ECG warning system is established for real time monitoring of a patient's electrocardiogram (ECG) and automatic detection of certainement cardiac diseases, namely myocardial infarction, hyperkalemia and atrioventricular block. A distinctive research work related on the assimilation of modern technologies: software, computer and information technologies. ECG early warning system's algorithm is developed in accordance to measuring the average of the ECG signatures and Interprets the data with simulated healthy curve. The prototype system INITIALLY classifies the data and Evaluated it with natural healthy simulated curve. Meanwhile the system discards f healthy curve exists otherwise the system stores the distinguished abnormalities in the curve then transfers Warning to the doctor and patient. Cardiac patients can be assisted by this warning system by detecting abnormalities at the very early stage. Consequently, doctors Provide better tools to identify cardiac diseases resulting things more accurate medical advice

Bradyarrhythmias are common clinical findings consisting of physiologic and pathologic conditions (sinus node dysfunction and atrioventricular [AV] conduction disturbances). Bradyarrhythmias can be benign, requiring no treatment; however, acute unstable bradycardia can lead to cardiac arrest. In patients with confirmed or suspected bradycardia, a thorough history and physical examination should include possible causes of sinoatrial node dysfunction or AV block. Management of bradycardia is based on the severity of symptoms, the underlying causes, presence of potentially reversible causes, presence of adverse signs, and risk of progression to asystole. Pharmacologic therapy and/or pacing are used to manage unstable or symptomatic bradyarrhythmias.

INTRODUÇÃO: O intervalo PR longo, em conjunto com a duração e a morfologia do QRS gerado pela estimulação cardíaca artificial, associam-se com dessincronia e disfunção cardíaca em diferentes níveis. Na doença do nó sinusal, durante a programação do marca-passo, podemos optar por duas estratégias: PR longo com QRS estreito (quando se busca evitar a ativação ventricular em detrimento do sincronismo atrioventricular) ou PR otimizado e QRS largo estimulado pelo marca-passo (quando se busca corrigir o intervalo atrioventricular em detrimento da sincronia ventricular). Neste estudo, buscamos comparar a evolução clínica e estrutural cardíaca destas estratégias. MÉTODOS: Acompanhou-se por 1 ano uma coorte com doença do nó sinusal, bloqueio atrioventricular de 1º grau (doença binodal) e marca-passo DDD. Através da ecocardiografia Doppler de fluxo transmitral avaliou-se a duração do enchimento diastólico ventricular e sincronia atrioventricular (? ondas E+A>=40% do ciclo cardíaco). Os pacientes dessincrônicos (DAV) tiveram o intervalo atrioventricular otimizado (intervenção) ao melhor rendimento hemodinâmico, porém com QRS estimulado. Estes retornavam ao PR basal após 6 meses (cross-over). Os sincrônicos (SAV) foram mantidos sob PR longo e QRS intrínseco durante todo o seguimento (controles). RESULTADOS: Quarenta e três pacientes foram incluídos e 41 completaram o estudo (idade média= 71,5 anos). Confirmou-se a existência dos 2 grupos (p= <0.001): os SAV (n=19), distintos daqueles com dessincronia atrioventricular (DAV, n=24). PR>=263ms mostrou especificidade de 78,9% para diagnóstico de dessincronia AV, e a maior duração do PR mostrou relação direta com pior função sistólica ventricular basal. Os DAV eram predominantemente homens, tinham PR mais longos (média= 283,5ms) e menor duração da diástole (p= 0.032). Um subgrupo dos DAV com PR >300ms mostrou pior qualidade de vida, maior duração do QRS quando estimulado, e dessincronia não corrigível por otimização. De modo notável, em 6 meses, o grupo DAV mostrou tendência a melhora da FEVE apesar do QRS alargado, e decréscimo ao retornar ao basal. O grupo SAV apresentava PR longo antifisiológico e evolutivamente piora da regurgitação mitral (p= 0.008) e também registros de fibrilação atrial de aparição mais precoce. Foram preditores independentes de dessincronia atrioventricular o PR >263ms (RR= 1,84; p= 0.024) e a duração da diástole inferior a 40% do ciclo cardíaco (RR= 0,99; p<0.001). CONCLUSÕES: Em pacientes com doença binodal e marca-passo DDD, a mera intenção de evitar o QRS largo da estimulação ventricular artificial não resolve todos os problemas. Intervalos PR longos (>263ms) associados ao decréscimo do enchimento diastólico ventricular caracterizariam outro prejuízo eletromecânico