Clinical characteristics of anomalous aortic origin of a coronary artery in children / 中华儿科杂志

Objective: To analyze the clinical characteristics, diagnosis and treatment of anomalous aortic origin of a coronary artery (AAOCA) in children. Methods: There were 17 children diagnosed with AAOCA from January 2013 to January 2022 in Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine.Their clinical manifestations, laboratory and imaging data, treatment and prognosis were retrospectively analyzed. Results: These 17 children included 14 males and 3 females, with the age of (8.7±3.5) years. There were 4 anomalous left coronary artery (ALCA) and 13 anomalous right coronary artery (ARCA). Seven children presented with chest pain or chest pain after exercise, three patients presented with cardiac syncope, one complained chest tightness and weakness, and the other six patients had no specific symptoms. Cardiac syncope and chest tightness occurred in patients with ALCA. Fourteen children had the dangerous anatomical basis of myocardial ischemia caused by coronary artery compression or stenosis on imaging. Seven children had coronary artery repair, of whom two were ALCA and five were ARCA. One patient had received heart transplantation because of heart failure. The incidence of adverse cardiovascular events and poor prognosis in ALCA group was higher than that in ARCA group (4/4 vs. 0/13, P<0.05). They were followed up in the outpatient department regularly for 6 (6, 12) months; except for the one who lost visit, the rest of the patients had a good prognosis. Conclusions: Cardiogenic syncope or cardiac insufficiency usually occurs in ALCA, and adverse cardiovascular events and poor prognosis are more common in ALCA than in ARCA. Early surgical treatment should be considered for children with ALCA and ARCA accompanied by myocardial ischemia.

Risk factors associated with malignant vasovagal syncope in children / 中华儿科杂志

Objective: To analyze the clinical characteristics and risk factors of malignant vasovagal syncope (VVS) in children. Methods: This was a case-control study. The data of 368 VVS patients who were treated in the Department of Cardiology, Children's Hospital, Capital Institute of Pediatrics from June 2017 to December 2021 was collected and analyzed. They were divided into malignant VVS group and non-malignant VVS group according to the presence of sinus arrest, and then their demographic characteristics were compared. The children with malignant VVS and complete clinical information were recruited into the case group and were matched by age and sex (1∶4 ratio) with non-malignant VVS patients during the same period.Their clinical characteristics and lab tests were compared. Independent sample t test, Mann Whitney U or χ2 test was used for comparison between groups.Logistic regression was used to analyze the risk factors for malignant VVS in children. Results: Eleven malignant VVS and 342 non-malignant VVS met the inclusion and exclusion critera. Eleven malignant VVS and 44 non-malignant children were recruited in the case-control study. Ten patients of the 11 malignant VVS had a cardiac arrest occurring at 35 (28, 35) minutes of the head-up tilt test, and the duration of sinus arrest was (9±5) s. One patient had syncope occurring while waiting for drawing blood, and the duration of sinus arrest was 3.4 s. The children with malignant vasovagal syncope were younger than non-malignant VVS patients (9 (7, 10) vs. 12 (10, 14) years old, P<0.05), and had higher mean corpuscular hemoglobin concentration (MCHC) and standard deviation of the mean cardiac cycle over 5-minute period within 24 hours ((347±9) vs. (340±8) g/L, (124±9) vs. (113±28) ms, both P<0.05). Logistic regression analysis showed that MCHC was an independent risk factor for malignant VVS in pediatric patients (OR=1.13, 95%CI 1.02-1.26, P=0.024). Conclusions: The onset age of malignant VVS was younger, with no other special clinical manifestations. MCHC was an independent risk factor for malignant VVS.

Síncope por mixoma auricular: a propósito de un caso clínico / Syncope due to atrial myxoma: presentation of a clinical case / Síncope por mixoma atrial: a propósito de um caso clínico

Las neoplasias cardíacas se dividen en tumores primarios y secundarios; estos últimos son 30 veces más frecuentes. La mayoría de las neoplasias cardíacas son benignas y dentro de este grupo destaca el mixoma auricular. La presentación clínica es muy variable en relación con su ubicación, tamaño y movilidad, y los hallazgos a nivel de las pruebas complementarias son inespecíficos. Dado que su historia natural muchas veces pasa inadvertida, el mixoma puede culminar en la obstrucción auriculoventricular, obstrucción del tracto de salida ventricular o incluso embolias sistémicas; de esta forma, es el causante de sintomatología severa como síncope, insuficiencia cardíaca y muerte súbita cardíaca. Su manifestación clínica como un síncope es bastante frecuente. Se expone el caso de un paciente de sexo masculino de 81 años que consultó en el servicio de urgencias por un síncope. Durante su valoración, se identificó el mixoma auricular como etiología de este.

