF (52.5 %). Most induced ventricular arrhythmias were monomorphic VT (80.7 %). A significant association was found between Rassi score risk classification and EPS changes (OR = 1.88 95 %CI: 1.15-3.06 p = 0.02). Higher Rassi scores correlated with VT presence on EPS (p = 0.0036). Syncope/pre-syncope had an OR 2.45 95 %CI:1.21-4.94; p = 0.012, independent of Rassi risk. Decreased ejection fraction was linked to EPS changes (p = 0.04). CONCLUSION: EPS changes among CCM was associated with progression of the Rassi score, indicating its utility as a stratification tool. Factors such as the presence of syncope/pre-syncope, decreased LVEF and wall motion abnormalities emerged as independent predictors within Rassi scores for changes in EPS.

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Background: Exercise-induced complete atrioventricular block (EIAVB) is a rare cardiac conduction abnormality presenting challenges in diagnosis due to non-specific symptoms such as exertional dyspnoea, dizziness, and syncope. Case summary: We present a case of a 76-year-old female with recurrent exercise-associated syncope. Non-invasive exercise testing played a crucial role in diagnosing her condition, revealing EIAVB and underscoring its importance in patients with cardiovascular risk factors. Discussion: This case provides insight into the pathophysiology of EIAVB, including altered atrioventricular nodal refractoriness and exercise-induced ischaemic imbalances. It highlights the need for heightened clinical vigilance in diagnosing exercise-related syncope, especially in pre-existing cardiovascular conditions. This case underscores the critical importance of non-invasive testing for diagnosing EIAVB, highlighting the necessity of thorough evaluation in patients presenting with ambiguous symptoms and cardiovascular risks. Consequently, it advocates for adherence to guidelines to enhance outcomes and reduce the need for unnecessary invasive procedures.

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BACKGROUND: Cardioneuroablation (CNA) is a novel therapeutic approach for functional bradyarrhythmias, specifically neurocardiogenic syncope or atrial fibrillation, achieved through endocardial radiofrequency catheter ablation of vagal innervation, obviating the need for pacemaker implantation. Originating in the nineties, the first series of CNA procedures was published in 2005. Extra-cardiac vagal stimulation (ECVS) is employed as a direct method for stepwise denervation control during CNA. OBJECTIVE: This study aimed to compare the long-term follow-up outcomes of patients with severe cardioinhibitory syncope undergoing CNA with and without denervation confirmation via ECVS. METHOD: A cohort of 48 patients, predominantly female (56.3%), suffering from recurrent syncope (5.1 ± 2.5 episodes annually) that remained unresponsive to clinical and pharmacological interventions, underwent CNA, divided into two groups: ECVS and NoECVS, consisting of 34 and 14 cases, respectively. ECVS procedures were conducted with and without atrial pacing. RESULTS: Demographic characteristics, left atrial size, and ejection fraction displayed no statistically significant differences between the groups. Follow-up duration was comparable, with 29.1 ± 15 months for the ECVS group and 31.9 ± 20 months for the NoECVS group (p = .24). Notably, syncope recurrence was significantly lower in the ECVS group (two cases vs. four cases, Log Rank p = .04). Moreover, the Hazard ratio revealed a fivefold higher risk of syncope recurrence in the NoECVS group. CONCLUSION: This study demonstrates that concluding CNA with denervation confirmation via ECVS yields a higher success rate and a substantially reduced risk of syncope recurrence compared to procedures without ECVS confirmation.