cardíaco: a dessincronia atrioventricular, disfunção que tem repercussão hemodinâmica, clínica e estrutural. / BACKGROUND: Long PR interval and wide QRS duration duo to ectopic morphology generated by artificial cardiac pacing are associated with cardiac dysfunction and dyssynchrony at different levels. When programming a permanent pacemaker in sinus node disease, two strategies can be considered: long PR with narrow QRS (avoiding ventricular pacing despite the risk of losing atrioventricular syncrony) or optimized PR interval with pacemaker-induced wide QRS (aiming to correct the atrioventricular delay despite loss of the ventricular synchrony). In this study, we aimed to compare the clinical and cardiac structural outcomes of these two strategies. METHODS: Sudy a cohort of patients with sinus node disease, first-degree AV block (binodal disease) and DDD pacemaker was followed for 1 year follow-up. atrioventrucular synchrony (AVS) was assessed echocardiographically by the ventricular diastolic filling time on Doppler transmitral flow: sum of the duration of E and A waves >=40% of the cardiac cycle. Patients with AV dyssynchrony (AVD) had the AV delay optimized (intervention group) for the best hemodynamic performance, but under wide artificially paced QRS. These returned to baseline PR interval after 6 months (cross-over). Those with AVS were kept under intrinsic QRS throughout the follow-up period despiste long PR interval (control group). RESULTS: Forty-three patients were included (mean age = 71.5 years), and 41 completed the 1-year follow-up. The existence of the 2 groups was confirmed (p<=0.001): patients with AVS (n=19), differed from those with AVD (n=24). Within a homogeneous sample (mean age= 71.5 years), PR >=263 ms had a specificity of 78.9% for the diagnosis of AVD, and longer PR intervals were associated with worse baseline ventricular systolic function. Most patients with AVD were men, had longer PR intervals (mean= 283.5 ms), and had significantly lower diastole duration (p= 0.032). A subgroup of AVD patients with PR >300 ms had poorer quality of life, significantly greater use of ?-blockers (p= 0.011), longer paced QRS width, and AV dyssynchrony that is non-correctable by optimization. Notably, at 6 months, the AVD group lean towards to have better LVEF values despite the wide QRS and a decrease when returning to baseline. Patients with AVS also had PR intervals different from the physiological condition (157.9 to 330 ms) and, over time, had worsening of mitral regurgitation (p=0.008) and earlier atrial fibrillation. PR>263 ms (RR= 1.84; p= 0.024) and diastole duration <40% of the cardiac cycle (RR= 0.99; p<0.001) were independent predictors of AVD. CONCLUSIONS: For patients with binodal disease and DDD pacemaker, the strategy of avoiding the wide QRS, usually applied by modern algorithms for minimizing ventricular pacing, is not enough to solve all problems. Long PR intervals (>=263 ms) may be associated with decreased ventricular diastolic filling time and characterize another cardiac electromechanical impairment: AV dyssynchrony, with hemodynamic, clinical and structural repercussions by itself.

INTRODUÇÃO: O intervalo PR longo, em conjunto com a duração e a morfologia do QRS gerado pela estimulação cardíaca artificial, associam-se com dessincronia e disfunção cardíaca em diferentes níveis. Na doença do nó sinusal, durante a programação do marca-passo, podemos optar por duas estratégias: PR longo com QRS estreito (quando se busca evitar a ativação ventricular em detrimento do sincronismo atrioventricular) ou PR otimizado e QRS largo estimulado pelo marca-passo (quando se busca corrigir o intervalo atrioventricular em detrimento da sincronia ventricular). Neste estudo, buscamos comparar a evolução clínica e estrutural cardíaca destas estratégias. MÉTODOS: Acompanhou-se por 1 ano uma coorte com doença do nó sinusal, bloqueio atrioventricular de 1º grau (doença binodal) e marca-passo DDD. Através da ecocardiografia Doppler de fluxo transmitral avaliou-se a duração do enchimento diastólico ventricular e sincronia atrioventricular (? ondas E+A>=40% do ciclo cardíaco). Os pacientes dessincrônicos (DAV) tiveram o intervalo atrioventricular otimizado (intervenção) ao melhor rendimento hemodinâmico, porém com QRS estimulado. Estes retornavam ao PR basal após 6 meses (cross-over). Os sincrônicos (SAV) foram mantidos sob PR longo e QRS intrínseco durante todo o seguimento (controles). RESULTADOS: Quarenta e três pacientes foram incluídos e 41 completaram o estudo (idade média= 71,5 anos). Confirmou-se a existência dos 2 grupos (p= <0.001): os SAV (n=19), distintos daqueles com dessincronia atrioventricular (DAV, n=24). PR>=263ms mostrou especificidade de 78,9% para diagnóstico de dessincronia AV, e a maior duração do PR mostrou relação direta com pior função sistólica ventricular basal. Os DAV eram predominantemente homens, tinham PR mais longos (média= 283,5ms) e menor duração da diástole (p= 0.032). Um subgrupo dos DAV com PR >300ms mostrou pior qualidade de vida, maior duração do QRS quando estimulado, e dessincronia não corrigível por otimização. De modo notável, em 6 meses, o grupo DAV mostrou tendência a melhora da FEVE apesar do QRS alargado, e decréscimo ao retornar ao basal. O grupo SAV apresentava PR longo antifisiológico e evolutivamente piora da regurgitação mitral (p= 0.008) e também registros de fibrilação atrial de aparição mais precoce. Foram preditores independentes de dessincronia atrioventricular o PR >263ms (RR= 1,84; p= 0.024) e a duração da diástole inferior a 40% do ciclo cardíaco (RR= 0,99; p<0.001). CONCLUSÕES: Em pacientes com doença binodal e marca-passo DDD, a mera intenção de evitar o QRS largo da estimulação ventricular artificial não resolve todos os problemas. Intervalos PR longos (>263ms) associados ao decréscimo do enchimento diastólico ventricular caracterizariam outro prejuízo eletromecânico cardíaco: a dessincronia atrioventricular, disfunção que tem repercussão hemodinâmica, clínica e estrutural. / BACKGROUND: Long PR interval and wide QRS duration duo to ectopic morphology generated by artificial cardiac pacing are associated with cardiac dysfunction and dyssynchrony at different levels. When programming a permanent pacemaker in sinus node disease, two strategies can be considered: long PR with narrow QRS (avoiding ventricular pacing despite the risk of losing atrioventricular syncrony) or optimized PR interval with pacemaker-induced wide QRS (aiming to correct the atrioventricular delay despite loss of the ventricular synchrony). In this study, we aimed to compare the clinical and cardiac structural outcomes of these two strategies. METHODS: Sudy a cohort of patients with sinus node disease, first-degree AV block (binodal disease) and DDD pacemaker was followed for 1 year follow-up. atrioventrucular synchrony (AVS) was assessed echocardiographically by the ventricular diastolic filling time on Doppler transmitral flow: sum of the duration of E and A waves >=40% of the cardiac cycle. Patients with AV dyssynchrony (AVD) had the AV delay optimized (intervention group) for the best hemodynamic performance, but under wide artificially paced QRS. These returned to baseline PR interval after 6 months (cross-over). Those with AVS were kept under intrinsic QRS throughout the follow-up period despiste long PR interval (control group). RESULTS: Forty-three patients were included (mean age = 71.5 years), and 41 completed the 1-year follow-up. The existence of the 2 groups was confirmed (p<=0.001): patients with AVS (n=19), differed from those with AVD (n=24). Within a homogeneous sample (mean age= 71.5 years), PR >=263 ms had a specificity of 78.9% for the diagnosis of AVD, and longer PR intervals were associated with worse baseline ventricular systolic function. Most patients with AVD were men, had longer PR intervals (mean= 283.5 ms), and had significantly lower diastole duration (p= 0.032). A subgroup of AVD patients with PR >300 ms had poorer quality of life, significantly greater use of ?-blockers (p= 0.011), longer paced QRS width, and AV dyssynchrony that is non-correctable by optimization. Notably, at 6 months, the AVD group lean towards to have better LVEF values despite the wide QRS and a decrease when returning to baseline. Patients with AVS also had PR intervals different from the physiological condition (157.9 to 330 ms) and, over time, had worsening of mitral regurgitation (p=0.008) and earlier atrial fibrillation. PR>263 ms (RR= 1.84; p= 0.024) and diastole duration <40% of the cardiac cycle (RR= 0.99; p<0.001) were independent predictors of AVD. CONCLUSIONS: For patients with binodal disease and DDD pacemaker, the strategy of avoiding the wide QRS, usually applied by modern algorithms for minimizing ventricular pacing, is not enough to solve all problems. Long PR intervals (>=263 ms) may be associated with decreased ventricular diastolic filling time and characterize another cardiac electromechanical impairment: AV dyssynchrony, with hemodynamic, clinical and structural repercussions by itself.

