Dysautonomia Evaluation by Holter in Chagas Heart Disease

Abstract Background Sudden cardiac death is the main lethal mechanism associated with Chagas cardiomyopathy. Studies suggest that dysautonomia may represent a relevant, intense, independent, and early phenomenon in the natural history of the disease, even when ventricular systolic function is preserved, and may also be the mechanism that triggers malignant ventricular arrhythmias. Objective To evaluate the degree of dysautonomia and its possible association with ventricular arrhythmias in patients with Chagas cardiomyopathy, according to different categories of mortality risk, as defined by the score proposed by Rassi, used as a surrogate outcome for death. Methods A cross-sectional study involving 43 patients with Chagas cardiomyopathy stratified into risk categories based on the Rassi score, with 23 being classified as low risk and 20 as intermediate-to-high risk. Heart rate variability (HRV) was assessed using Holter monitoring for long-term recordings of 24 hours (time domain) and for short-term recordings of 5 minutes (frequency domain) at rest and after autonomic tests: deep breathing and Valsalva maneuver. The HRV variables were compared between the groups using the Student's t-test and α=0.05. Results Comparison of the results between the risk stratification groups showed no differences in HRV indexes, either in the time or frequency domain. However, results showed a significant increase in the number of arrhythmias as a function of increased risk (p=0.02). Conclusion There was no association between the degree of dysautonomia, evaluated by Holter monitoring, and the categories of mortality risk, despite a direct association between the rate of arrhythmias and the higher risk group.

Acrocianosis como primera manifestación de síndrome de taquicardia postural ortostática en dos adolescentes / Acrocyanosis as the first manifestation of orthostatic postural tachycardia syndrome in two adolescents

La acrocianosis desencadenada por la bipedestación como signo principal de la taquicardia postural ortostática (POTS, por su sigla en inglés) es poco conocida, aunque bien descrita en la bibliografía especializada. Se describen dos casos clínicos de adolescentes que consultaron en el Servicio de Urgencias por acrocianosis e intolerancia al ortostatismo. El primer paciente, de 13 años, presentó un aumento de la frecuencia cardíaca de 40 latidos por minuto (lpm) al pasar del decúbito a la bipedestación. El segundo, de 14 años, presentó un aumento de la frecuencia cardíaca hasta 125 lpm al incorporarse. En ambos, la tensión arterial y los estudios complementarios fueron normales. El POTS se define en la edad pediátrica como una intolerancia al ortostatismo que se acompaña de aumento de la frecuencia cardíaca excesiva sin hipotensión arterial. Conocer la acrocianosis como primer signo de presentación es de utilidad para el diagnóstico de esta enfermedad y para evitar estudios complementarios innecesarios.

Acrocyanosis triggered by standing position as the main sign of postural orthostatic tachycardia (POTS) is little known but well described in scientific literature. In pediatric age, POTS is defined as orthostatic intolerance that is accompanied by an excessive increase in heart rate without arterial hypotension. We present two clinical reports of teenagers who were admitted in the Emergency Department with acrocyanosis and orthostatic intolerance. The first patient was 13-year-old and had an increase in heart rate of 40 bpm when moving from a reclining to a standing position. The second patient was 14-year-old and showed an increasing in heart rate up to 125 bpm after upright position. In both patients' blood pressure was normal and all investigations were negative. They were finally diagnosed with postural orthostatic tachycardia. Recognizing acrocyanosis as first sign of this disease is useful for diagnosis and can help to avoid unnecessary testing.

