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.

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.

La autopsia molecular en la muerte súbita cardiaca / Molecular autopsy in sudden cardiac death

Resumen Actualmente hay un porcentaje importante de autopsias que quedan sin un diag nóstico concluyente del fallecimiento, especialmente cuando este evento letal se produce súbitamente. El análisis genético se ha ido incorporando recientemente al campo de la medicina forense, sobre todo en aquellos pacientes que han fallecido de forma repentina, y donde no se identifica causa concluyente del fallecimiento tras una autopsia médico-legal completa. En estos casos las enfermedades eléctricas primarias son las principales responsables del fallecimiento. Hasta la fecha se han descrito más de 40 genes asociados a afecciones arritmogénicas causantes de muerte súbita cardiaca. Las principales enfermedades arritmogénicas son el síndrome de QT largo y la taquicardia ventricular; estudios genéticos post-mortem no solo permiten llevar a cabo un diagnóstico de la causa del fallecimiento, sino que también permiten una traslación clínica hacia los familiares, focalizado en la identificación precoz de individuos en riesgo de síncope, así como adopción de medidas terapéuticas personalizadas para la prevención de un episodio arrítmico letal.

Abstract Currently, there are a significant percentage of autopsies left without a conclusive diagnosis of death, especially when this lethal event occurs suddenly. Genetic analysis has been recently incorporated into the field of forensic medicine, especially in patients with sudden death and where no conclusive cause of death is identified after a complete medical- legal autopsy. Inherited arrhythmogenic diseases are the main cause of death in these cases. To date, more than 40 genes have been associated with arrhythmogenic disease, and causing sudden cardiac death has been described. The main arrhythmogenic diseases are Long QT Syndrome, Catecholaminergic Polymorphic Ventricular Tachycardia, Brugada Syndrome, and Short QT Syndrome. These post-mortem genetic studies, not only allow a diagnosis of the cause of death, but also allow a clinical translation in relatives, focusing on the early identification of individuals at risk of syncope, as well as adopting personalised therapeutic measures for the prevention of a lethal arrhythmic episode.

Syncope: Clinical Presentation of Bronchial Asthma / Síncope: presentación clínica del asma bronquial

ABSTRACT Syncope, first described by Hippocrates, can be differentiated into neurological, cardiac and non-cardiac in origin, and this differentiation is of prognostic significance. The cardiac causes of syncope, which can be structural, electrophysiological or infectious, have a relatively poor prognosis and are associated with ethnicity, geographic location and sudden cardiac death. In decreasing frequency, the cardiac causes are hypertrophic cardiomyopathy, anomalous coronary arteries, Marfan Syndrome and dilated cardiomyopathy. Electrophysiological causes include supraventricular causes, Wolf-Parkinson-White syndrome, ion channelopathies, long QT syndrome and Brugada syndrome. The index case with bronchial asthma presented with syncope. There is an increased morbidity and mortality of this specific group of patients, if undiagnosed and not optimally treated; hence the need for a high index of suspicion and early diagnosis, after exclusion of cardiac and more common neurological causes. This is the first documented case of syncope secondary to bronchial asthma in an Afro-Caribbean.

RESUMEN El síncope, primeramente, descrito por Hipócrates, se puede clasificar como neurológico, cardíaco y no cardíaco atendiendo a su origen, y esta diferenciación tiene importancia pronóstica. Las causas cardíacas del síncope - que pueden ser estructurales, electrofisiológicas o infecciosas - tienen un pronóstico relativamente pobre y se asocian con la etnicidad, la localización geográfica y la muerte cardiaca repentina. En frecuencia decreciente, las causas cardíacas son la cardiomiopatía hipertrófica, las arterias coronarias anómalas, el síndrome de Marfan y la cardiomiopatía dilatada. Las causas electrofisiológicas incluyen las causas supraventriculares, el síndrome de Wolf-Parkinson-White, las canalopatías iónicas, el síndrome de QT largo, y el síndrome de Brugada. El caso índice con asma bronquial se presentó con síncope. Hay una mayor morbilidad y mortalidad de este grupo específico de pacientes, si no se diagnostica y no se trata de forma óptima. De ahí, la necesidad de un alto índice de suspicacia y diagnóstico temprano, luego de la exclusión de las causas cardíacas y las causas neurológicas más comunes. Éste es el primer caso documentado de síncope secundario al asma bronquial en un afrocaribeño.