L'évaluation de l'asynchronisme mécanique ventriculaire sous stress a soulevé une attention importante en tant que facteur prédictif de la réponse au traitement de resynchronisation cardiaque (CRT). De plus, il semble exister une relation significative entre le devenir du patient et la présence d’asynchronisme au repos. Plusieurs méthodes échocardiographiques peuvent être utilisées pour évaluer l’asynchronisme. Cependant, parmi toutes les différentes méthodologies ou index existant dans ce domaine, aucun critère ne fait l’unanimité. Cette thèse étudie l'importance des techniques d'imagerie nucléaire dans le cadre de l’évaluation de l’asynchronisme cardiaque induit par le stress en utilisant trois différents modèles canins expérimentaux. Le premier chapitre vise à examiner les effets du stress sur le synchronisme de la contraction du ventricule gauche (VG) en utilisant l'imagerie synchronisée de perfusion myocardique dans une cohorte canine normale. Le stress a été induit par différents niveaux d’infusion de dobutamine sur six sujets sains. Les paramètres hémodynamiques et l’asynchronisme ont été évalués par des mesures de pressions ventriculaires. L'analyse de phase sur l’imagerie s’est effectuée en utilisant un logiciel commercialement disponible (QGS) et un logiciel interne (MHI4MPI), basée sur le déplacement et l’épaississement des parois ventriculaires. L’augmentation de la concentration de dobutamine a démontré une amélioration de la capacité fonctionnelle et une réduction de l’asynchronisme ventriculaire. L’analyse de l’asynchronisme calculée à partir de l’épaississement de la paroi semble plus robuste et plus sensible que l’utilisation du déplacement des parois. (Salimian et. al., J Nucl Cardiol., 2014) Le second chapitre étudie les différents paramètres d’asynchronisme au repos et à différents niveaux de stress dans un modèle de cardiomyopathie dilatée et à QRS étroit. Ce modèle a été créé sur dix chiens par tachycardie via stimulation de l'apex du ventricule droit pendant 3-4 semaines, permettant d’atteindre une fraction d'éjection cible de 35% ou moins. Le stress a ensuite été induit par une perfusion de dobutamine jusqu'à un maximum de 20 μg/kg/min. Les données hémodynamiques et l’asynchronisme ont été analysés par des mesures de pression ventriculaire et l’analyse de l’imagerie dynamique du compartiment sanguin. L’importante variabilité individuelle des sujets inclus dans notre cohorte empêche toute conclusion définitive sur la mesure de l’asynchronisme interventriculaire. Cependant, les différents niveaux de stress, même dans des intervalles rapprochés, ont démontré un effet significatif sur les paramètres hémodynamiques et l’asynchronisme. (Salimian et. al., J Nucl Cardiol., 2015) La troisième section vise à déterminer si l’estimation du mode de stimulation optimal effectuée au repos demeure le choix optimal lorsque le niveau d’activité cardiaque s’intensifie pour des sujets avec bloc auriculo-ventriculaire (AV) et fonction ventriculaire normale. Cinq chiens ont été soumis à une ablation du nœud AV et des sondes de stimulation ont été insérées dans l'oreillette droite pour la détection, l’apex du ventricule droit (VD) et une veine postérolatérale du VG pour la stimulation. Cinq modes de stimulation ont été utilisés : LV pur, biventriculaire (BiV) avec pré-activation de 20 ms du LV (LVRV20), BiV pur, BiV avec pré-activation de 20 ms du VD (RVLV20), VD pur. Des niveaux jusqu’à 20 μg/kg/min de dobutamine ont été atteints. Le stress a modifié l’étendue de l’asynchronisme de base et ce, pour tous les modes de stimulation. De plus, les effets physiologiques intrinsèques du stress permettent une évaluation plus précise de l’asynchronisme ventriculaire, diminuant la variabilité inter-sujet. Le mode de stimulation LVRV20 semble le mode optimal dans ce modèle, supportant l’utilisation de la stimulation bi-ventriculaire. / Assessment of ventricular mechanical dyssynchrony (MD) under stress has attracted a large amount of attention as a stronger predictor of response to cardiac resynchronization therapy (CRT) and as a parameter whose variation bears a greater relationship to clinical outcomes than resting-MD either in CRT candidates or another subset of patients. Several echocardiographic methods can be used to assess stress-MD. However, no standardized approach is currently used to explore stress-induced variations in inter- and intraventricular MD. This dissertation