Parkinson's disease effect on autonomic modulation: an analysis using geometric indices / Efeito da doença de Parkinson na modulação autonômica: análise por meio de índices geométricos

ABSTRACT Background: Parkinson's disease (PD) produces autonomic changes, indicating lower parasympathetic modulation and global variability, but these changes need further studying regarding geometric methods. Objective: To investigate the autonomic modulation in individuals with PD using heart rate variability (HRV) indices obtained through geometric methods. Methods: This is a cross-sectional study that assessed 50 individuals, split into two groups: PD group (PDG; n=26; 75.36±5.21 years) and control group (CG; n=24; 75.36±5.21 years). We evaluated the autonomic modulation by measuring the heart rate beat-to-beat for 30 min with the individual in supine rest using a heart rate monitor and assessed geometric indices (RRtri, TINN, SD1, SD2, SD1/SD2 ratio, and qualitative analysis of the Poincaré plot). Results: Significant reductions were found in RRtri, TINN, SD1, and SD2 indices among PDG compared to CG. Regarding the SD1/SD2 ratio, no significant changes were detected between the groups. The Poincaré plot demonstrated that individuals with PD had lower beat-to-beat dispersion in RR intervals, in addition to greater long-term dispersion of RR intervals compared to CG. Conclusions: The results suggest a reduction in the parasympathetic autonomic modulation and global variability in individuals with PD compared to controls, regardless of sex, age, and body mass index.

RESUMO Introdução: A doença de Parkinson (DP) produz alterações autonômicas, que indicam menor modulação parassimpática e variabilidade global, mas que devem ser investigadas quanto aos métodos geométricos. Objetivo: Investigar a modulação autonômica em indivíduos com DP, por meio de índices de variabilidade da frequência cardíaca (VFC) obtidos pelos métodos geométricos. Métodos: Estudo transversal, no qualforam avaliados 50 voluntários, divididos em dois grupos: o grupo doença de Parkinson (GDP; n=26; 75,36±5,21 anos) e o grupo controle (GC; n=24; 75,36±5,21 anos). Para a avaliação da modulação autonômica a frequência cardíaca foi captada batimento a batimento por meio de um cardiofrequencímetro com os indivíduos em decúbito dorsal por 30 min e índices geométricos da VFC foram avaliados (RRtri, TINN, SD1, SD2 e plot de Poincaré). Resultados: Houve reduções nos índices RRtri, TINN, SD1 e SD2 para o GDP em comparação ao GC. Para a relação SD1/SD2, diferenças significantes não foram observadas entre os grupos. O plot de Poincaré mostrou que indivíduos com DP têm menor dispersão batimento a batimento dos intervalos RR, bem como maior dispersão dos intervalos RR a longo prazo em relação ao GC. Conclusão: Os resultados sugerem haver diminuição da modulação autonômica parassimpática e da variabilidade global em indivíduos com DP em relação a indivíduos sem a doença, as quais são independentes de sexo, idade e índice de massa corporal.

Evaluation of the Autonomic Nervous System in Chronic Chagasic Cardiopathy: A Systematic Review of the Literature

Abstract Background The importance of regional sympathetic denervation in the pathophysiology and prognosis of Chagas disease has been recognized. Objective To conduct a review of studies that have assessed dysautonomia in chronic Chagas heart disease. Methods The search was performed on the Medline, Pubmed, Lilacs and SciELO databases. The inclusion criteria were: original articles published in full; studies on individuals with Chagas disease, that used diagnostic methods for chagasic cardiomyopathy, and had clear inclusion and exclusion criteria. Duplicate studies, studies including children (0 to 10 years old), studies involving animals, in vitro experiments, case reports, editorials, theses, and dissertations were excluded. Results A total of 281 articles were retrieved, and 10 met the inclusion criteria and were analyzed. There was great heterogeneity as to the technique for assessing dysautonomia, groups of patients studied and classification of Chagas disease. The methods used for studying the autonomic system was immunohistochemistry (n=1), Valsalva and tilt-test (n=1), scintigraphy (n=6) and Holter monitoring (n=2). The results indicated dysautonomia in the indeterminate, digestive and cardiac forms of Chagas disease, and sympathetic denervation in the indeterminate and cardiac forms of the disease. There was agreement between areas of denervation, hypoperfusion and fibrosis, but areas of denervation were larger than those of hypoperfusion. The frequency of denervation and its extension increased from the indeterminate to the cardiac form. There was an association between extension of denervation and previous history of malignant ventricular arrhythmia. Conclusions The evidence presented in this review supports that an early diagnosis of autonomic denervation in chronic Chagas' disease allows the identification of patients with an increased risk of sudden death. Int J Cardiovasc Sci. 2020; [online].ahead print, PP.0-0

