Crisis adrenérgica como forma de debut de un neuroblastoma / Adrenergic crisis as a debut form of a neuroblastoma

INTRODUCCIÓN: La forma clínica de presentación más común del neuroblastoma es el de una masa abdominal, pero puede presentarse con sintomatología menos habitual, como es la crisis adrenérgica por liberación de catecolaminas. OBJETIVO: Describir una forma de presentación inusual de neuroblastoma y el amplio diagnóstico diferencial que existe en un lactante con síntomas adrenérgicos. CASO CLÍNICO: Lactante femenina de 7 semanas de vida, consultó por historia de tres semanas de sudoración e irritabilidad a lo que se asoció fiebre de 24 h de evolución y dificultad respiratoria. Al ingreso presentaba mal esta do general, irritabilidad, sudoración, enrojecimiento facial, taquipnea y palidez cutánea, taquicardia sinusal extrema e hipertensión arterial (HTA), interpretadas como sintomatología adrenérgica. Se completó el estudio con una ecografía abdominal y resonancia magnética que mostraron una gran masa retroperitoneal compatible con neuroblastoma. Las catecolaminas en sangre y en orina mostraron altos niveles de dopamina, adrenalina y noradrenalina, probablemente de origen tumoral. Se inició tratamiento antihipertensivo con fármacos alfa bloqueantes con buen control de la tensión arterial. Se resecó quirúrgicamente el tumor sin incidencias y con una adecuada recuperación posterior. La paciente presentó evolución favorable a tres años de seguimiento. CONCLUSIONES: en un lactante con sintomatología adrenérgica como irritabilidad, enrojecimiento, sudoración asociada a HTA, se debe descartar patología cardiaca, metabólica (hipoglucemia), intoxicaciones y/o patología suprarrenal. Dentro de esta última, el neuroblastoma es la primera posibilidad diagnóstica, por ser uno de los principales tumores en la infancia y aunque esta presentación no es habitual puede producir estos síntomas.

INTRODUCTION: The most common clinical presentation of neuroblastoma is an abdominal mass, but it can present with uncommon symptoms, such as adrenergic storm due to catecholamine release. OBJECTIVE: To describe an unusual presentation of neuroblastoma and the wide differential diagnosis that exists in an infant with adrenergic symptoms. CLINICAL CASE: A 7-week old female infant was evaluated due to a 3-week history of sweating and irritability associated with a 24-hour fever and respiratory distress. At admission, she presented poor general condition, irritability, sweating, facial redness, tachypnea and skin paleness, extreme sinus tachycardia, and high blood pressure (HBP), interpreted as adrenergic symptoms. The study was completed with abdominal ultrasound and magnetic reso nance imaging that showed a large retroperitoneal mass compatible with neuroblastoma. Plasma and urinary catecholamines tests showed high levels of dopamine, adrenaline, and noradrenaline, probably of tumor origin. We started antihypertensive treatment with alpha-blocker drugs, showing a good blood pressure control. The tumor was surgically resected without incidents and adequate subsequent recovery. The patient presented a favorable evolution after three years of follow-up. CONCLUSIONS: In an infant with adrenergic symptoms such as irritability, redness, sweating associated with HBP, it should be ruled out pathology heart or metabolic (hypoglycemia) pathology, intoxications, and/or adrenal pathology. Within this last one, neuroblastoma is the first diagnostic possibility, since it is one of the main tumors in childhood and, although this presentation is not usual, it can produce these symptoms.

Trombo serpiginoso em átrio direito: apresentação atípica de tromboembolismo pulmonar. Relato de caso / Serpiginous thrombus in right atrium: atypical presentation of pulmonary thromboembolism. Case report