studies the importance of nuclear imaging techniques in assessing stress-induced MD variations by providing three different experimental canine models. The first chapter sought to examine the impacts of stress on the left ventricular (LV) synchrony with phase analysis of gated SPECT myocardial perfusion imaging (GMPS) within a normal canine cohort. Stress was induced by different levels of dobutamine infusion in six healthy subjects. Hemodynamic and LV MD parameters were assessed by LV pressure measurements and phase analysis of GMPS using commercially available QGS software and in-house MHI4MPI software with thickening- and displacement-based methodology. The increase of dobutamine level was shown to be in accordance with the improvement of LV functional capacity and reduction of MD parameters. MD analysis based on wall thickening was more robust and sensitive than the global wall displacement. (Salimian et. al., J Nucl Cardiol., 2014) The second chapter investigated the range of difference in inter- and intraventricular MD parameters from rest to various levels of stress in a dilated cardiomyopathy (DCM) and narrow QRS complex model. Ten large dogs were submitted to tachycardia-induced DCM by pacing the right ventricular apex for 3-4 weeks to reach a target ejection fraction of 35% or less. Stress was then induced by infusion of dobutamine up to a maximum of 20 μg/kg/min. Hemodynamic and MD data were analyzed by LV pressure measurements and gated-blood pool SPECT (GBPS) imaging. Individual differences in the magnitude and pattern of change in the various levels of stress precluded any definitive conclusion about interventricular MD. However, different levels of stress, even in close intervals, showed a significant positive impact on hemodynamic and intraventricular MD parameters. (Salimian et. al., J Nucl Cardiol., 2015) The third chapter sought to examine if the optimal pacing mode at rest could be the best one during the maximum stress level in terms of MD parameters in subjects with an atrioventricular (AV) block and normal function. Five dogs were submitted to AV node ablation and pacing leads were placed in the right atrium for sensing, in right ventricular (RV) apex, and in posterolateral LV vein for pacing in five modes of LV, biventricular (BiV) with 20 ms of LV pre-activation (LVRV20), BiV, BiV with 20 ms of RV pre-activation (RVLV20) and RV pacing. Stress was induced by dobutamine infusion up to a maximum of 20 μg/kg/min. Data analyses were the same as chapter one. Dobutamine stress changed the extent of resting-LV MD at all pacing modes. Intrinsic physiologic effects of stress resulted in more accurate MD assessment with lesser variability in subjects who underwent pacing. LVRV20 was the preferred site of stimulation in this model rather than single-site pacing.

Introdução: O bloqueio atrioventricular congênito isolado (BAVCi) é raro e tem múltiplas apresentações clínicas. O implante de marca-passo cardíaco permanente (MP) é o tratamento de escolha, resultando em evolução clínica satisfatória para a maioria dos casos, porém, aproximadamente 10% deles apresentam remodelamento ventricular e insuficiência cardíaca grave. Objetivos: Estudar a evolução tardia de crianças e adultos jovens com BAVCi e estimulação crônica do ventrículo direito (VD), visando determinar: a prevalência de sinais clínicos e laboratoriais de insuficiência cardíaca e de remodelamento ventricular; a capacidade funcional; a qualidade de vida e fatores preditores de alterações clínicas, funcionais ou ecocardiográficas. Métodos: Estudo transversal realizado em coorte de portadores de BAVCi e MP implantado antes de 21 anos de idade com estimulação no VD há mais de um ano. Todos os indivíduos foram submetidos a avaliação clínica e laboratorial, da capacidade funcional, da qualidade de vida e a ecocardiograma. Mães e sujeitos da pesquisa foram investigados para doenças reumatológicas. Os dados foram armazenados no sistema REDCap (Research Electronic Data Capture) e analisados pelos programas SAS (Statistical Analysis System), SPSS (Statistical Package for the Social Sciences) e R Studio. A análise dos dados incluiu: análise univariada para pesquisa de associações entre variáveis preditoras e desfechos, coeficiente de correlação de Pearson e modelo de regressão linear multivariado. Resultados: De março/2010 a dezembro/2013, foram avaliados 63 indivíduos, 68% do sexo feminino, com idade de 1 a 40 anos, com