Cardiomiopatia não compactada: doença do nó sinusal como manifestação inicial tratada com marcapasso / Noncompaction cardiomyopathy: sinus node disease as an initial manifestation treated with bicameral pacemaker

A cardiomiopatia não compactada é uma doença congênita, que resulta de falha da compactação do miocárdio na vida embrionária. Nesse processo, há a persistência de trabeculações e recessos profundos, que se comunicam com a cavidade ventricular e geram espessamento do miocárdio em duas camadas distintas. O aspecto clínico dessa doença tanto em crianças como em adultos é muito heterogêneo, variando desde a ausência de sintomas até a tríade composta por insuficiência cardíaca congestiva, arritmias e tromboembolismo sistêmico, porém bradicardias sintomáticas são muito raras. Relatamos o caso de uma paciente com doença do nó sinusal como manifestação inicial de cardiomiopatia não compactada

Noncompaction cardiomyopathy is a congenital disease that results frommyocardial compaction failure in embryonic life. In this process there is the persistence of deep trabeculations and recesses that communicate with the ventricular cavity, resulting in myocardial thickening in two distinct layers. The clinical aspect of this disease in both children and adults is very heterogeneous, ranging from absence of symptomatology to a triad including congestive heart failure, arrhythmias and systemic thromboembolism. However, symptomatic bradycardias are very rare. We report the case of a patient with sinus node disease as the initial manifestation of non-compaction cardiomyopathy

Implante de marcapasso cardíaco definitivo por disfunção do nó sinusal, utilizando a veia cava superior, após angioplastia do trajeto em pós-operatório tardio de operação de Mustard e Senning / Permanent cardiac pacemaker implatation due to sinus node dysfunction, using the superior vena cava, after angioplasty in the late postoperative period of Mustard and Senning procedure

A síndrome do nó sinusal está comumente vinculada às lesões de células sinusais ou da junção sinoatrialou a afecções do tecido intersticial que circunda essas células. Nos casos avançados, o marcapasso cardíaco artificialé o tratamento mais difundido. O caso descrito relata a síndrome do nó sinusal de um paciente em evolução tardiado pós-operatório de operação de Mustard e Senning com complicações anatômicas, levando à necessidade deimplante de marcapasso definitivo, após angioplastia do trajeto, utilizando a veia cava superior.

Sick sinus syndrome is usually related to lesions of the sinus node and sinoatrial node cells or tocollagen tissue disease surrounding these cells. In advanced cases, pacemaker implantation is the most widespreadtreatment option. This is a case report of a patient with sick sinus syndrome in the late postoperative period ofMustard and Senning procedure with anatomical complications requiring definitive pacemaker implantationafter angioplasty through the superior vena cava.

Implante de marcapasso cardíaco definitivo em paciente com síndrome bradicardia-taquicardia, transplantado renal e com persistência de veia cava superior esquerda / Pacemaker implantation in a patient with bradycardia-tachycardia syndrome, kidney transplantation and persitent left superior vena cava

A persistência da veia cava superior esquerda é rara, principalmente quando associada a ausência da veiacava superior direita, e mais ainda quando associada a síndrome bradicardia-taquicardia. Relata-se o caso em queo diagnóstico dessa anomalia venosa foi feito durante o implante de marcapasso cardíaco artificial definitivo paratratamento de síndrome bradicardia-taquicardia. O paciente havia sido submetido a transplante renal e apresentavafunção renal limítrofe, razão pela qual se optou por não utilizar contraste para realização de venografia, dandopreferência à análise do trajeto do fio guia.

Persistent left superior vena cava is a rare anomaly, mainly when associated with absent right superiorvena cava, especially when associated with bradycardia-tachycardia syndrome. We report the case of a patient,whose diagnosis of venous anomaly was made during definitive artificial pacemaker implantation for treatment ofbradycardia-tachycardia syndrome. The patient had been submitted to a kidney transplantation and had borderlinerenal function, and therefore we chose not to use a guidewire instead of contrast media to perform venography.