Cardiac neoplasms are divided into primary and secondary tumors, the latter are 30 times more frequent. Most are benign and within this group the atrial myxoma stands out. The clinical presentation is very variable in relation to its location, size and mobility, and the findings in the complementary tests are nonspecific. Since its natural history often goes unnoticed, the myxoma can culminate in atrial ventricular obstruction, ventricular outflow tract obstruction or even systemic embolism, causing severe symptoms such as syncope, heart failure and sudden cardiac death. Its clinical manifestation as syncope is quite common. The case of an 81-year-old male patient who consulted at the emergency department for syncope is presented, in which the atrial myxoma was subsequently identified as the etiology of the event during the further evaluation.

As neoplasias cardíacas dividem-se em tumores primários e secundários, estes últimos são 30 vezes mais frequentes. A maioria são benignas e dentro deste grupo destaca-se o mixoma auricular. A apresentação clínica é muito variável em relação com sua localização, tamanho e mobilidade, e os achados a nível dos testes complementares são inespecíficos. Dado que a sua história natural muitas vezes passa despercebida, o mixoma pode culminar na obstrução aurículo-ventricular, obstrução do tracto de saída ventricular ou mesmo embolias sistémicas, sendo assim o causador de sintomatologia severa como síncope, insuficiência cardíaca e morte súbita cardíaca. Sua manifestação clínica como síncope é bastante comum. Apresenta-se o caso de um paciente de sexo masculino de 81 anos que consultou no serviço de urgências para um síncope. Durante a sua avaliação, se identificou mixoma auricular como etiologia deste

Risk factors for syncope in children with severe idiopathic pulmonary arterial hypertension / 中华儿科杂志

Objective: To explore the risk factors for syncope in children with severe idiopathic pulmonary arterial hypertension (IPAH). Methods: Forty-four patients (age<18 years) with IPAH admitted to the Department of Pediatric Cardiology, Beijing Anzhen Hospital between May 2011 and October 2021 were retrospectively included. Patients were devided into syncope group and non-syncope group. Clinical manifestation and hemodynamic parameters including echocardiography, blood tests, right heart catheterization and acute pulmonary vascular expansion test were compared between two groups. Comparisons between groups were performed with unpaired Student t test, or Mann-Whitney U test or chi-square test. Logistic regression was used to calculate the odds ratio (OR) for parameters with statistically significant differences between groups and analyze the statistical correlation. Results: Among the 44 patients, 16 were males, the onset age was (7.2±3.9) years. Twenty-four (55%) children presented with 1 to 11 times of episodes of syncope, and 18 cases of whom induced by physical activity. Syncope group had a larger proportion of New York Heart Association (NYHA) heart function class Ⅲ-Ⅳ (67% (16/24) vs. 25% (5/20), χ2=7.59, P=0.006), higher brain natriuretic peptide (BNP) value ((251±39) vs. (61±40) pg/L, t=-2.18, P=0.035), higher pulmonary-to-aorta diameter ratio (1.6±0.4 vs. 1.4±0.2, t=-2.25, P=0.030) and larger pulmonary vascular resistance index ((22±11) vs. (16±7) WU/m2, t=-2.13, P=0.039) compared with non-syncope group. The proportion of patent foramen ovale (4% (1/24) vs. 45% (9/20), χ2=10.36, P=0.001), left ventricular ejection fraction (LVEF) ((68±5)% vs. (72±8)%, t=2.23, P=0.031) and the positive rate of acute pulmonary vascular expansion test (8% (2/24) vs. 35% (7/20), χ2=4.77, P=0.029) of syncope group were significantly lower than those of non-syncope group. Multiple Logistic regression analysis showed that NYHA heart function Ⅲ-Ⅳ (OR=6.787, 95%CI 1.445-31.880), pulmonary vascular resistance index (OR=1.247, 95%CI 1.020-1.525) and BNP (OR=1.036, 95%CI 1.007-1.066) were independent risk factors for syncope. The patent foramen ovale (OR=0.010, 95%CI 0.000-0.586) was a protective factor for syncope. Conclusions: NYHA cardiac function grade, pulmonary vascular resistance index and BNP are independent risk factors for syncope. Patent foramen ovale is a protective factor for syncope. Exercise is the main inducement of syncope in children with IPAH.