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Abstract Background: Vasovagal syncope (VVS) results in impaired quality of life (QoL). The response during the head-up tilt test (HUTT) influences QoL and recurrence. Objectives: To analyze the influence of the type of HUTT response on QoL in patients with VVS and recurrence of events after the exam. Methods: The SF-36 and Impact of Syncope on Quality of Life (ISQL) questionnaires were applied over 12 months after the HUTT. Unpaired Student's t test was used for differences between 2 groups of quantitative data with normal distribution. The recurrence of syncope episodes was analyzed using a Kaplan-Meier curve, and the log-rank test was applied to compare the curves regarding responses to the HUTT. Statistical significance was set at p value < 0.05. Results: We analyzed 82 patients (43.7 years old), 69% with previous recurrence (2.8 prior episodes). Cardioinhibitory response occurred in 46 patients; vasodepressor response occurred in 36, and 85.4% of patients received non-pharmacological treatment after the HUTT. During clinical follow-up, 43.9% had recurrence, mainly young patients (35.7 years; p = 0.002). On the SF-36, the best score was in functional capacity in men (p = 0.04) and patients without prior trauma (p = 0.001). There were lower limitations due to pain in patients without prior trauma (p = 0.003) and patients without prodromes (p = 0.009). On the ISQL, there were better mean scores in men (p = 0.002) and in patients without prior trauma (p = 0.02). Patients with cardioinhibitory response had better SF-36 and ISQL scores (p < 0.001). There was greater VVS recurrence in the cardioinhibitory response group (log-rank p = 0.011; hazard ratio: 8.48; 95% confidence interval: 7.59 to 9.3) from the second to the fourth month, with stabilization in the eighth month after the HUTT, when compared to patients with vasodepressor response. Conclusion: The majority of patients with VVS reproduced during the HUTT under non-pharmacological treatment did not report worsening of QoL during clinical follow-up. Worse QoL was observed in non-young patients and in patients with vasodepressor response, and it was not influenced by recurrence after the HUTT.

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BACKGROUND: Functional bradycardia is a challenging condition that affects a healthy population. Ganglionated plexus ablation has emerged as a therapeutic alternative to avoid a pacemaker. OBJECTIVES: The purpose of this study is to evaluate long-term effects of anatomically guided cardiac denervation. METHODS: This is a prospective longitudinal study that included 36 patients with symptomatic functional bradycardia. Electroanatomic reconstruction of both atria was carried out, and the main septal ganglionated plexi were anatomically located and targeted. RESULTS: Ablation endpoints were: 1) heart rate increment; 2) Wenckebach cycle length shortening; and 3) atrio-Hisian (AH) interval shortening. A sinus node denervation was obtained in all patients with an increment of 21.6% in the mean heart rate. All patients presented a negative atropine test after ablation. Twenty-eight (77.7%) patients presented immediate sings of atrioventricular node denervation, with a shortening of 15.6% of mean Wenckebach cycle length and 15.9% of the mean AH interval. All heart rate variability parameters showed a significant reduction after 12 months, enduring after 18 months. Thirty (83.3%) patients remained free of events after a mean follow-up of 52.1 ± 35.2 months. One patient (2.77%) presented acute sinus node artery occlusion during ablation with persistent sinus dysfunction and had a pacemaker implantation; 3 (8.3%) other patients evolved with sinus tachycardia, and 4 (11.1%) patients presented syncope recurrence during follow-up, 3 (8.3%) of them requiring a pacemaker implantation. No other tachyarrhythmia was observed. CONCLUSIONS: The anatomically guided septal approach is an effective technique for syncope prevention, promoting long-lasting autonomic changes. No significant proarrhythmia effect has been observed during the long-term follow-up.

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Since its original description in 2005, catheter ablation techniques, commonly called cardioneuroablation, have emerged as a potential strategy for modulating autonomic function. Multiple investigators have provided observational data on the potential benefits of this technique in a variety of conditions associated with or exacerbated by increased vagal tone such as vasovagal syncope, functional atrioventricular block, and sinus node dysfunction. Patient selection, current techniques including the various mapping strategies, clinical experience, and limitations of cardioablation are reviewed. Finally, while cardioneuroablation has potential to be a treatment option for selected patients with symptoms mediated by hypervagotonia, the document outlines the important knowledge gaps that currently exist and the necessary next steps required before this technique can be widely implemented into clinical practice.

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The tachycardia-bradycardia syndrome is the most frequent form of presentation of Sick sinus node syndrome and is commonly characterized by episodes of paroxysmal atrial fibrillation followed by significant pauses, especially in older adult patients. Other frequently associated tachyarrhythmias are atrial tachycardia and atrial flutter. The association between orthodromic tachycardia and significant pauses in these patients is an unusual presentation. We present the case of an older adult with bradycardia-tachycardia syndrome and syncope, who presented with a hidden accessory pathway and who, after successful ablation of it, did not present syncope again.