O tromboembolismo pulmonar é um grave problema de saúde pública devido ao subdiagnóstico e às elevadas morbidade e mortalidade. Quando a embolia pulmonar é maciça com repercussão hemodinâmica importante e a terapia adequada não ocorre nas primeiras horas, a mortalidade é superior a 85%. Na suspeita clínica de tromboembolismo pulmonar, a avaliação ecocardiográfica pode ter papel fundamental na avaliação da mobilidade e da estrutura do ventrículo direito, presença de hipertensão pulmonar e documentação da presença de trombo. A detecção ecocardiográfica de trombo móvel nas câmaras cardíacas direitas permite identificar um grupo de pacientes de alto risco, com mortalidade muito elevada, quando comparada ao tromboembolismo pulmonar em geral. Além da terapia clínica clássica, com heparinas e trombolíticos, as terapêuticas endovascular e cirúrgica devem ser consideradas e podem contribuir para o prognóstico desses pacientes. Relata-se um caso de uma paciente de 33 anos de idade admitida em uma unidade de emergência da no 8o dia de pós-operatório de apendicectomia, com queixas de dor torácica e dispneia de início súbito. Ecocardiograma transtorácico evidenciou presença de trombo serpiginoso solto em átrio direito, que ocluía intermitentemente a valva tricúspide durante o ciclo cardíaco. Diante das características ecocardiográficas atípicas do trombo e da significativa chance de embolização maciça, optou-se por intervenção cirúrgica de emergência.

Pulmonary thromboembolism is a serious public health problem due to misdiagnosis and high morbidity and mortality. When pulmonary embolism is massive with important hemodynamic repercussion, and the appropriate therapy does not take place in the early hours, mortality is higher than 85%. If there is clinical suspicion of pulmonary thromboembolism, an echocardiographic evaluation may have a key role in the evaluation of mobility and structure of the right ventricle, presence of pulmonary hypertension, and documentation of the presence of thrombus. Echocardiographic detection of mobile thrombus in right cardiac chambers allows the identification of a group of high-risk patients with very high mortality when compared to pulmonary thromboembolism in general . In addition to the classical clinical therapy with heparins and thrombolytics, endovascular and surgical therapy should be considered and may contribute to these patients' prognosis. A case is reported of a 33-year-old female patient admitted to an Emergency Unit at 8th postoperative day (POD) of appendectomy, with complaints of chest pain and dyspnea of sudden onset. Transthoracic echocardiography showed the presence of a floating serpiginous thrombus in the right atrium, which intermittently occluded the tricuspid valve during the cardiac cycle. Due to the atypical echocardiographic features of the thrombus, and significant chance of massive embolization, an emergency surgery was chosen.

Síndrome de hiperatividade simpática paroxística causada por síndrome da embolia gordurosa / Paroxysmal sympathetic hyperactivity syndrome caused by fat embolism syndrome

RESUMO A hiperatividade simpática paroxística representa uma complicação incomum, com potencial risco à vida, de lesões cerebrais graves, mais comumente de origem traumática. Seu diagnóstico clínico se baseia na manifestação recorrente de taquicardia, hipertensão, diaforese, taquipneia e, às vezes, febre, além de posturas distônicas. Os episódios podem ser induzidos por estímulos ou ocorrer de forma espontânea. É comum que ocorra subdiagnóstico desta síndrome, e o retardamento de seu reconhecimento pode aumentar a morbidade e a incapacidade em longo prazo. Evitar os desencadeantes e a farmacoterapia podem ter muito sucesso no controle desta complicação. A síndrome da embolia gordurosa é uma complicação rara, mas grave, das fraturas de ossos longos. Sinais neurológicos, petéquias hemorrágicas e insuficiência respiratória aguda são as características que constituem seu quadro clínico. O termo "embolia gordurosa cerebral" é estabelecido quando predomina o envolvimento neurológico. O diagnóstico é clínico, porém achados específicos de neuroimagem podem confirmá-lo. As manifestações neurológicas incluem diferentes graus de alteração da consciência, défices focais ou convulsões. Seu tratamento é de suporte, porém são possíveis desfechos favoráveis, mesmo nos casos com apresentação grave. Relatamos dois casos de hiperatividade simpática paroxística após embolia gordurosa cerebral, uma associação muito incomum.