Initial Experience with Fractionation Mapping in the Identification of Vagal Ganglionated Plexus During Cardioneuroablation

This is a series case report of five symptomatic patients presented with diagnosis of sinus bradycardia, first and second degrees atrioventricular (AV) blocks, that were referred to pacemaker implantation. During the screening, a functional cause for the bradycardia and AV blocks were documented by treadmill stress test, 24-hour Holter monitoring and atropine test. After the confirmation of the diagnosis, patients were submitted to cardioneuroablation on an anatomical basis supported by a tridimensional electroanatomical fractionation mapping software. The technique and the acute and short-term results of the cardioneuroablation are described.

Síncope y paro cardiorrespiratorio, formas graves de presentación de la embolia pulmonar central: reporte de tres casos clínicos / Syncope and cardiorespiratory arrest, severe forms of presentation of central pulmonary embolism: report of three clinical cases / Síncope e parada cardiorrespiratória, formas graves de apresentação de embolia pulmonar central: relato de três casos clínicos

El tromboembolismo pulmonar tiene una presentación clínica variada. Es fundamental tener un alto índice de sospecha para arribar al diagnóstico oportuno. El síncope se asocia a casos graves y tiene importancia pronóstica. El tratamiento trombolítico es la piedra angular en el subgrupo de pacientes de alto riesgo. Se presentan tres casos clínicos de tromboembolia pulmonar de alto riesgo con el fin de discutir el escenario clínico de presentación y el tratamiento instaurado.

Pulmonary thromboembolism has a varied clinical presentation. It is essential to have a high index of suspicion to arrive at a timely diagnosis. Syncope is associated with severe cases and is of prognostic significance. Thrombolytic treatment is the cornerstone in the subgroup of high-risk patients. Three clinical cases of high-risk pulmonary thromboembolism are presented in order to discuss the clinical presentation scenario and the established treatment.

O tromboembolismo pulmonar tem a presentação clínica variada. É essencial ter um alto índice de suspeita para chegar a um diagnóstico oportuno. A síncope está associada a casos graves e tem significado prognóstico. O tratamento trombolítico é a pedra angular no subgrupo de pacientes de alto risco. São apresentados três casos clínicos de tromboembolismo pulmonar de alto risco para discutir o quadro clínico e o tratamento instituído.

Detección de síncope vasovagal con adaptación de la Escala de Calgary en estudiantes universitarios colombianos / Using a Calgary Scale adaptation to detect vasovagal syncope in Colombian university students

Resumen Introducción: El síncope vasovagal tiene una incidencia entre 10% y 19% en la población general, el primer episodio suele aparecer en la juventud. Objetivo: Estimar la prevalencia de síncope vasovagal en una población universitaria del sur de Colombia, utilizando una adaptación de la Escala de Calgary como cuestionario diagnóstico. Materiales y métodos: Después de la firma voluntaria del consentimiento informado, los estudiantes fueron entrevistados y se les aplicó la Escala de Calgary. Posteriormente, se realizó un cuestionario de seguimiento a los participantes positivos. Resultados: Participaron 280 estudiantes, el 17% obtuvo un puntaje positivo para síncope vasovagal. La edad media fue 21,45±5,18; con mayor positividad para el género femenino. En el seguimiento, el 10% de los positivos, consultó a urgencias por pérdida de conciencia, 4% fue ingresado a hospitalización y 10% presentó lesiones secundarias. Conclusiones: Un número representativo de participantes fue positivo en la Escala de Calgary, ninguno de ellos había recibido diagnóstico de síncope neuralmente mediado, pese a haber consultado a urgencias y/o presentar lesiones secundarias por pérdida de conciencia. Incluir la Escala de Calgary en la evaluación inicial del paciente consultante por pérdida de conciencia, podría ayudar al diagnóstico temprano de síncope vasovagal.

Abstract Introduction: Vasovagal syncope has an incidence between 10% and 19% in the general population, whose first episodes appear in youth. Objective: To estimate the prevalence of vasovagal syncope in a university student population from the southern Colombia, using a diagnostic questionnaire adapted from the Calgary Scale. Materials and methods: After voluntary signing of the informed consent form, students were surveyed through the Calgary Scale, and a subsequent follow-up questionnaire was conducted on positive participants. Results: 280 students participated in the study and 17% of them obtained a positive score for vasovagal syncope, being positive female participants the largest group. The mean age of participants was 21.45±5.18 years. At the follow-up, 10% of the positive cases had sought out treatment in emergency rooms because of consciousness loss, 4% were hospitalized, and 10% had secondary injuries. Conclusions: Even though an important number of patients was positive on the Calgary Scale, none of them had been diagnosed with neutrally mediated syncope, despite being treated in emergency rooms and/or presenting secondary lesions due to consciousness loss. The use of the Calgary Scale during the assessment of patients being treated for consciousness loss could help to early diagnose vasovagal syncope.