Implantation of lumenless pacing leads at the inter-atrial septum and right ventricular outflow tract with deflectable catheter-sheath / 华中科技大学学报（医学）（英德文版）

Current permanent right ventricular and right atrial endocardial pacing leads are implanted utilizing a central lumen stylet. Right ventricular apex pacing initiates an abnormal asynchronous electrical activation pattern, which results in asynchronous ventricular contraction and relaxation. When pacing from right atrial appendage, the conduction time between two atria will be prolonged, which results in heterogeneity for both depolarization and repolarization. Six patients with Class I indication for permanent pacing were implanted with either single chamber or dual chamber pacemaker. The SelectSecure 3830 4-French (Fr) lumenless lead and the SelectSite C304 8.5-Fr steerable catheter-sheath (Medtronic Inc., USA) were used. Pre-selected pacing sites included inter-atrial septum and right ventricular outflow tract, which were defined by ECG and fluoroscopic criteria. All the implanting procedures were successful without complication. Testing results (mean atrial pacing threshold: 0.87 V; mean P wave amplitude: 2.28 mV; mean ventricular pacing threshold: 0.53V; mean R wave amplitude: 8.75 mV) were satisfactory. It is concluded that implantation of a 4-Fr lumenless pacing lead by using a streerable catheter-sheath to achieve inter-atrial septum or right ventricular outflow tract pacing is safe and feasible.

Efeitos da associação da estimulação atrial dinâmica em duplo sítio atrial com atenolol na prevenção da fibrilação atrial recorrente / Effects of the association of dual-site dynamic atrial overdrive and atenolol in preventing recurrent atrial fibrillation

OBJETIVO: Avaliar os efeitos da estimulação atrial otimizada (EAO) (estimulação duplo-sítio atrial, freqüência acima da intrínseca e algoritmo funcional específico) e uso de atenolol, na prevenção da fibrilação atrial (FA) recorrente. Desfecho primário: quantificar a taxa de episódios de FA. Desfechos secundários: qualidade de vida, avaliação de sintomas específicos cardiovasculares, taxa de internações hospitalares, taxa de cardioversões elétricas e farmacológicas e eventos cardíacos adversos. MÉTODOS: Vinte e sete pacientes com FA paroxística recorrente e doença do nó sinusal foram submetidos ao implante de marcapasso duplo-sítio atrial e ventricular e iniciaram com atenolol 100 mg/dia, a seguir foram randomizados em dois grupos, grupo I (3 meses iniciais com EAO e algoritmo especifico ligado e mais 3 com o mesmo desligado) e grupo II (seqüência inversa do grupo I). O modo de estimulação foi DDDR e após 3 meses, foram submetidos à avaliação clínica e eletrônica do sistema de estimulação - mudança automática de modo (AMS), Holter de 24 horas, ecocardiograma e questionário SF-36. Em seguida, foram cruzados e após 6 meses, nova avaliação. RESULTADOS: Pacientes com EAO, quando comparados ao grupo com algoritmo desligado, apresentaram menores taxas de: FA/semana (p<0,001), ativações do AMS (p<0,01), hospitalizações (p<0,001), cardioversões (p<0,001), além de melhores índices dos componentes físico e mental da qualidade de vida. CONCLUSÃO: A terapêutica híbrida, EAO associada ao uso de atenolol, reduziu a taxa de recorrência de FA e proporcionou melhora clínico funcional de pacientes com bradiarritmias sintomáticas.

OBJECTIVE: Evaluate the effects of optimized atrial stimulation - OAS (dual-site atrial pacing, heart rate above the intrinsic rate, and specific functional algorithm), and the use of atenolol in preventing recurrent atrial fibrillation (AF). Primary endpoint: to quantify the rate of AF episodes. Secondary endpoints: assessment of quality of life, specific cardiovascular symptoms, rate of hospital admissions, rate of electrical and pharmacological cardioversions, and adverse cardiac events. METHODS: Twenty-five patients with recurrent episodes of paroxysmal AF and sinus node disease had dual-site atrial and ventricular pacemakers implanted, and were started on atenolol, 100 mg/day. Next, they were randomized to two groups: GROUP I: first three months with OAS and the specific pacing algorithm (DAO) turned on, and three more months with the algorithm off. GROUP II: the inverse sequence to GROUP I. The pacing mode chosen was DDDR, and after three months patients underwent clinical and electronic evaluations of the stimulation system by: automatic mode switch (AMS), 24-hour Holter monitoring, Doppler echocardiogram, and SF-36 questionnaire. Following, a crossover comparison took place, and a new assessment was performed six months later. RESULTS: When compared to the group with the algorithm turned off, OAS patients had lower rates of: AF/week (p < 0.001); AMS activations (p < 0.01); hospitalizations (p < 0.001); cardioversions (p < 0.001), and higher scores on the physical and mental components of quality of life. CONCLUSION: The hybrid therapy adopted, OAS associated with the use of atenolol, reduced the rate of recurrent AF and improved the clinical-functional status of patients with symptomatic bradyarrhythmias.