ABSTRACT Paroxysmal sympathetic hyperactivity represents an uncommon and potentially life-threatening complication of severe brain injuries, which are most commonly traumatic. This syndrome is a clinical diagnosis based on the recurrent occurrence of tachycardia, hypertension, diaphoresis, tachypnea, and occasionally high fever and dystonic postures. The episodes may be induced by stimulation or may occur spontaneously. Underdiagnosis is common, and delayed recognition may increase morbidity and long-term disability. Trigger avoidance and pharmacological therapy can be very successful in controlling this complication. Fat embolism syndrome is a rare but serious complication of long bone fractures. Neurologic signs, petechial hemorrhages and acute respiratory failure constitute the characteristic presenting triad. The term cerebral fat embolism is used when the neurological involvement predominates. The diagnosis is clinical, but specific neuroimaging findings can be supportive. The neurologic manifestations include different degrees of alteration of consciousness, focal deficits or seizures. Management is supportive, but good outcomes are possible even in cases with very severe presentation. We report two cases of paroxysmal sympathetic hyperactivity after cerebral fat embolism, which is a very uncommon association.

Taquicardia persistente en mujer joven deportista / A young woman with persistent tachycardia

Resumen: Una mujer joven, deportista, evoluciona con compromiso del estado general y síntomas constitucionales, asociado a taquicardia persistente y baja de peso de casi un año de evolución, con laboratorio, electrocardiograma y ecocardiograma inicialmente anodinos.

Abstracts: An athletic young woman presented with almost one year of general symptoms, weight loss, and persistent tachycardia. The initial laboratory, electrocardiogram and echocardiogram findings were unremarkable.

Dengue, presentación inusual en un lactante: reporte de un caso / Dengue with unusual clinical features in an infant: case report

El estado de Yucatán (México) es un área endémica para dengue. Durante el 2015 hubo un incremento en el número de casos esperados. OBJETIVO: Describir y analizar la presentación clínica, evolución y manejo de un caso de infección por dengue con manifestaciones clínicas inespecíficas en un lactante menor, que derivaron en síndrome de choque por dengue. CASO CLÍNICO: Lactante de un mes que consulta por eritema generalizado y antecedente de picadura de insecto. Su manejo hospitalario se basó en la resolución del episodio agudo de anafilaxia. Durante su estadía presentó hipotensión, taquicardia, anemia y dificultad respiratoria. Se derivó a terapia intensiva pediátrica, pero al quinto día de estancia falleció debido a síndrome de choque. La RPC y serología de anticuerpos IgG confirmaron etiología por dengue. CONCLUSIONES: Las infecciones por dengue en el lactante menor pueden cursar afebriles, por lo cual es importante sospecharlas oportunamente ante la presencia de eritema generalizado, taquicardia e hipotensión, con la finalidad de evitar las consecuencias letales del choque por dengue.

The state of Yucatan, in Mexico, is an endemic area for dengue. During 2015, there was an unpredicted increase in the number of expected cases of dengue fever. OBJECTIVE: To describe and analyse the clinical presentation, progress, and management of a case of dengue infection with non-specific clinical manifestations in an infant which resulted in a dengue shock syndrome. CASE REPORT: One month old infant admitted to hospital with a generalised rash and a history of being bitten by an insect. He was diagnosed with anaphylaxis based on clinical manifestations and anamnesis. While in hospital, he developed hypotension, tachycardia, anaemia, and respiratory distress. He was transferred to the intensive care unit, but died on the fifth day. He tested positive to dengue virus in the PCR test and for IgG antibodies using Elisa. The basic cause of death was dengue shock syndrome. CONCLUSIONS: Dengue fever in young infant infections may be afebrile, so it is important to suspect them appropriately in the presence of a generalised rash, tachycardia, and hypotension, in order to avoid the deadly consequences of dengue shock.

Efficacy of nalbuphine in preventing hemodynamic response to laryngoscopy and intubation

To determine the efficacy of nalbuphine in preventing increase in heart rate and mean arterial pressure in response to laryngoscopy and tracheal intubation. This double blind randomized controlled trial was conducted on 100 ASA [American Society of Anesthesiologists] grade I-II patients scheduled for general anesthesia. Patients were randomly allocated to receive either saline [group I, control group, n=50] or nalbuphine 0.2 mg kg-1 [group II, study group, n=50] as a bolus dose 5 minutes before laryngoscopy. Anaesthesia was then induced with propofol [2mg kg-1] and atracurium [0.6mg kg-1] and or tracheal intubation was then performed within 30 seconds. Heart rate [HR] and mean arterial pressures[MAP] were recorded before the administration of the study drug, baseline value [T-0], 3 minutes after study drug administration [T-1], immediately after tracheal intubation [T-2] and then after every 1 minute up to 5 minutes [T3-7] and then after 10 minutes of intubation [T-8]. The Nalbuphine group showed significantly lesser rise in HR compared to control group after laryngoscopy and or tracheal intubation that continued till 10 minutes after intubation [p-value from ?0.0001-0.0297]. The Nalbuphine group also showed significantly lesser rise in MAP compared to control group after laryngoscopy and or tracheal intubation that continued till 5 minutes after intubation [p-value from ?0.0001-0.0152]. At 10 minutes post intubation though the rise in MAP was still lesser in Nalbuphine group than control group but it was not significant [p-value=0.0540]. Nalbuphine 0.2 mg kg-1 prevents a marked rise in heart rate and mean arterial pressure associated with laryngoscopy and or tracheal intubation