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Resumo Fundamento A síncope, na população pediátrica, tem como sua principal causa, a vasovagal (SVV). Sua avaliação deve ser feita por métodos clínicos e o teste de inclinação (TI) pode contribuir para seu diagnóstico. Objetivos Analisar o perfil clínico, os escores de Calgary e de Calgary modificado, a resposta ao TI e a variabilidade da frequência cardíaca (VFC) de pacientes ≤ 18 anos de idade, com presumida SVV. Comparar as variáveis entre pacientes com resposta positiva e negativa ao TI. Método Estudo observacional e prospectivo, com 73 pacientes com idades entre 6 e 18 anos, submetidos à avaliação clínica e ao cálculo dos escores, sem o conhecimento do TI. Este foi feito a 70º sob monitoramento para análise da VFC. Valor-p < 0,05 foi considerado como o critério de significância estatística. Resultados A mediana de idade foi de 14,0 anos, sendo que 52% eram no sexo feminino, 72 apresentaram Calgary ≥ -2 (média 1,80) e 69 com Calgary modificado ≥ -3 (média 1,38). Ocorreram pródromos em 59 pacientes, recorrência em 50 e trauma em 19. A resposta ao TI foi positiva em 54 (49 vasovagal, com 39 vasodepressora), com aumento do componente de baixa frequência (BF) e diminuição da alta frequência (AF) (p < 0,0001). Na posição supina, o BF foi de 33,6 no sexo feminino e 47,4 em unidades normalizadas no sexo masculino (p = 0,02). Aplicando-se a curva de operação característica para TI positivo, não houve significância estatística para VFC e os escores. Conclusões A maioria das crianças e adolescentes com diagnóstico presumido de SVV apresentaram um cenário clínico típico, com escore de Calgary ≥ -2, e resposta vasodepressora predominante ao TI. Verificou-se uma maior ativação simpática na posição supina no sexo masculino. Os escores de Calgary e a ativação simpática não permitiram predizer a resposta ao TI.

Abstract Background In the pediatric population, syncope is mainly from vasovagal (VVS) origin. Its evaluation must be done by clinical methods, and the tilt test (TT) can contribute to the diagnosis. Objectives To analyze the clinical profile, Calgary and modified Calgary scores, response to TT and heart rate variability (HRV) of patients aged ≤ 18 years with presumed VVS. To compare the variables between patients with positive and negative responses to TT. Method Observational and prospective study, with 73 patients aged between 6 and 18 years, submitted to clinical evaluation and calculation of scores without previous knowledge of the TT. It was done at 70º under monitoring for HRV analysis. P-value < 0.05 was the statistical significance criterion. Results Median age was 14.0 years; 52% of participants were female, 72 had Calgary ≥ -2 (mean 1.80), and 69 had modified Calgary ≥ -3 (mean 1.38). Prodromes were observed in 59 patients, recurrence in 50 and trauma in 19. The response to TT was positive in 54 participants (49 vasovagal, with 39 vasodepressor responses), with an increase in the low frequency (LF) component and a decrease in the high frequency (HF) component (p < 0,0001). In the supine position, LF was 33.6 in females and 47.4 in normalized units for males (p = 0.02). When applying the operating characteristic curve for positive TT, there was no statistical significance for HRV and scores. Conclusion Most children and adolescents with a presumed diagnosis of VVS presented a typical clinical scenario, with a Calgary score ≥ -2, and a predominant vasodepressor response to TT. Greater sympathetic activation was observed in the supine position in males. Calgary scores and sympathetic activation did not predict the response to TT.

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Introducción:El síncope vasovagal es la principal causa de pérdida transitoria de la conciencia, y es un motivo de consulta cada vez más frecuente en pediatría y medicina del adulto. La midodrina es un agonista de los recepto-res alfa, de acción periférica, empleada principalmente en el manejo de la hipotensión ortostática; sin embargo, también se ha evaluado en el síncope vasovagal, con resultados prometedores.Objetivo:Analizar la evidencia más reciente sobre la utilidad de la midodrina para el control y la prevención del síncope vasovagal.Materiales y métodos: Se realizó una búsqueda bibliográfica utilizando términos de búsqueda como Vasovagal Syncope y Midodrine, así como sinónimos, que se combinaron con operadores booleanos, en cinco bases de datos, hasta octubre del 2022. Se incluyeron estudios originales, revisiones sistemáticas y metanálisis, publicados tanto en inglés como en español.Resultados:Ensayos controlados aleatorizados y revisiones sistemáticas y metanálisis difieren ligeramente entre resultados, pero estos demuestran un efecto global protector. La evidencia más reciente y completa indica que utilizar este agente reduce significativamente la positividad al realizar la prueba de la mesa inclinada y que previene la aparición de episodios sincopales.Conclusiones:Aunque la evidencia actual sobre la eficacia de la midodrina respecto a la prevención y control del síncope vasovagal es limitada, se observa un efecto protector significativo, porque disminuye el riesgo de sufrir un episodio sincopal, aproximadamente hasta en un 50 %.Palabras clave: midodrina; síncope vasovagal; síncope; adrenérgicos; medicina basada en la evidencia