Compromiso cardíaco por amiloidosis AL: la desemejanza como atributo. Reporte de 3 casos / Cardiac compromise due to AL amyloidosis: dissimilarity as an attribute. Report of 3 cases

RESUMEN La amiloidosis AL (antes denominada amiloidosis primaria) es una entidad sistémica poco frecuente, con incidencia desconocida en el mundo, que puede llegar a presentar compromiso cardíaco en casi la mitad de los pacientes, llevando a una cardiomiopatía restrictiva por depósito de tejido amiloide. A continuación, presentamos 3 casos de pacientes que consultaron por falla cardíaca aguda y síncope, en quienes finalmente se confirmó el diagnóstico de amiloidosis AL. Al final, se realiza una breve revisión de la literatura, enfatizando en los elementos clínicos para un diagnóstico temprano.

SUMMARY AL amyloidosis (formerly called primary amyloidosis) is a rare systemic entity, with an unknown incidence in the world, which can develop heart involvement in almost half of patients, leading to restrictive cardiomyopathy by amyloid tissue deposit. We present 3 cases of patients who consulted for acute heart failure and syncope, in which the diagnosis of AL amyloidosis was finally confirmed. We conclude with a brief review of the literature, emphasizing clinical elements for an early diagnosis.

Características operativas de la regla de predicción STePS, para desenlaces graves a corto plazo en pacientes con síncope / Operative characteristics of the STePS prediction rule for short-term serious outcomes in patients with syncope

Resumen Introducción: el síncope es una entidad clínica frecuente en urgencias con un pronóstico variable que depende de su etiología. El estudio STePS identificó cuatro variables que se asociaron independientemente con desenlaces graves a 10 días. El objetivo del estudio fue evaluar su rendimiento en una población de pacientes que acuden al servicio de urgencias por síncope. Metodología: estudio prospectivo observacional de pacientes que consultaron a urgencias del Hospital Universitario Clínica San Rafael con diagnóstico de síncope. Se realizó un análisis descriptivo de las variables demográficas y clínicas de la población, y un análisis comparativo según el ingreso o no a UCI. Se analizaron las variables cualitativas y cuantitativas por medio de prueba de chi cuadrado y t student respectivamente. Las características operativas de la regla STePS fueron evaluadas en una gráfica considerándose como aceptable un área bajo la curva (AUC) mayor a 0.8 y como óptima un AUC mayor a 0.9. Resultados: se incluyeron 98 pacientes. El rendimiento de la regla de predicción STePS fue AUC-ROC 0.64 (IC95%; 0.53-0.75). Sólo el electrocardiograma anormal (OR 13.98, IC 95% 1.29151.9) y trauma concomitante (OR 5.22, IC 95% 1.20-22.67) demostraron ser factores de riesgo para desenlaces graves a 10 días. Se identificaron factores prevalentes en la población con ingreso a UCI: edad >65 años (p=0.02), antecedente de falla cardiaca (p=0.047), enfermedad renal crónica (p=0.002) y cardiopatía (p=0.01). Conclusión: la regla de predicción STePS no tuvo un rendimiento favorable para predicción de desenlaces graves a 10 días del evento sincopal en esta población estudiada.

Abstract Introduction: syncope is a common clinical condition in the emergency room with a variable prognosis depending on its etiology. The STePS study identified four variables which were independently related to serious outcomes within 10 days. The objective of the study was to evaluate its performance in a population of patients seen in the emergency room for syncope. Methods: a prospective observational study of patients seen in the emergency room of the Hospital Universitario Clínica San Rafael with a diagnosis of syncope. A descriptive analysis of the population's demographic and clinical variables was conducted, along with a comparative analysis according to admission or non-admission to the ICU. Qualitative and quantitative variables were analyzed using Chi-square or Student's t test, respectively. The operative characteristics of the STePS rule were evaluated in a graph, with an area under the curve (AUC) greater than 0.8 considered to be acceptable, and greater than 0.9 considered to be optimal. Results: Ninety-eight patients were included. The performance of the STePS prediction rule was AUC-ROC 0.64 (95% CI; 0.53-0.75). Only an abnormal electrocardiogram (OR 13.98, 95% CI 1.29-151.9) and concomitant trauma (OR 5.22, 95% CI 1.20-22.67) proved to be risk factors for serious outcomes within 10 days. Prevalent factors in the population admitted to the ICU were: age >65 years (p=0.02), a history of heart failure (p=0.047), chronic kidney disease (p=0.002) and heart disease (p=0.01). Conclusion: the STePS prediction rule did not perform favorably for predicting serious outcomes within 10 days of the syncopal event in this study population.