Simplified progressive approach for the ablation of scar related atrial macroreentrant tachycardias / Ablación de arritmias por macroreentrada auricular relacionadas con cicatriz posquirúrgica, mediante un abordaje escalonado

Introduction: Radiofrequency ablation of scar related right atrial flutter is challenging. Long procedures, prolonged fluoroscopic times and high percentages of recurrences are of concern. We present a simple and progressive approach based on a single electroanatomic map of the right atrium. Methods: Twenty-two consecutive patients with atrial flutter and history of cardiac surgery were included. An electrophysiologic study was performed to define localization (left or right) and cavo-tricuspid isthmus participation using entrainment mapping. After a critical isthmus was localized, ablation was performed with an external irrigated tip catheter with a power limit of 30 W. Potential ablation sites were confirmed by entrainment. Results: The predominant cardiopathy was atrial septal defect. All arrhythmias were localized in the right atrium; mean cycle length of the clinical flutter was 274 ± 31 ms. Only 40% had cavo-tricuspid isthmus participation. None of the patients with successful ablation had recurrences after 13 ± 9.4 months of follow-up. Conclusions: A progressive approach with only one activation/voltage CARTO® map of the atrium and ablation of all potential circuits is a highly effective method for ablating scar related macroreentrant atrial arrhythmias.

Introducción: La ablación con radiofrecuencia de flutter auricular relacionado con cicatrices posquirúrgicas es compleja. Procedimientos prolongados, con tiempos de fluoroscopia altos y una tasa de recurrencia elevada son problemas habituales. Mostramos un abordaje simple y progresivo basado en un solo mapa de cartografía electroanatómica de la aurícula derecha. Métodos: Se incluyeron 22 pacientes consecutivos con flutter auricular e historia de cirugía cardiaca. Se realizó estudio electrofisiológico para definir la localización del circuito de flutter (derecho o izquierdo) y la participación o no del istmo cavotricuspideo mediante encarrilamiento. Una vez localizado la zona de conducción lenta o critica del circuito, se realizó ablación con radiofrecuencia con catéter de irrigación externa a 30W. Posteriormente se llevó a cabo ablación de todos los circuitos potenciales. Resultados: La cardiopatía más dominante fue la comunicación interauricular. Todas las arritmias se localizaron en la aurícula derecha. El ciclo de flutter fue de 274 ± 31 ms. En solo 40% de los casos se demostró participación del istmo cavotricuspideo. No se observaron recurrencias de la arritmia durante un seguimiento de 13 ± 9.4 meses. Conclusiones: Este abordaje escalonado con un solo mapa CARTO® de activación/voltaje de la aurícula y la ablación de todos los circuitos potenciales es altamente efectivo para el tratamiento de arritmias por macrorreentrada relacionadas con cicatriz posquirúrgica.

Evolução clínica e capacidade funcional de pacientes com cardiomiopatia dilatada após quatro anos do transplante / Clinical and functional capacity of patients with dilated cardiomyopathy after four years of transplantation