Introduction: Vasovagal syncope is the main cause of transient loss of consciousness, being an in-creasingly frequent reason for consultation in pediatrics and adult medicine. Midodrine, a periphe-rally acting alpha-receptor agonist, is mainly used in the management of orthostatic hypotension. However, it has also been evaluated in vasovagal syncope, with promising results. Objective: To analyze the most recent evidence on the usefulness of midodrine for the control and prevention of vasovagal syncope. Materials and Methods: A literature search was performed using search terms such as "Vasovagal Syncope" and "Midodrine," as well as synonyms, which were combined with Boolean operators, in 5 databases until October 2022. Original studies, systematic reviews and meta-analyses, published in both English and Spanish, were included. Results: Randomized controlled trials and systematic reviews and meta-analyses differ slightly between results, but these demonstrate an overall protective effect. The most recent and complete evidence shows that using this agent significantly reduces the probability of positivity when performing the tilt table test and prevents the occurrence of syncopal episodes. Conclusions: Although current evidence on the efficacy of midodrine with respect to the prevention and control of vasovagal syncope is limited, a significant protective effect is observed, reducing the risk of suffering syncopal episode by approximately up to 50%

Introdução: a síncope vasovagal é a principal causa de perda transitória de consciência e é um motivo cada vez mais comum de consulta em pediatria e medicina de adultos. A midodrina é um agonista do receptor alfa de ação periférica usado principalmente no tratamento da hipotensão ortostática; no entanto, ela também foi avaliada na síncope vasovagal, com resultados promissores. Objetivo: Revisar as evidências mais recentes sobre a utilidade da midodrina para o controle e a pre-venção da síncope vasovagal. Materiais e métodos: Foi realizada uma pesquisa na literatura usando termos de pesquisa como Va-sovagal, Syncope e Medodrine, bem como sinônimos, que foram combinados com operadores boo-leanos, em cinco bancos de dados, até outubro de 2022. Foram incluídos estudos originais, revisões sistemáticas e metanálises, publicados em inglês e espanhol. Resultados: Os ensaios clínicos randomizados, as revisões sistemáticas e as metanálises diferem ligei-ramente entre os resultados, mas demonstram um efeito protetor geral. As evidências mais recentes e abrangentes indicam que o uso desse agente reduz significativamente a positividade no teste de inclinação da mesa e evita a ocorrência de episódios de síncope. Conclusões: Embora as evidências atuais sobre a eficácia da midodrina em relação à prevenção e ao controle da síncope vasovagal sejam limitadas, observa-se um efeito protetor significativo, pois ela diminui o risco de sofrer um episódio sincopal em aproximadamente 50%

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SUMMARY BACKGROUND: Investigation of syncope involves the use of electrophysiological study, particularly in patients with cardiac conduction disorder. There is conflicting evidence about the role of electrophysiological study in patients with Chagas disease. OBJECTIVE: The objective of this study was to evaluate the electrophysiological study findings in patients with Chagas disease and bundle branch block and/or divisional block presenting with syncope. METHODS: This is a retrospective study of patients with Chagas disease and cardiac conduction disorder who underwent electrophysiological study from 2017 to 2021 for the investigation of syncope in a tertiary hospital in São Paulo, Brazil. Those with non-interpretable ECG, known coronary artery disease, and/or other cardiomyopathies were excluded. HV interval and electrophysiological study-induced malignant ventricular arrhythmias data were analyzed. RESULTS: A total of 45 patients (60.2±11.29 years, 57.8% males) were included. The mean HV interval was 58.37 ms±10.68; 22.2% of the studied population presented an HV interval of ≥70 ms; and malignant ventricular arrhythmias were induced in 57.8% patients. The use of beta-blockers and amiodarone (p=0.002 and 0.036, respectively), NYHA functional class≥II (p=0.013), wide QRS (p=0.047), increased HV interval (p=0.02), Rassi score >6.5 (p=0.003), and reduced left ventricular ejection fraction (p=0.031) were associated with increased risk of inducible malignant ventricular arrhythmias. CONCLUSION: More than half of the patients with Chagas disease, syncope, and cardiac conduction disorder have inducible malignant ventricular arrhythmias. Prolonged HV interval was observed in only 20% of population. Wide QRS, prolonged HV, reduced ejection fraction, and higher Rassi score were associated with increased risk of malignant ventricular arrhythmias.