Disautonomías neurovegetativas: un caso clínico interesante / Neurovegetative dysautonomies: an interesting clinical case

RESUMEN Fundamento: la hiperactividad simpática paroxística consiste en episodios autolimitados de hipertensión arterial, taquicardia, taquipnea, hiperhidrosis, disminución del nivel de conciencia, aumento del tono muscular con postura en extensión, hipertermia, sialorrea y midriasis. Con frecuencia se retrasa su reconocimiento lo que incrementa la morbilidad y mortalidad. Objetivo: conocer la importancia de un diagnóstico y tratamiento precoz de la enfermedad para mayor supervivencia del paciente afectado. Presentación del caso: paciente de 33 años de edad, femenina, que desarrolló una hiperactividad simpática paroxística asociada con hidrocefalia obstructiva. Conclusiones: debe sospecharse la enfermedad en pacientes con daño cerebral agudo de diversas causas. El diagnóstico temprano es vital para evitar estudios diagnósticos e intervenciones innecesarias e iniciar un tratamiento rápido y apropiado que modifique la evolución del síndrome.

ABSTRACT Background: paroxysmal sympathetic hyperactivity consists of self-limited episodes of arterial hypertension, tachycardia, tachypnea, hyperhidrosis, decreased level of consciousness, increased muscle tone with extension posture, hyperthermia, sialorrhea and mydriasis. Frequently their recognition is delayed, which increases morbidity and mortality. Objective: to make known the importance of an early diagnosis and treatment of the entity for greater survival of the affected patient. Case report: a 33-years-old female patient who developed a paroxysmal sympathetic hyperactivity associated with obstructive hydrocephalus. Conclusions: the entity should be suspected in patients with acute brain damage of various etiologies. Early diagnosis is vital to avoid unnecessary diagnostic studies and interventions and to initiate a rapid and appropriate treatment that modifies the evolution of the syndrome.

Brain Hypometabolism in Cerebellar Ataxia, Neuropathy, Vestibular Areflexia Syndrome / 대한평형의학회지

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a slowing progressive ataxic disorder characterized by bilateral vestibulopathy, cerebellar ataxia and somatosensory impairment. Autonomic dysfunction is recently considered as a core feature in CANVAS in addition to these symptoms. In most cases, patients with CANVAS show cerebellar atrophy in brain imaging, but some cases show minimal or no atrophy of cerebellum. Brain (18F)-fluoro-2-deoxy-D-glucose positron emission tomography (¹⁸F-FDG PET) study can be a complimentary tool to diagnosis CANVAS in cases of no structural abnormality such as cerebellar atrophy. Hereby, we present a case of CANVAS with minimal atrophy of cerebellum but showing a prominent hypometabolism in cerebellum, thalamus and posterior cingulate cortex in ¹⁸F-FDG PET.

Autonomic thermoregulatory dysfunction in neurofibromatosis type 1 / Disfunção autonômica termorregulatória na neurofibromatose do tipo 1

ABSTRACT Objective Neurofibromatosis type 1 (NF1) causes neural and cutaneous disorders and reduced exercise capacity. Exercise/heat exposure increasing internal temperature must be compensated by eccrine sweat function and warmed skin vasodilation. We suspected NF1 could adversely affect eccrine sweat function and/or vascular thermoregulatory responses (VTR). Methods The eccrine sweat function and VTR of 25 NF1 volunteers (14 males, 11 females; 16–57 years old) were compared with 23 non-NF1 controls matched by sex, age, height and weight (CG). Sweating was induced by 1) pilocarpine 1% iontophoresis (PILO); and 2) by passive heating (HEAT) via the lower third of the legs being immersed in 42°C water for one hour. Previously established eccrine sweat function and VTR protocols were used. Results The NF1 group showed: a) lower sweat rate than the CG group during PILO; b) a smaller diastolic pressure decrease; and c) higher tympanic temperatures than controls during HEAT (p < 0.05). Conclusion Reduced sweating and vascular thermoregulatory responses suggest autonomic dysfunction in NF1 individuals.