OBJETIVO: Avaliar a evolução do paciente miocardiopata após transplante (Tx) cardíaco, analisando sua sobrevida, complicações trans e pós-operatórias e respostas cardiovasculares após cerca de quatro anos do procedimento cirúrgico. MÉTODOS: A pesquisa foi realizada no período de fevereiro a maio de 2011, com pacientes submetidos a Tx cardíaco no Hospital Dr. Carlos Alberto Studart Gomes - Hospital de Messejana (HDM). A amostra foi composta de todos os pacientes transplantados no ano de 2007 no referido hospital. Inicialmente, foi aplicada uma ficha de avaliação, coletando dados dos prontuários, sobre a evolução do paciente no período trans e pós-operatório até a alta hospitalar. Após a coleta dessas informações, os pacientes foram submetidos ao teste da caminhada dos seis minutos (TC6). Os valores encontrados na distância percorrida foram comparados aos valores de referência esperados para a população utilizando a equação de Enright e Sherrill. RESULTADOS: Do total de 24 pacientes que realizaram Tx cardíaco no HDM no ano de 2007, 14 foram avaliados e 10 excluídos do estudo. Com relação às complicações, no período transoperatório, a mais evidenciada foi a disfunção do ventrículo direito (64,3%) e, no pós-operatório, quadro de taquicardia (64,3%). Analisando o TC6 observou-se diminuição de 11,6% na distância percorrida quando comparada à distância estimada (486 ± 55 m, 550 ± 59 m, respectivamente). CONCLUSÃO: Os resultados obtidos neste estudo perante o TC6 evidenciam que as respostas cardiovasculares dos pacientes avaliados estão abaixo do estimado, contudo dentro da faixa de normalidade estabelecida.

OBJECTIVE: To evaluate patient with cardiomyopathy's progress after cardiac transplant, by analyzing his survival, complications and cardiovascular responses after nearly four years of surgery. METHODS: The survey was conducted from February to May 2011, with patients undergoing cardiac transplantation at Dr. Carlos Alberto Studart Gomes Hospital - Messejana Hospital (HDM). The sample consisted of all transplanted patients in 2007 in this hospital. Initially an evaluation form developed by the researchers, which was based on collected data from patients' medical records, was applied, about trans and postoperative period. After collecting these informations, patients underwent the six-minute walk test (6WT). The marks found in walking distance were compared with reference marks expected for this population by using Enright and Sherrill's equation. RESULTS: From all the 24 patients who underwent cardiac transplantation in HDM in 2007, 14 were evaluated and 10 were excluded. Regarding the complications, in the trans-operatory period, the most evident was the right ventricular dysfunction (64.3%) and tachycardia (64.3%) was more evident on the postoperative period. Analyzing the 6WT it was observed a decrease of 11.6% in walking distance when compared with the estimated distance (486 ± 55 m, 550 ± 59 m, respectively). CONCLUSION: Survival of heart transplant patients was equivalent to about 70%. The results of this study before the 6WT showed that patients' cardiovascular responses are below the estimated, nevertheless within the normal range established.

Taquicardia irregular de QRS ancho en el servicio de urgencia / Irregular tachycardia of wide QRS in the emergency department

La consulta por taquiarritmias en el servicio de urgencia es frecuente. Es importante realizar un correcto diagnóstico diferencial de las taquiarritmias en general y de las taquicardias irregulares de complejo ancho en particular. Una de sus posibles causas podría ser la fibrilación auricular en presencia de preexcitación (síndrome Wolff Parkinson White). El estudio electrofisiológico puede ser diagnóstico y terapéutico.

Treinamento físico intervalado como ferramenta na terapia cognitivo-comportamental do transtorno de pânico / Interval aerobic training as a tool in the cognitive-behavioral treatment of panic disorder

Este trabalho consiste no relato da utilização de um programa de exercícios físicos aeró-bicos intervalados no contexto da terapia cognitivo-comportamental em um caso de transtorno de pânico e agorafobia. São descritos os procedimentos utilizados, bem como os resultados positivos obtidos na redução da ansiedade cardíaca e na ansiedade cotidiana em relação a situações que exigem esforço físico.

The present article reports the use of an interval aerobic exercise program as an adjunct intervention in the context of cognitive behavioural therapy in a patient with panic disorder and agoraphobia. We describe the procedures used in the exercise program and also the positive results achieved regarding cardiac anxiety reduction and everyday anxiety reduction concerning activities that requested physical effort.