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La enfermedad de Addison es un trastorno raro que afecta progresivamente a las glándulas suprarrenales. La etiología es variada siendo su principal causa autoinmune, el diagnóstico se obtiene mediante valores de laboratorio y exploración clínica. Se expone el caso de paciente femenino que en consulta presentó un evento sincopal, en la revisión clínica se observó pigmentación de mucosas orales y uñas de manos y pies; los valores de laboratorio mostraron: ACTH elevada, cortisol disminuido y alteraciones de electrolitos, confirmándose Enfermedad de Addison. Un diagnóstico oportuno evita complicaciones mortales en quien la padece.

Addison's disease is a rare disorder that progressively affects the adrenal glands. The etiology is varied, being its main autoimmune cause, the diagnosis is obtained through laboratory values and clinical examination. The case of a female patient who presented a syncopal event in the consultation is exposed. In the clinical review, pigmentation of the oral mucosa and fingernails and toenails was observed; Laboratory values showed: elevated ACTH, decreased cortisol and electrolyte disturbances, confirming Addison's disease. A timely diagnosis prevents fatal complications in those who suffer from it.

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Autonomic dysfunction related to severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection is increasingly described in the literature. We report the case of a 30-year-old male with a background of asthma and migraine who experienced a second episode of SARS-CoV-2 infection characterized by mild respiratory symptoms. Twenty-four days after the symptom onset, he developed acute syncope. A tilt test revealed a neuromediated cardioinhibitory response with asystole (Vasovagal Syncope International Study ­ VASIS type 2B). The temporal association between SARS-CoV-2 infection and syncope seems to indicate a probable causal relationship, which requires corroboration by future studies.

Disfunção autonômica relacionada à infecção por coronavírus-2 da síndrome respiratória aguda grave (SARS-CoV-2) vem sendo cada vez mais descrita na literatura. Relatamos o caso de um homem de 30 anos de idade, com histórico de asma e enxaqueca, que apresentou um segundo episódio de infecção por SARS-CoV-2 caracterizado por sintomas respiratórios leves. Vinte e quatro dias após o início dos sintomas, desenvolveu um quadro agudo de síncope. Um teste de inclinação revelou uma resposta cardioinibitória neuromediada com assistolia (Vasovagal Syncope International Study ­ VASIS tipo 2B). A associação temporal entre infecção por SARS-CoV-2 e síncope parece indicar uma provável relação causal, a qual requer corroboração por estudos futuros.

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Background: Vasovagal syncope (VVS) is the most common cause of syncope. Some stages of its pathophysiological mechanisms remain unclear. Vasoactive substances such as nitric oxide metabolites (NOx) and endothelin (ET) may be involved during acute orthostatic stress. Objective: To analyze plasma changes in NOx and ET and heart rate variability (HRV) in the supine positions (T1) and during the head-up tilt test (HUTT) (T2), in patients with VVS (case group) and control group. Methods: Thirty-seven patients (17 in the case group and 20 in the control group), matched for age and sex (mean aged 31.8 years) underwent HUTT with simultaneous HRV recording and venipuncture. Blood samples were collected during phases T1 and T2 and the analysis was performed without knowledge of the HUTT result. Results: In the total sample, there was an increase in NOx values (P = .014), however there was no increase in ET values from phase T1 to phase T2. Patients with VVS tended to increase plasma NOx values (P = .057) and had significantly higher plasma values compared to ET (P = .033) between phases T1 to T2. In the control group, there was no significant change in the values of these vasoactive substances. Regarding HRV, there were a decrease in the component HF (high frequency) and increased of the LF (low frequency)/HF ratio during HUTT. Conclusions: There was an increase in ET during HUTT occurred only in the case group. These patients are more likely to have an imbalance between antagonistic vasoactive biomarkers during orthostatic stress.