RESUMO Objetivo Neurofibromatose do tipo 1 (NF1) causa problemas neurais e cutâneos e diminuição da capacidade física. O aumento da temperatura interna durante exercício e exposição ao calor precisa ser compensada pela função sudorípara écrina (FSE) e aquecimento cutâneo por vasodilatação (RVT). Suspeitou-se clinicamente que a NF1 poderia prejudicar a FSE e a RVT. Métodos A FSE e RVT de 25 voluntários com NF1 (14 homens, 11 mulheres; 16–57 anos) e de 23 sem-NF1, emparelhados por sexo, idade, estatura e peso corporal, foram medidas com protocolos validados anteriormente. A sudorese foi induzida por iontoforese com pilocarpina (PILO) e aquecimento passivo por imersão das pernas em água a 42°C durante uma hora (HEAT). Resultados O grupo NF1 apresentou menor taxa de sudorese na situação PILO, menor redução da pressão diastólica e maior temperatura timpânica na situação HEAT (p < 0.05). Conclusão As respostas sudorípara e vascular reduzidas sugerem disfunção autonômica nas pessoas com NF1.

COMPARAÇÃO DA MODULAÇÃO AUTONÔMICA CARDÍACA DURANTE ESFORÇO DE FUMANTES E NÃO FUMANTES / COMPARISON OF CARDIAC AUTONOMIC MODULATION DURING EFFORT OF SMOKERS AND NON-SMOKERS / COMPARACIÓN DE LA MODULACIÓN AUTONÓMICA CARDÍACA DURANTE ESFUERZO DE LOS FUMADORES Y LOS NO FUMADORES

Introdução O tabagismo é uma das principais causas de morbimortalidade em todo o mundo. Indivíduos fumantes têm risco aumentado de desenvolver disfunção autonômica, a qual pode ser avaliada tanto pela variabilidade da frequência cardíaca (VFC) como pela frequência cardíaca de recuperação (FCRec). A maioria dos estudos envolvendo esta análise é realizada em adultos de idade avançada e na condição de repouso. Objetivos Comparar a atividade autonômica cardíaca em repouso e esforço, em homens jovens, fumantes e não fumantes. Métodos Trinta e dois jovens voluntários, (idade 22,0 ± 2,8 anos) foram distribuídos em dois grupos: o grupo fumante (GF; n=15) e o não fumante (GNF; n=17). Realizou-se o teste de Cooper, com análise da VFC pelo cardiofrequencímetro Polar(r)s810i, em repouso e durante o esforço e FCRec. Resultados No GF, 73% foram classificados com nível de dependência nicotínica "muito baixa" segundo questionário de Fargeström. A classificação de ativos e muito ativos pelo questionário IPAQ correspondeu a mais de 50% da amostra em ambos os grupos. Não se observou diferenças significativas entre os grupos na VFC, tanto no repouso quanto no esforço. Entretanto, em cada grupo, notou-se diferença na maioria dos índices de VFC do repouso para o esforço. No teste de Cooper não foram observadas diferenças significativas na FCmédia, FCpico e de FCRec entre os grupos, mas notou-se uma melhor capacidade funcional no GNF pela distância caminhada (2050,2 ± 300,0 vs. 1780,3 ± 390,4 m, p=0,036). Conclusão O GF apresentou menor capacidade funcional e ativação parassimpática durante o esforço, além de menores índices de VFC durante repouso, o que pode sugerir um comprometimento precoce na modulação autonômica cardíaca.