Effects of sedation during upper gastrointestinal endoscopy on endocrine response and cardiorespiratory function

Upper gastrointestinal endoscopy is often accompanied by tachycardia which is known to be an important pathogenic factor in the development of myocardial ischemia. The pathogenesis of tachycardia is unknown but the condition is thought to be due to the endocrine response to endoscopy. The purpose of the present study was to investigate the effects of sedation on the endocrine response and cardiorespiratory function. Forty patients scheduled for diagnostic upper gastrointestinal endoscopy were randomized into 2 groups. While the patients in the first group did not receive sedation during upper gastrointestinal endoscopy, the patients in the second group were sedated with intravenous midazolam at the dose of 5 mg for those under 65 years or 2.5 mg for those aged 65 years or more. Midazolam was administered by slow infusion. In both groups, blood pressure, ECG tracing, heart rate, and peripheral oxygen saturation (SpO2) were monitored during endoscopy. In addition, blood samples for the determination of cortisol, glucose and C-reactive protein levels were obtained from patients in both groups prior to and following endoscopy. Heart rate and systolic arterial pressure changes were within normal limits in both groups. Comparison of the two groups regarding the values of these two parameters did not reveal a significant difference, while a statistically significant reduction in SpO2 was found in the sedation group. No significant differences in serum cortisol, glucose or C-reactive protein levels were observed between the sedated and non-sedated group. Sedation with midazolam did not reduce the endocrine response and the tachycardia developing during upper gastrointestinal endoscopy, but increased the reduction in SpO2.

Síndrome de choque por dengue (SCD) y embarazo: reporte de un caso / Syndrome of shock by dengue (ACD) and pregnancy: a case report

El Dengue Hemorrágico (DH) es una variante del Dengue, que cursa con alteraciones hemostáticas y vasculares potencialmente fatales. El Síndrome de Choque por Dengue (SCD) es la forma más severa del DH. A continuación, se presenta un caso de SCD en una paciente con gestación de 9 semanas, y su manejo. Actualmente no se cuenta con conocimiento suficiente acerca del comportamiento de esta enfermedad en pacientes embarazadas. Vale la pena destacar, que no hallamos ningún reporte de SDC en pacientes embarazadas, ni encontramos pautas para el manejo de las mismas, por lo que consideramos de mucha importancia la publicación y diseminación del mismo, como base para nuevas investigaciones.

Neonatal arrhythmias.

OBJECTIVE: Neonatal arrhythmias are not uncommon; however, they rarely cause hemodynamic compromise. This paper aims to study the etiology, spectrum and outcome of neonates with arrhythmias who presented to a pediatric department. METHODS: All neonates, either inborn or brought to the pediatric emergency with rhythm disorders, between August 1999 to August 2002, were included prospectively. Evaluation including a search for secondary causes of rhythm disorder and a chest X-ray, standard 12-lead electrocardiography and echocardiography in all. The management required in each and the outcomes were noted. RESULTS: Nine neonates were identified, of which 4 were inborn. Tachycardia was seen in 8 neonates and bradycardia in only one. Three neonates had an antenatal onset of arrhythmias; in the rest it was postnatal in onset. Five neonates had a secondary rhythm disorder, secondary to metabolic derangements in 4 and a cardiac mass in 1. Five had ventricular arrhythmias and 5 had hemodynamic compromise due to the arrhythmia. The outcome was poor in 4 and was related to the underlying illness. CONCLUSION: Tachyarrhythmia is more common than bradyarrhythmia in the neonate. Arrhythmias secondary to various metabolic causes are more common than primary rhythm disorders.

Persistent narrow complex tachycardia: what is the diagnosis?

Supraventricular tachycardia [SVT] with long RPinterval and short PR interval is a unique form of tachycardia. The differential diagnosis includes sinus tachycardia, focal atrial tachycardia, atrial flutter with two to one ventricular response and atrioventricular reciprocating tachycardia with slow retrograde ventriculoatrial conduction. In this report, we present a case of long RP SVT and a review of the electrocardiographic features of each type of tachycardia

Mapeo electroanatómico 3D y ablación percutánea transtorácica de taquicardia auricular en pacientes post cirugía de Fontan / Electro anatomic 3-D mapping and percutaneous transthoracic ablation of atrial tachycardia after Fontan surgery