Implante de marcapasos a través de la vena cava superior izquierda persistente. Reporte de un caso / Pacemaker lead implant via the persistent left superior vena cava

Antecedentes: La persistencia de la vena cava superior izquierda (VCSIP) es una anomalía estructural infrecuente y asintomática, presente en el 0.5% de la población general. Habitualmente se descubre de forma incidental, y es durante el implante de marcapasos cuando adquiere relevancia anatómica. La vía de abordaje venoso para el implante de marcapasos definitivos más utilizada en la actualidad es a través de la subclavia izquierda; la VCSIP dificulta, aunque no imposibilita el implante del o los electrodos para la estimulación eléctrica auricular o ventricular. El presente reporte está enfocado como referencia práctica para el diagnóstico y consideraciones técnicas durante el implante. Presentación del caso: Mujer de 26 años de edad en quien se documentó enfermedad intrínseca del nodo sinusal sintomática, variedad paros sinusales. Durante el abordaje venoso, la clave diagnóstica que hizo sospechar la anomalía fue la trayectoria medial del electrodo, corroborándose por venografía la desembocadura de la VCSIP en el seno coronario y su drenaje al atrio derecho. Se avanzó sin dificultad un electrodo de fijación activa implantándolo en la pared libre anterosuperior de la aurícula derecha. Tras 24 meses de seguimiento no se han presentado complicaciones y la paciente cursa asintomática. Discusión: La VCSIP es una anomalia congénita rara. Además de relacionarse con otras malformaciones congénitas, podría tener implicaciones desde el punto de vista de su desarrollo morfológico en los trastornos de la formación y conducción del impulso cardíaco. La falta de regresión de la vena cardinal izquierda puede influir en gran medida en el desarrollo ontogénico del nodo sinusal, el nodo auriculoventricular y el haz de His, relacionándose por lo tanto con diversos trastornos del ritmo. En casos de difícil manipulación del electrodo a través del seno coronario, se recomienda el abordaje venoso derecho después de visualizar la vena cava por venografía o ecocardiografía pues su ausencia o hipolasia (defecto que se reporta hasta en el 10% de los casos) representa un obstáculo aún mayor y que debiera sugerir el implante epicárdico. Conclusión: Ahora que la mayoría de implantes de marcapasos definitivos se abordan vía vena subclavia izquierda, el operador debe conocer esta anomalía venosa, que puede plantear dificultades técnicas en el implante de los electrodos. El conocimiento de esta anomalía puede ser de utilidad para otras especialidades que requieran el implante de catéteres vasculares permanentes a través de la vena subclavia izquierda.

Background: Persistent left superiorvena cava (PLSVC) is a structural, asymptomatic and infrequent anomaly, present in 0.5% of the general population. Typically the diagnosis reveals itself unexpectedly at the time of pacemaker implantation, when it acquires anatomic relevancy. Several techniques are used forthetransvenous approach to enter the central venous circulation; the left subclavian vein has become a common access site for electrode implantation and, occasionally, one can find an anomalous venous structure such as a PLSVC. Placement of electrodes through this anomalous venous structure can prove challenging, if not impossible. The present report tries to explore aspects of transimplantation diagnosis from a practical point of view. It also address the knowledge of anatomy, implant technique and radiographic orientation. Case presentation: Twenty-six-year-old woman with confirmed Symptomatic Sick Sinus Syndrome variety Sinus Arrest. The diagnosis of PLSVC was discovered unexpectedly at the time of the transvenous approach. The tip for the diagnosis was the "unusually medial" position of the lead, and the venogram showed the venous traject towards the coronary sinus and drainage into the right atrium. An active-fixation screw-in electrode was positioned in the antero-superior margin of the free wall of the right atrium. After 24 months of successful placement of the pacemaker, the patient is asymptomatic. Discussion: PLSVC is a rare congenital vascular abnormality. Besides its association with congenital anomalies, its most relevant clinical implication is the association with disturbances of cardiac rhythm, impulse formation and conduction. The ontogenetic development of the sinus node, the atrioventricular node, and the His bundle might be heavily influenced by the lack of regression of the left cardinal vein. When isolated, the PLSVC is usually not recognized until left superior approach to the heart is required, when it becomes ...