Introduction Smoking is one of the major causes of morbidity and mortality worldwide. Smokers have an increased risk of developing autonomic dysfunction, which can be evaluated by both heart rate variability (HRV) and heart rate recovery (HRRec). Most studies involving this analysis is performed on adults of advanced age and in condition of rest. Objectives To compare the cardiac autonomic activity, at rest and during exercise in young men, smokers and non-smokers. Methods Thirty-two young volunteers (aged 22.0 ± 2.8 years) were divided into two groups: the smoking group (SG, n = 15) and non-smokers (NSG, n = 17). We carried out the Cooper test and HRV analysis by heart rate monitor Polar(r)s810i, at rest and during exercise and HRRec. Results In SG, 73% were classified as having a "very low" level of nicotine dependence according to the Fargeström's questionnaire. Classification of active and very active by the IPAQ questionnaire accounted for over 50% of sample in both groups. There were no significant differences between groups in HRV, both at rest and during exercise. However, in each group, difference has been noted in most HRV indexes from rest to exercise. During Cooper test there were no significant differences in mean HR, HRpeak and HRRec between groups, but a better functional capacity was noted in NSG in the distance walked (2050.2 ± 300.0 vs. 1780.3 ± 390.4m, p = 0.036). Conclusion The SG showed lower functional capacity and parasympathetic activation during exercise, besides lower HRV indexes during rest, suggesting an early impairment in cardiac autonomic modulation.

Introducción El consumo de cigarrillos es una de las más importantes causas de morbilidad y mortalidad en todo el mundo. Los individuos fumadores tienen mayor riesgo de desarrollar disfunción autonómica, que puede ser evaluada tanto por la variabilidad del ritmo cardíaco (VRC) como por la frecuencia cardiaca de recuperación (FCrec). La mayoría de los estudios con estos análisis se lleva a cabo en las personas más viejas y en situación de reposo. Objetivos Comparar la actividad autonómica cardiaca, en momento de reposo y durante ejercicios, en hombres jóvenes, fumadores y no fumadores. Métodos Treinta y dos voluntarios jóvenes (edad 22,0 ± 2,8 años) fueron divididos en dos grupos: grupo de fumadores (GF; n = 15) y no fumadores (GNF; n = 17). Se realizó la prueba de Cooper con el análisis de la VRC por pulsómetro Polar(r)s810i, durante reposo y ejercicio y FCrec. Resultados En el GF, el 73% fueron clasificados con niveles de dependencia por la nicotina "muy bajo", según cuestionario Fargeström. La clasificación de activos y muy activos, mediante cuestionario IPAQ, correspondió a más de 50% de la amuestra en ambos los grupos. No hubo diferencia significativa entre los grupos en la VRC, tanto en reposo cuanto en ejercicio. Sin embargo, en cada grupo, observamos una diferencia en la mayoría de los índices de VRC del reposo para el ejercicio. En la prueba de Cooper no se observó diferencias significativas en las FC media, FCpico y FCrec, pero se verificó una mejor capacidad funcional en el GNF por la distancia caminada (2050.2 ± 300.0 vs. 1780,3 ± 390,4 m, p = 0,036). Conclusión El GF mostró una capacidad funcional y una activación parasimpática más baja durante el ejercicio, así como tasas más bajas de VRC en reposo, lo que puede sugerir un deterioro temprano en la modulación autonómica cardíaca.

Neurogenic Bladder Dysfunction Caused by Seronegative Autoimmune Autonomic Ganglionopathy

Autoimmune autonomic ganglionopathy is a form of acquired autonomic failure affecting parasympathetic, sympathetic functions, usually affecting healthy young people. The disorder affects both sympathetic and parasympathetic nervous systems, with acute onset, monophasic course, and partial recovery with relative preservation of motor and sensory function. We experienced a case of young man with acute autoimmune autonomic ganglionopathy who developed voiding difficulty, sudden blurred vision and gastrointestinal discomfort without motor or sensory dysfunction. Fever developed 5 days earlier and persisted until onset of autonomic failure. Patient complained voiding difficulty and urodynamic study revealed detrusor are flexia with failure to initiate and sustain adequate detrusor contraction. Sympathetic skin response and several autonomic function tests showed abnormalities. Intravenous immunoglobulin was applied for 5 days but symptoms persisted. Thus, 5 days of plasmapheresis treatment was followed showing improvements in most of the symptoms. However bladder dysfunction persisted at 6 months follow-up, showing partial recovery at bethanechol administration.