Descripción de dos familias con síndrome de QT largo / Description of 2 families with long QT syndrome

Resumen El síndrome de QT largo representa un grupo de desórdenes electrofisiológicos cardiacos, caracterizados por la prolongación del intervalo QT, que se asocian a muerte súbita, taquicardias ventriculares y síncope. Se presenta el caso de dos familias con la descripción clínica de los afectados, el estudio genético y el respectivo manejo, y se hace una breve actualización de la literatura sobre el síndrome de QT largo.

Abstract Long QT syndrome represents a group of electrophysiologic disorders characterized by a prolongation in the QT interval that are associated with sudden death, ventricular tachycardia and syncope. We present 2 families describing the clinical presentation, the genetic study and their respective treatment also there is a brief review about long QT syndrome.

Syncope in patients with cardiac pacemakers

Abstract Introduction: It is challenging to diagnose syncope in patients with pacemakers. Because these patients have increased morbidity and mortality risks, they require immediate attention to determine the causes in order to provide appropriate treatment. This study aimed to investigate the causes and predictive factors of syncope as well as the methods used to diagnose syncope in cardiac pacemaker patients. Methods: Patients with pacemakers implanted owing to sinus node disease or atrioventricular block were evaluated with standardized questionnaires, endocavitary electrograms, and other tests based on the suspected causes of syncope. Mann-Whitney U tests were used to analyze continuous variables and Chi-squared or Fisher's exact tests were used for categorical variables. Logistic regression was used for multivariate analyses. Statistical significance was P<0.05. Results: The study included 95 patients with pacemakers: 47 experienced syncope in the last 12 months and 48 did not. Of the 100 documented episodes of syncope, 48.9% were vasovagal syncopes, 17% had cardiac-related causes, 10.6% had unknown causes, and 8.5% had pacemaker failure. The multivariate analysis showed that a New York Heart Association (NYHA) Functional Class II was a significant factor for developing syncope (P<0.01). Conclusion: While the most common type of syncope in pacemaker patients was neurally mediated, it is important to perform detailed evaluations in this population as the causes of syncope can be life-threatening. The best diagnostic methods were stored electrogram analysis and the tilt table test. NYHA Functional Class II patients were found to have a higher risk for syncope.

Clinical features of vestibular syncope associated with tumarkin attacks in delayed endolymphatic hydrops / 中华耳鼻咽喉头颈外科杂志

Objective: To analyze the clinical characteristics of vestibular syncope (VS) associated with drop attacks (DA) in delayed endolymphatic hydrops (DEH). Methods: DEH cases with complete data were retrospectively analyzed, including three DEH cases with DA and VS (VS group), and six DEH cases without DA or VS (control group). The clinical profile, the results of neurotological examinations [such as pure tone audiometry, electrocochleography (EcochG), caloric test, vestibular evoked myogenic potentials (VEMP), and video head impulse test (vHIT)] and treatment outcomes were analyzed. Results: (1) In the VS group, there were three cases of ipsilateral DEH; in the control group, there were six cases of ipsilateral type. One case in each group had a history of migraine. (2) The prevalence of abnormal results in caloric test, vHIT, cervical VEMP, and ocular VEMP in the VS group was 3/3, 1/3, 2/2, and 2/2, respectively, and in the control group was 3/6, 0/3, 1/6, and 4/6, respectively. Two cases in each group underwent EcochG, and no identifiable waveform was elicited on the affected side, and-SP/AP ratio of unaffected side was less than 0.4. (3) Patients in both groups were initially treated with conservative medication. Two cases in the VS group subsequently received intratympanic injections of dexamethasone. No DA or VS occurred during a follow-up period lasting over one year. All patients achieved good control of vertigo during the follow-up period. Conclusions: VS may occur in the patients with DEH. The differential diagnosis of syncope in patients with otogenic vertiginous disease can help improve clinical diagnosis and treatment.