Síncope único o muy poco frecuente en 104 casos / Single or very isolated syncope in 104 cases

Resumen Introducción: Es frecuente de encontrar personas enviadas para realizar la prueba de Tilt Test (TT), que sufrieron un solo síncope aislado o que ocurre muy ocasionalmente durante su vida. En este trabajo nos preguntamos en qué se diferencian estas personas de las que nunca tienen síncopes. Métodos: Realizamos el examen de TT en 104 pacientes que sufrieron sólo uno o como máximo 5 síncopes por cualquier causa durante su vida. Intentamos explicar cómo actúan los diferentes factores predisponentes para provocar el síncope. Resultados: Encontramos diferencias en factores predisponentes del síncope, entre nuestros pacientes y los controles en factores como: la herencia, la hipermovilidad articular, el encharcamiento venoso durante el TT, la ingesta de alimentos, el uso de fármacos, el estrés y la emoción. Conclusiones: En el síncope único o muy ocasional, deben conjugarse en la persona dos factores. Lo explicamos: Estos pacientes tienen una predisposición orgánica (herencia -hipermovilidad articular- falla en los baroreflejos, algunas enfermedades neurológicas, etc.) a presentar síncopes. Pero los síncopes no ocurrirán a menos que se agregue simultáneamente un factor ambiental, que actúa como un desencadenante (estadía larga de pie, estrés, dolor y emoción, deshidratación, drogas, comida abundante, etc.), es por eso que en estos pacientes los síncopes son tan raros.

Introduction: It is frequent meeting people sent to perform a tilt test suffering from a single or such isolated syncope that occur very occasionally during the patient's life. We ask ourselves how these people differ from those who never have syncopes. Methods: We performed tilt test in 104 patients who suffer a single or maximum 5 syncopes from any cause during their lifetimes. We try to explain how different predisposing factors act to provoke syncope. Results: We found differences between cases and controls in inheritance of syncopes, joint hypermobility, venous pooling during tilt test, food intake, use of drugs, stress and emotion as a trigger for syncope. Conclusions: Patients with single or occasional syncope have an organic predisposition (inheritance - joint hypermobility - failure in the baroreflexes, some neurological diseases, etc.) to present syncopes, but syncopes do not occur unless it is added simultaneously an environmental factor, which acts as a trigger (prolonged standing up - stress, pain and emotion - dehydration - drugs - abundant food, etc.) that is why these episodes are so rare.

Structural heart disease as the cause of syncope

We described the clinical evolution of patients with structural heart disease presenting at the emergency room with syncope. Patients were stratified according to their syncope etiology and available scores for syncope prognostication. Cox proportional hazard models were used to investigate the relationship between etiology of the syncope and event-free survival. Of the 82,678 emergency visits during the study period, 160 (0.16%) patients were there due to syncope, having a previous diagnosis of structural heart disease. During the median follow-up of 33.8±13.8 months, mean age at the qualifying syncope event was 68.3 years and 40.6% of patients were male. Syncope was vasovagal in 32%, cardiogenic in 57%, orthostatic hypotension in 6%, and of unknown causes in 5% of patients. The primary composite endpoint death, readmission, and emergency visit in 30 days was 39.4% in vasovagal syncope and 60.6% cardiogenic syncope (P<0.001). Primary endpoint-free survival was lower for patients with cardiogenic syncope (HR=2.97, 95%CI=1.94-4.55; P<0.001). The scores were analyzed for diagnostic performance with area under the curve (AUC) and did not help differentiate patients with an increased risk of adverse events. The differential diagnosis of syncope causes in patients with structural heart disease is important, because vasovagal and postural hypotension have better survival and less probability of emergency room or hospital readmission. The available scores are not reliable tools for prognosis in this specific patient population.