Estudo da função ventricular na técnica de plicatura da parede livre do ventrículo esquerdo em cães / Left ventricular function after plication of the left ventricular free wall in dogs

OBJETIVO: Avaliar os efeitos da técnica na função ventricular esquerda em cães hígidos e com cardiomiopatia dilatada induzida pela doxorrubicina. MÉTODO: De 13 cães, oito receberam doxorrubicina até que a fração de encurtamento (FE) fosse menor que 20 por cento. Destes, quatro animais e os cinco não induzidos foram submetidos à plicatura da parede livre do ventrículo esquerdo (PPLVE). Os demais cães não foram operados. Foram avaliados débito cardíaco (DC), pressão arterial, exame físico, eletrocardiografia, sistema "Holter" e ecocardiografia, por 180 dias. RESULTADOS: Houve redução do volume ventricular esquerdo. Os cães induzidos melhoraram após a operação e a fração de ejeção (FEj) retornou aos valores normais para a espécie. O DC e a FE aumentaram após a operação. Um cão foi a óbito. Nos cães não operados, a FE diminuiu e foram a óbito em torno de 40 dias após a indução; nos cães não induzidos, esta não se alterou. Houve extra-sístoles ventriculares, que se resolveram espontaneamente. CONCLUSÕES: A PPLVE sem circulação extracorpórea reduz o volume ventricular esquerdo e melhora a função cardíaca dos cães com cardiomiopatia dilatada induzida pela doxorrubicina, demonstrando baixa morbidade e mortalidade tardia.

Electrical Injury As A Possible Cause of Sick Sinus Syndrome

Electrical injury is a serious public health problem. Heart is one of the most frequently affected organs. Electrical injury can cause life-threatening cardiac complications such as asystole, ventricular fibrillation, and myocardial rupture. In this case report, we present a 20-yr-old male patient with sick sinus syndrome that developed years after electrical injury.

Acquired atrioventricular block in a patient with sinus node disease on single-chamber atrial pacing.

Single-chamber atrial pacing is the most physiological and yet economical modality of treatment in patients with symptomatic sinus node disease with normal atrioventricular conduction. However, because of the possibility of future development of a high-degree atrioventricular block and atrial fibrillation, most patients are implanted either dual- or single-chamber right ventricular pacemakers. We report a patient with symptomatic sinus node disease on single-chamber atrial pacing for the past 7 years who developed a progressive increase in the PR interval and ultimately presented with symptomatic high-degree atrioventricular block requiring pacemaker upgradation. The pacemaker was upgraded to the single-chamber ventricular mode with one additional ventricular lead introduced from the same side percutaneously.

Marcapasos bicamerales: resultados inmediatos y alejados / Short and long terms results of dual chamber pacemaker use

Background: Continuous improvement of dual chamber DDD pacemakers, electrode stability and programmed sequential stimulation changed the prognosis of patients implanted with these devices. Aim: To report our experience with the use of dual chamber pacemakers. Material and methods: One hundred seventy six patients (116 male), aged 13 to 91 years old, who received a dual chamber pacemaker implant, are reported. Patients were followed for a mean of 2.6 years. Results: Indications for DDD pacemaker were complete atrioventricular block in 43 percent, sick sinus syndrome in 32 percent, paroxysmal A-V block in 24 percent. All pacemakers were Siemens-Pacesetter and were provided with an automatic sensing and threshold device. J shaped atrium electrodes were used in 78 percent of patients and screw-in electrodes in 22 percent. Post operative complications were displacement of atrial electrode in 8 patients, of ventricular electrode in 6 patients, infection in two patients and a hematoma in one. Chronic parameters, measured after six months, were within expected ranges and allowed a good reprogramming of pacemakers. Long term programming aimed to reduce battery depletion, enhance device performance and improve hemodynamic conditions. Normal sequential stimulation was achieved in 154 patients (87 percent), 14 (85) patients died of cardiovascular disease not related to pacemaker function. Eight patients were in atrial fibrillation and were reprogrammed to VVI and DDI modes. Conclusions: DDD pacemakers are reliable and afford symptomatic relief in a broad spectrum of patients