Desenlaces a un año en pacientes con síncope de alto y bajo riesgo / One-year outcomes in patients with high-risk and low-risk syncope

Resumen Introducción: El síncope es un síntoma complejo de evaluar, que además representa un reto diagnóstico; la estratificación inadecuada del riesgo de los pacientes puede conllevar mal uso de los recursos en salud y aumentar así el costo derivado de la atención. Objetivo: Comparar la incidencia de desenlaces a un año en pacientes con síncope de alto y bajo riesgo en cuatro hospitales de Bogotá. Metodología: Estudio de cohorte retrospectiva, en el que se incluyeron pacientes mayores de 18 años que consultaron a urgencias por síncope, y que fueron seguidos desde febrero de 2013 hasta julio de 2015. Fueron clasificados en bajo y alto riesgo según la escala de Martin et al. Se hizo seguimiento telefónico para evaluar los desenlaces a un año. Resultados: Los pacientes de alto riesgo tienen mayor frecuencia de mortalidad, recurrencia del síncope, requerimiento de hospitalización, eventos neurológicos y necesidad de intervención cardiovascular. La mortalidad global está alrededor del 6% y en el subgrupo de pacientes de mayor puntaje (4 puntos) asciende al 16,6%. La mayoría de eventos se presentaron en los primeros 6 meses de seguimiento. La mortalidad y la necesidad de intervención cardiovascular tuvieron un incremento de su frecuencia a medida que aumentó el puntaje de la escala. Conclusión: Los pacientes clasificados en alto riesgo según la escala de Martin et al. tienen mayor frecuencia de desenlaces adversos a un año de seguimiento, y por tanto podrían beneficiarse de un estudio más amplio y expedito de la causa del síncope.

Abstract Introduction: Syncope is a complex symptom to assess, and is a diagnostic challenge. The inadequate risk stratification can lead to the inappropriate use of health resources and to an increase in the costs arising from the care. Objective: To compare the outcomes at one year in patients with high and low risk syncope in four Bogota hospitals. Material and method: A retrospective cohort study was conducted that included patients over 18 years-old that were seen in the Emergency Department due to a syncope. They were followed-up from February 2013 until July 2015. They were classified into low and high risk according to the score on the scale of Martin et al. At one year, a telephone call follow-up was made to assess the outcomes. Results: The high risk patients had higher rates of mortality and recurrence of syncope, required more hospital admissions, had more neurological events, and a greater need for cardiac intervention. The overall mortality was 6%, and in sub-group of patients with a higher score (4 points) it increased to 16.6%. The majority of events occurred in the first 6 months of follow-up. The mortality and the need for cardiovascular intervention were associated with the increase in the score on the scale. Conclusion: Patients classified as high risk according to the scale of Martin et al. are more likely to have adverse outcomes at one year of follow-up, and thus could benefit from a larger study and directed at the cause of the syncope.

Taquicardia ventricular polimórfica y miocardio no compactado, ¿una nueva variante genética? / Polymorphic ventricular tachycardia and non-compacted myocardium: A new genetic variant?

Resumen La taquicardia ventricular polimórfica catecolaminérgica es una enfermedad caracterizada por arritmias ventriculares desencadenadas por estrés o actividad física. Existen casos descritos de taquicardia ventricular polimórfica catecolaminérgica asociada a ventrículo izquierdo no compactado, en relación con mutaciones del gen RYR2 localizadas en el exón 3. Se expone el caso clínico de una paciente joven que debutó con clínica de síncopes recurrentes asociados a estrés físico o emocional. En el estudio posterior se descubrió taquicardia ventricular polimórfica catecolaminérgica, con áreas de miocardio no compactado y una nueva variante genética posiblemente asociada a la enfermedad.

Abstract Catecholaminergic polymorphic ventricular tachycardia is disease characterised by ventricular arrhythmias triggered by stress or physical activity. There are some cases of catecholaminergic polymorphic ventricular tachycardia described that are associated with non-compacted left ventricle in relation to mutations of the RYR2 gene located in exon 3. A case is presented of a young patient in whom the clinical signs started with recurrent syncope associated with physical or emotional stress. In the subsequent study, catecholaminergic polymorphic ventricular tachycardia was discovered, with areas of non-compacted myocardium and new genetic variant possibly associated with the disease.