Crisis otolítica de Tumarkin: revisión de la literatura / Tumarkin's otolithic crisis: a review

Las crisis de Tumarkin consisten en caídas bruscas al suelo sin pródromos previos ni pérdida de conciencia y de segundos de duración que pueden ocurrir con frecuencia relativa en pacientes con enfermedad de Ménière. Si bien pueden presentarse de manera aislada durante la evolución de la enfermedad, existen casos con crisis recurrentes que comprometen significativamente la calidad de vida de los pacientes. Se postula que las crisis se producen por una alteración de la función del órgano otolítico, específicamente del sáculo. El tratamiento puede ser desde el manejo expectante hasta el uso de laberintectomía química o quirúrgica.

Tumarkin´s otolithic crisis is a sudden fall that comes with no loss of consciousness, and without warning or prodrome. It has a short duration and can occur with relative frequency in patients with Meniere disease. When it is present, it significantly compromises life quality of patients. There is no certainty about its mechanism, but it is assumed that the crises are caused by an otolithic organ disfunction, specifically a collapse of the saccule. Treatment can range from observation to chemical or surgical labyrinthectomy.

Síncope secundario a mixoma gigante del anillo mitral: reporte de un caso / Large mitral annulus myxoma presenting with syncope: report of one case

We report a 23-year-old woman, with three recent exertional syncopes. Transthoracic (TTE) and transesophageal (TEE) echocardiography found a large heterogeneous mass (38 x 35 mm) arising from the posterior mitral annulus, protruding in systole through the left ventricular outflow tract (LVOT). Heart MRI confirmed the echocardiography findings, suggesting a cardiac myxoma. Cardiac surgery accomplished the complete resection of the lesion, confirming a mass arising from the posterior mitral annulus and preserving mitral anatomy and function. Pathology was positive for a myxoma. Uneventful evolution allowed the discharge of the patient at the fifth postoperative day. Control TTE discarded any complication.

Accidente isquémico transitorio de causa hemodinámica / Transient ischemic attack of hemodynamic cause

El síncope es una perdida súbita y transitoria del estado de conciencia y el tono postural con restitución completa. Según su etiología se clasifica como reflejo (neuromediado), cardíaco, neurológico (isquemia vertebrobasilar) o indeterminado. Los síncopes neurológicos se observan en contexto de accidente cerebrovascular isquémico o accidente isquémico transitorio; frecuentemente se asocian a signos deficitarios focales. Presentamos el caso de un síncope no neurológico con signos deficitarios focales en una paciente con marcada enfermedad ateromatosa. (AU)

Syncope is the abrupt and transient loss of consciousness associated with absence of postural tone, followed by complete and usually rapid spontaneous recovery. In terms of etiology, syncope is classified as reflex (neurally mediated), cardiac, neurologic (vertebrobasilar ischemia) or indeterminate. The neurologic syncope occurs in the setting of stroke or transient ischemic attack, being most frequently associated with focal neurologic symptoms. We report a case of non-neurologic syncope followed with focal neurologic symptoms in a patient with atherosclerosis disease. (AU)

Anaphylaxis caused by stings from the Solenopsis invicta, lava-pés ant or red imported fi re ant

Ants are social insects with species of medical interest, such as the fi re ants (Solenopsis sp.). The sting causes inflammation, vesicles and sterile pustules, which may cause allergic phenomena and even anaphylactic shock. We describe a patient who suffered a large number of stings and an episode of syncope with fall in blood pressure and complete regression of symptoms after resuscitation and medication for anaphylaxis. Considering the clinical manifestations and images of wheals and blisters on the patient’s feet at the time of syncope, this report should serve as a warning for the diagnosis and treatment of this condition and even for counseling and prevention regarding patients exposed to this risk.

Stenting for Symptomatic Vertebral Artery Stenosis Associated with Bilateral Carotid Rete Mirabile: The Long-Term Clinical and Angiographic Outcome

Symptomatic vertebral artery (VA) stenosis associated with bilateral carotid rete mirabile (CRM) has not been reported. We report the long-term clinical and angiographic outcome after stenting for symptomatic VA stenosis in the patient with bilateral CRM. This report is the first case that symptomatic VA stenosis associated with bilateral CRM was treated with stenting.