Bradiarritmias sinusais / Sinus bradyarrhythmias

As bradiarritmias sinusais representam atualmente uma das maiores indicaçöes de marcapasso. São arritmias geradas por alteraçöes no nódulo sinusal, podendo localizar-se na célula P ou na junção sinoatrial, associadas ou não a lesão do átrio. Podem ser secundárias a doença estrutural do coração ou devido à ação de drogas. Estão incluídas nas bradiarritmias sinusais a doença do nódulo sinusal, o bloqueio sinoatrial, a síndrome taqui-bradi e a hipersensibilidade do seio carotídeo. A incidência é maior entre a sexta e a sétima décadas, notando-se espectro amplo de sintomas que variam de astenia e fraqueza até síncope. Vários exames subsidiários são importantes para o diagnóstico, destacando-se o eletrocardiograma comum ou de alta resolução, o Holter de 24 horas, e o estudo eletrofisiológico transesofágico ou invasivo. O tratamento geralmente é realizado com marcapasso, exceto nas formas agudas, em que a retirada da droga possa reverter os sintomas. Dá-se preferência ao marcapasso AAI por ser mais fisiológico, seguido pelo marcapasso DDD, visto que o modo de estimulação VVI pode raramente, ser seguido pela síndrome do marcapasso.

Cardiopulmonary and echocardiographic evaluation of AAIR and DDR pacing in patients with impaired sinus node chronotropy

This study compared the results of AAIR and DDR pacing modes, using echocardiography and cardiopulmonary exercise testing. Fourteen patients underwent an echocardiographic evaluation of cardiac output (CO) during AAI and DDD pacing at rates of 60 and 100 bpm. Subsequently, the oxygen consumption at he anaerobic threshold was measured during exercise testing under AAIR mode and DDR mode with and AV delay of 120 ms. At rest and at low heart rates AAI pacing appears to maintain a higher CO than does D pacing. However, at higher heart rates there is no significant difference between the two pacing modes. On exertion, AAIR pacing does not appear to offer any advantage over DDR pacing.

Estimulaçäo atrial (AAI-AAIR) em pacientes chagásicos / Atrial pacing (AAI-AAIR) in Chagas disease patients

Doze (12 pacientes chagásicos com estimulaçäo atrial, sendo 05 do sexo masculino e a idade média de 48,5 anos, estäo sendo acompanhados desde novembro de 1981. A indicaçäo para implante de marcapasso foi de 100 por cento devido a doença do nó sinusal. Na evoluçäo, estes pacientes apresentaram perda da sensibilidade atrial (3/13) e bloqueio atrioventricular (3/12) que demandaram a mudança do modo de estimulaçäo

Long term performance of silicone insulated and polyurethane insulated cardiac pacing leads.

The long term performance of various pacing leads in use for at least one year is reported from one centre. Between January 1975 and December 1990, 1056 cardiac pacing leads were implanted in 881 patients (mean age 57 +/- 13 years). Eight hundred and three leads were silicone insulated (SI) and 253 leads were polyurethane insulated (PUI). Leads from different manufacturers were used (Medtronic, CPI, Telectronics). The average duration of follow up was 56.6 +/- 77 months (range 12-221 months) for SI and 47.1 +/- 24 months (range 12-99 months) for PUI leads. Overall lead failure occurred more often in the PUI group (n = 46, 18.2%) compared to SI group (n = 93, 11.3%, p < 0.025). Majority of lead failures occurred in the first 36 months after implantation. In particular, Medtronic models 6971, 6972 (urethane) and 6901 (silicone) showed higher rate of complications (25.5%, 26.3% and 44.5% respectively).

Sick sinus syndrome and primary systemic amyloidosis

A sixty-eight-year-old woman with documented sick sinus syndrome was found to have, five years later, florid clinical features of systemic amyloidosis which only then led to recognition of an underlying IgA, Kappa paraproteinaemia. The literature on this association is outlined. Underlying plasma cell dyscrasias should be excluded in cases of unexplained sick sinus syndrome.

Sinus node dysfunction: the role of hypervagotomia and its treatment

Dos pacientes con historial de mareos y síncope fueron evaluados en nuestra institución. Estudios electrofisiológicos demostraron valores normales para la función de los nódulos sinusal y atrio ventricular. Grabaciones del Holter de 24 horas, electroencefalograma, tolerancia de glucosa fueron normales. El diagnóstico de disfunción sinusal por hypervagotonia fue hecho y tratado con marcapasos fisiológicos. Ambos han permanecido sin síntomas