Manuseio do bigeminismo atrial / Handling of atrial bigeminism

INTRODUÇÃO: As ectopias atriais podem manifestar-se de diversas formas desde a ausência de sintomas até síncope. Descrevemos um caso de bigeminismo atrial bloqueado com prolongamento do intervalo QT e consequente Torsades de Pointes. Relato de caso: Paciente do sexo feminino, 75 anos, foi admitida no serviço de pronto atendimento devido a queixa de episódios recorrentes de síncope em posição sentada após sensação inespecífica de mal-estar, dispneia e turvação visual associado a liberação esfincteriana. ECG na admissão revelou ritmo sinusal e ectopias supraventriculares frequentes, motivo pelo qual iniciou-se terapia com amiodarona. Holter de 24h demonstrou bigeminismo atrial bloqueado e prolongamento do intervalo QT seguido por episódios de Torsade de Pointes e fibrilação ventricular. Realizado implante de marcapasso provisório seguido por ablação do foco arritmogênico em região de anel triscuspídeo. A paciente evoluiu com melhora da sintomatologia e Holter de controle demonstrou ausência de arritmias.

Analysis of Conduction Intervals in Normal Electrophysiological Studies: Establishment of Reference Values the Brazilian Population

Abstract Background In the investigation of cardiac rhythm disorders, a normal electrophysiological (EPS) study is associated with a favorable prognosis. One of the normality criteria is established by conduction intervals within expected range. Objective To establish reference values in EPS for the intracavitary conduction intervals (PA, AH and HV) in a Brazilian population. Methods A retrospective cohort study of the first 1,500 patients submitted to EPS ablation was performed at Instituto de Cardiologia do Rio Grande do Sul, Brazil. The EPS was considered normal if the test was performed for diagnostic purpose; absence of induced arrhythmias; and conduction intervals within the expected range. The REDCap software was used for data collection and management, and the SPSS Statistics 22.0 used for data analysis. Continuous variables were compared with Student's t-test for independent samples and categorical variables with the chi-square test (X 2 ). Values of p ≤ 0.05 were considered significant. Results A total of 124 (8.3%) with EPS considered normal were included; mean age was 52 ± 21 years, and 63 were male. The mean values in milliseconds of PA, AH and HV were 23 ± 9, 88 ± 25 and 44 ± 7, respectively. The PA, AH, and HV percentile ranges were 13 - 25, 81-107 and 40 - 52, respectively. When the patients were divided into three age groups (1 to 18 years, 19 to 64 years and 65 or more), we observed that the group of older patients had significantly higher values of PA, AH and HV compared with younger patients. Conclusion This study showed that intracavitary conduction intervals in a sample of the Brazilian population were similar to previously published studies. Elderly patients tend to have higher values of intracavitary conduction intervals in EPS. Future studies including broader age ranges could enable the acquisition of more reliable and reproducible reference values. (Int J Cardiovasc Sci. 2020; [online].ahead print, PP.0-0)

Fibrilación auricular por vía accesoria con patrón de preexcitación ventricular: Informe de un caso / Atrial fibrillation by accessory pathway with pattern of ventricular pre-excitation: A case report

RESUMEN La fibrilación auricular por vía accesoria es una peligrosa enfermedad que puede provocar muerte súbita; es poco frecuente y necesita una conducta exacta, mucho más si aparece un patrón de preexcitación ventricular. Se presenta el caso de un adolescente masculino de 17 años de edad y piel blanca, con antecedentes de palpitaciones cortas a esfuerzos moderados y pérdida ocasional del conocimiento de breve duración, sin otra sintomatología asociada. Al momento del ingreso presenta un cuadro de palpitaciones, fatiga, palidez e hipotensión arterial, y en el electrocardiograma se observa una taquicardia con QRS ancho, irregular, con onda delta y frecuencia ventricular rápida, que fue interpretada como fibrilación auricular preexcitada. Se realizó cardioversión eléctrica sincronizada al QRS con 100 julios y se logró restaurar el ritmo sinusal con patrón electrocardiográfico de preexitación ventricular (síndrome de Wolff-Parkinson-White).

ABSTRACT Atrial fibrillation by accessory pathway is a dangerous disease that can cause sudden death, it is rare and it needs a careful management, even more, if a pattern of ventricular pre-excitation appears. The case of a white skin 17-year-old teenager with a history of short palpitations at moderate effort, and occasional loss of consciousness of short duration, without other associated symptoms, is presented. At the moment of admission, he presents palpitations, fatigue, pallor and low blood pressure; in the electrocardiogram a tachycardia with wide, irregular QRS, with delta wave and rapid ventricular rate is observed, which was interpreted as pre-excited atrial fibrillation. Synchronized electrical cardioversion to QRS with 100 joules was performed, and sinus rhythm was restored with electrocardiographic pattern of ventricular pre-excitation (Wolff-Parkinson-White syndrome).