Cuadro sincopal en el contexto del síndrome de Eagle / Episode of syncope in Eagles syndrome

El síndrome de Eagle es una patología infrecuente, caracterizada por la presencia de dolor cervicofacial, cefalea y calambres que se proyectan en la garganta, en el cuello y en el oído. Describimos el caso de una mujer de 36 años, con historia de tumefacción cervical, sensación de irritación faríngea y síncope, en la que en los estudios radiológicos se encontró una osificación total de ambos ligamentos estilohioideo, causante de su cuadro sincopal por compresión carotídea.

Eagle's syndrome is an infrequent pathology characterized by the presence of cervicofacial pain, headache and cramps that are projected in the throat, neck and ear. We reported a case of a 36 year-old woman with a history of cervical swelling, sore throat and syncope, which on imaging studies was found ossification both stylohyoid ligaments, causing carotid compression and syncope.

Preditores de internação hospitalar em pacientes com síncope atendidos em hospital cardiológico / Predictors of hospitalization in patients with syncope assisted in specialized cardiology hospital

FUNDAMENTO: No atendimento ao episódio sincopal é necessário estratificar o risco para melhor diferenciar pacientes que necessitam de internação hospitalar daqueles que podem ser liberados. Os critérios utilizados pelos médicos avaliadores desses pacientes em emergências cardiológicas em nosso meio são desconhecidos. OBJETIVOS: Analisar quais os critérios adotados para internação hospitalar, diferenciá-los dos utilizados nos pacientes liberados e compará-los com os preditores de alto risco definidos pelo escore de OESIL já validado para esse fim. MÉTODOS: Estudo transversal em pacientes diagnosticados com síncope na emergência em nossa instituição no ano de 2011. RESULTADOS: Dos 46.476 atendimentos realizados naquele ano, 216 foram descritos como síncope. Dos 216 pacientes analisados, 39% foram internados, sendo que as principais variáveis associadas à admissão foram síncope prévia, doença cardíaca conhecida, história negativa para acidente vascular encefálico no passado, ECG alterado e possuir plano de súde. Na comparação internação contra não internação, os escores OESIL 0-1 foram associados a maior chance de liberação hospitalar; os escores 2-3 apresentaram maior associação com internação. Um escore OESIL >2 demonstrou razão de chances 7,8 vezes maior de internação comparado com o escore 0 (p < 0,001; IC95%: 4,03-15,11). Aproximadamente 39% dos pacientes não tiveram definição etiológica e em 18% foi identificada uma causa cardiológica. CONCLUSÕES: Fatores como doença cardiovascular conhecida, história sincopal prévia, ausência de AVC prévio, possuir seguro de saúde e eletrocardiograma alterado foram os critérios utilizados pelos médicos em emergência para indicar internação hospitalar. Houve boa correlação entre os critérios clínicos e os critérios de risco do OESIL descritos na literatura.

BACKGROUND: Risk stratification of a syncopal episode is necessary to better differentiate patients needing hospitalization of those who can be safely set home from the emergency department. Currently there are no strict guidelines from our Brazilian medical societies to guide the cardiologist that evaluate patients in an emergency setting. OBJECTIVES: To analyze the criteria adopted for defining the need for hospitalization and compare them with the predictors of high risk for adverse outcome defined by the OESIL score that is already validated in the medical literature for assessing syncope. METHODS: A cross-sectional study of patients diagnosed with syncope during emergency department evaluation at our institution in the year 2011. RESULTS: Of the 46,476 emergency visits made in that year, 216 were due to syncope. Of the 216 patients analyzed, 39% were hospitalized. The variables associated with the need of hospital admission were - having health care insurance, previous known cardiovascular disease, no history of prior stroke, previous syncope and abnormal electrocardiograms during the presentation. In comparison with those not admitted OESIL scores of 0-1 were associated with a greater chance of emergency discharge; 2-3 scores showed greater association with the need of hospitalization. A score > 2 OESIL provided an odds ratio 7.8 times higher for hospitalization compared to score 0 (p <0.001, 95% CI:4,03-15,11). In approximately 39% no etiological cause for syncope was found and in 18% cardiac cause was identified. CONCLUSIONS: Factors such as cardiovascular disease, prior history of syncope, health insurance, no previous stroke and abnormal electrocardiograms, were the criteria used by doctors to indicate hospital admission. There was a good correlation between the clinical judgment and the OESIL criteria for high risk described in literature.