Disautonomía: presentación de una serie de casos manejados con repolarizador magnético celular / Dysautonomia: serial cases managed with magnectic cellular repolarized system

Objetivo: describir el comportamiento de la disautonomía mediante su tratamiento con terapias complementarias como la medicina alternativa con repolarizador magnético celular (RMC). Metodología: estudio de serie de casos en pacientes disautonómicas en quienes se empleó el RMC, en quienes se analizaron características demográficas, presencia de síncopes y de síntomas presincopales, cambios en las cifras de presión arterial y frecuencia cardiaca, y resultados de calidad de vida (se utilizaron 2 cuestionarios SF36 y EQ-5D y se agruparon las pacientes en 4 grupos de acuerdo con las puntuaciones). Resultados: se encontró similitud con otras series en cuanto a datos demográficos, predominio y severidad en los síntomas en el sexo femenino con edades entre 16 y 25 años, además de pesos ligeros. Todas las pacientes presentaron pródromos en algún momento de su enfermedad, mientras los síncopes se presentaron en el 57%. En la presión arterial 6/7 presentaron hipotensión y la frecuencia cardiaca tuvo un promedio de 70 latidos/minuto y 80 latidos/minuto en el inicio y en el seguimiento, respectivamente. En los puntajes se presentó mejoría de la calidad de vida, si se compara el seguimiento con el inicio. Conclusiones: la disautonomía afecta más a mujeres jóvenes y delgadas, está relacionada con regular a mala calidad de vida, pero ésta mejora sustancialmente con tratamientos bien instaurados, como aumento en la ingesta de sal y líquidos y con maniobras de contrapresión para mejorar el retorno vascular. También se observó mejoría con el RMC.

Objective: to describe the behavior of disautonomia through its management with complementary therapies like alternative medicine with magnetic cellular repolarized system (MCRS).Methods: case study done in disautonomic patients that were managed with MCRS, in whom the demographic characteristics, the presence of syncopes and presyncopal symptoms, changes in the blood pressure and heart rate, and the results of quality of life were analyzed (two questionnaires were used, SF36 and EQ-5D, and the patients were grouped in four groups according to the punctuations).Results: there were similarities found with other series in regards to demographic data, finding a prevalence and severity in the female symptoms with ages between 16 and 25 years old, in addition to light weight. All of the patients presented prodromes sometime during their disease, while the syncopes were presented by 57% of them. In blood pressure 6/7 presented hypotension and the heart rate had an average of 70 beats per minute and 80 beats per minute at the beginning and follow up respectively. In the punctuation there was improvement of the quality of life, comparing the follow up with the beginning. Conclusions: disautonomia affects more young and thin women, it is related to regular and bad quality of life, but it gets better with well-established treatment, basic measures such as the increase of salt and liquid consumption and compression maneuver to improve the vascular return, improvement was also seen with MCRS.

Trastornos autonómicos en enfermedad de Parkinson / Autonomic disturbances in Parkinson disease

The current concept of Parkinson Disease comprises a group of non-motor symptoms. Among these, dysautonomia is a common problem that deteriorates the quality oflife ofpatients. In this article we review the most common dysautonomic manifestations that are observed in cardiovascular, gastrointestinal, urinary, genital and skin systems. Their possible role as risk factors, premotor symptoms and their implications in the pathogenesis of Parkinson Disease are discussed. A general appro-ach to the main syndromes, based in the available evidence and in our experience is also presented.