Introducción: La incidencia de taquicardias auriculares (TA) luego de la operación de Fontan es alta. El acceso a la aurícula pulmonar (AuP) requiere de una punción transeptal o acceso aórtico retrógrado. Se presentan resultados iniciales con una técnica percutánea que permite el acceso a la AuP para mapeo y ablación por radiofrecuencia (ARF). Métodos: Seis EEF con mapeo 3D electroanatómico (CARTO) y ARF se efectuaron en 5 pacientes (1.2 a 17 años). Todos tenían un Fontan (túnel lateral) y TA. Vía transtoráxica se posicionó en la AuP un catéter Navistar para mapeo, estimulación y ARF. Se indujo TA con estimulación programada e isoproterenol. La secuencia de activación, áreas de bloqueo eléctrico y anatómico, y la ubicación del electrograma de His fueron definidas con mapeo 3D electroanatómico. Las zonas de interés fueron estudiadas con técnicas de “entrainment”. Luego de la ARF, el éxito se definió como la imposibilidad de reinducir TA. Resultados: Se encontró taquicardia por reentrada auricular en 5 casos y taquicardia ectópica en uno. La ARF fue exitosa en todos ellos. Hubo 1 pneumotórax y hemotórax en dos casos. En un paciente se observó recurrencia de la TA a los 3 meses, que requirió una segunda ARF, sin nueva recidiva. Ningún paciente esta recibiendo tratamiento antiarrítmico. Resumen: El acceso transtorácico percutáneo a la AuP parece apropiado para mapear y tratar las taquicardias auriculares en pacientes con cirugía de Fontan.

Distrofia miotônica e cardiopatia: comportamento dos eventos arrítmicos e dos distúrbios da condução / Myotonic dystrophy and heart disease: behavior of arrhythmic events and conduction disturbances

OBJETIVO: Estudar a prevalência e a evolução natural dos eventos arrítmicos e distúrbios da condução, correlacionar o defeito genético com achados cardiovasculares, avaliar a mortalidade cardíaca, freqüência e fatores preditivos de morte súbita, correlacionar a gravidade do envolvimento neuromuscular e cardíaco e definir o papel do estudo eletrofisiológico (EEF), na distrofia miotônica. MÉTODOS: Realizados periodicamente avaliação clínica e exames complementares, exame genético, eletrocardiograma, ecocardiograma e Holter (exceto exame genético) em 83 pacientes consecutivos com tempo médio de seguimento de 42±30,63 meses, sendo o estudo eletrofisiológico realizado em 59 casos. RESULTADOS: Taquiarritmia atrial foi observada em 10 (12 por cento) pacientes, taquicardia ventricular não sustentada (TVNS) em 14 (17 por cento), bloqueio átrio-ventricular (BAV) 1° grau em 24 (29 por cento), bloqueio de ramo esquerdo (BRE) em 19 (23 por cento), bloqueio de ramo direito (BRD) em 13 (16 por cento). Sintomas, aumento do intervalo PR, alargamento do QRS, fração de ejeção do ventrículo esquerdo (FEVE) <60 por cento e idade foram preditivos de óbito. Ocorreram 9 mortes (4 súbitas; 2 insuficiência cardíaca; 3 outras). EEF: HV>70ms em 34 por cento e >100ms em 11 por cento (pós-procainamida). CONCLUSÃO: A prevalência dos eventos arrítmicos e distúrbios da condução foi de 50 por cento a 80 por cento após 6 anos, não se correlacionando ao defeito genético, sendo o flutter atrial, a arritmia sustentada mais freqüente. O envolvimento cardíaco aumentou com a piora da doença neuromuscular, mas essa progressão foi mais rápida que a neuromuscular. A mortalidade total foi baixa (11 por cento) e morte súbita ocorreu em metade dos casos. EEF identificou grupo de risco para implante de marcapasso.

Henoch schonlein purpura with rheumatic carditis.

Henoch Schonlein Purpura with acute rheumatic carditis is a rare entity and only few cases have been reported. An 8 year-old-girl presented with abdominal pain, arthralgia and rashes and was diagnosed as a case of Henoch Schonlein Purpura. She was managed conservatively and discharged. She was readmitted after 1 week with abdominal pain, fever and cough. She developed tachycardia with gallop rhythm on the third day of admission and pansystolic murmur of mitral regurgitation. Echocardiography showed features of myopericarditis, mild pericardial effusion and mitral regurgitation. She was diagnosed and managed as a case of acute rheumatic carditis.