Consideraciones sobre las condiciones neuropsiquiátricas del Quijote de la Mancha / Considerations about the Neuropsychiatry Conditions of Quixote of La Mancha

RESUMEN Don Quijote de la Mancha, el pintoresco personaje creado por Miguel de Cervantes Saavedra en su obra inmortal El Ingenioso Hidalgo Don Quijote de la Mancha, presenta varias condiciones neuropsiquiátricas que incluyen tremor, trastornos del sueño, síntomas neuropsiquiátricos, síncope, trastornos de la percepción y traumatismo craneoencefálico. A lo largo de la obra, se presentan episodios en los que se hacen evidentes los diferentes trastornos mencionados. El artículo hace un recuento de ellos a través de la novela y un análisis a la luz de los conocimientos actuales sobre dichas condiciones.

ABSTRACT Don Quixote of La Mancha, the picturesque character created by Miguel de Cervantes Saavedra in his immortal book The Ingenious Nobleman Sir Quixote of La Mancha, presents several neuropsychiatric conditions, including tremor, sleep disturbances, neuropsychiatric symptoms, syncope, perception disorders and traumatic brain injury. Throughout the masterpiece, there are episodes where the aforementioned disorders are evident. This paper makes a list of them and analyses them in the light of the current knowledge of those conditions.

Pediatric patients head-up-tilt-test with pharmacological challenge, it is safe? / Mesa inclinada en pacientes pediátricos con reto farmacológico, ¿es seguro?

Abstract Syncope in pediatrics represents an important cause of visits to the emergency units. For this reason, excluding a cardiac or malignant origin is essential at the time of the initial approach to determine what is the next step in management, or if they need to be referred to a pediatric cardiologist and/or electrophysiologist. Vasovagal syncope is the most frequent cause of syncope in pediatrics, in which a detailed clinical history is enough to make the diagnosis. If no diagnosis is concluded by the history, or if it is necessary to define the hemodynamic response of the patients, the head-up-tilt-test is indicated; this will trigger syncope due to an orthostatic stress caused by the angulated table (passive phase). If a negative response remains, it can be followed by a pharmacologic challenge to trigger the hemodynamic response, which is still controversial in pediatrics. The pharmacologic challenge increases the sensitivity with a slight reduction in test specificity. Although there is not a specific drug for the challenge in pediatric patients yet, the most commonly drugs used are nitrates and isoproterenol, the latter related to a great number of adverse effects. Sublingual administration of nitrates in the challenge has been proven to be ideal, effective, and safe in this specific age group. The aim of this article is to make a literature search to demonstrate the effectiveness and safety of the pharmacologic challenge during the head-up-tilt-test in pediatrics, emphasizing a study conducted at the National Institute of Cardiology with isosorbide dinitrate.

Resumen El síncope en edades pediátricas representa una causa importante en las visitas a unidades de urgencias, por lo que excluir un origen cardíaco o maligno es fundamental al momento del abordaje inicial para determinar la conducta a seguir o la necesidad de derivar al cardiólogo pediatra o electrofisiólogo. El síncope vasovagal (SVV) es la causa más frecuente de síncope en pediatría, para cuyo diagnóstico basta una historia clínica detallada. Cuando ésta no es suficiente para determinar el diagnóstico de síncope reflejo o es necesario definir el tipo de respuesta que lo origina, está indicada una prueba de mesa inclinada que produce un estrés ortostático por la angulación y ello desencadena un síncope (fase pasiva). En pruebas no concluyentes está indicado un reto farmacológico para precipitar la respuesta hemodinámica, pero aún es un tema de controversia en edades pediátricas. El reto farmacológico incrementa la sensibilidad de la prueba, con una ligera reducción de la especificidad. Si bien no existe todavía un medicamento específico para la población pediátrica, los más empleados son los nitratos y el isoproterenol, este último relacionado con un mayor número de efectos adversos. La administración sublingual de los nitratos utilizados ha demostrado ser ideal, efectiva y segura en los pacientes pediátricos. El objetivo del artículo es realizar una revisión de las publicaciones médicas que demuestran la efectividad y seguridad del reto farmacológico durante la prueba de mesa inclinada en pacientes pediátricos, con énfasis en un estudio conducido en el Instituto Nacional de Cardiología con dinitrato de isosorbida (DNIS).