MP por 13,4 ± 6,5 anos e estimulação do VD por 10,0 ± 5,4 anos. O modo de estimulação era atrioventricular em 55,6%, o percentual de estimulação de VD de 97,9 ± 4,2% e a duração do complexo QRS estimulado de 152,4 ± 20,1 ms. A maioria (88,9%) era assintomática e não utilizava medicamentos de ação cardiovascular. Maior tempo de MP (P= 0,013), maior tempo de estimulação do VD (P= 0,005), maior idade na inclusão no estudo (P= 0,032) ou menor fração de ejeção do ventrículo (FEVE) (P= 0,013) associaram-se com a presença de classe funcional II (NYHA) e/ou uso medicamentos. Os valores do peptídeo natriurético tipo B foram normais em todos os exames laboratoriais, mas houve alteração da proteína C reativa ultrassensível, fator de necrose tumoral alfa e interleucina-6 em 66%, 34% e 13% exames, respectivamente. A distância média percorrida no teste de caminha de seis minutos foi de 546,9 ± 76,2 metros (91,0 ± 12,5% do valor predito). Os escores médios da qualidade de vida foram 78,1 ± 17,7 para o \"Sumário Físico\" e 76,6 ± 17,1 para o \"Sumário Mental\" do questionário Short Form 36 (SF-36) e de 77,4 ± 18,5, para o \"Sumário Físico\" e de 77,7 ± 21,6 para o \"Sumário Psicossocial\", do Child Health Questionnaire - Parent Form 50 (CHQ-PF50). Diminuição da FEVE foi detectada em 39,7% e aumento do diâmetro diastólico do ventrículo esquerdo (DDVE) em 22,2% dos indivíduos. A idade mais avançada no primeiro implante de MP se correlacionou negativamente com menor FEVE (r= -0,302; P= 0,016); a duração do complexo QRS estimulado (r= 0,447; P= 0,002) e maior tempo sob estimulação do VD (r= 0,416; P= 0,007) se correlacionaram positivamente ao aumento do DDVE. Foi detectada dissincronia ventricular em 60,3% dos indivíduos. O retardo da ativação eletromecânica intraventricular esquerda foi de 86,5 ± 56,9 ms e interventricular, de 141,9 ± 88 ms. Contudo, não houve correlação com os fatores estudados. Autoanticorpos anti-Ro/SSA foram detectados em 18 (32,1%) mães, com associação entre idade no momento do implante do MP (P= 0,032) e uso de MP ventricular no momento do estudo (P= 0,022). A regressão linear multivariada confirmou a correlação entre a idade no momento do implante do MP com a FEVE (P= 0,016), da duração do complexo QRS estimulado (P= 0,004) e do tempo sob estimulação do VD (P= 0,014) com o DDVE. Conclusões: A prevalência de manifestações clínicas e laboratoriais de insuficiência cardíaca foi baixa; por outro lado, a de remodelamento ventricular esquerdo foi elevada. A capacidade funcional foi satisfatória, assim como a qualidade de vida, nos aspectos físicos e emocionais. Idade mais avançada no primeiro implante de MP, maior tempo sob estimulação cardíaca e complexo QRS estimulado mais alargado foram fatores independentes de remodelamento ventricular e/ou de manifestação de insuficiência cardíaca / Introduction: Isolated congenital atrioventricular block (iCAVB) is a rare condition with multiple clinical presentations. Permanent cardiac pacing is the most effective therapy for this population resulting in satisfactory long-term outcomes. However, approximately 10% of patients may have ventricular remodeling and severe heart failure. Objectives: To study the long-term effects of chronic right ventricular (RV) pacing in children and young adults with iCAVB in order to determine: prevalence of clinical and laboratory signs of heart failure and ventricular remodeling, functional capacity, quality of life and predictors of clinical, functional or echocardiographic abnormalities. Methods: Cross-sectional study of a cohort of iCAVB patients with <= 21 years old at initial pacemaker (PM) implantation and single or dual-chamber pacing in a unique RV site for a minimum of one year. All subjects underwent clinical and laboratory assessment, functional capacity, quality of life and echocardiogram. Mothers and research subjects were investigated for rheumatic diseases. Data were stored in REDCap (Research Electronic Data Capture) system and analyzed by SAS (Statistical Analysis System), SPSS (Statistical Package for the Social Sciences) and R Studio programs. Data analysis included: univariate analysis for associations between predictor variables and outcomes, Pearson correlation coefficient and linear regression multivariate model. Results: Between March/2010 and December/2013, we evaluated 63 subjects aged 1-40 years old, 68% female, under PM for 13.4 ± 6.5 years and under RV pacing for 10.0 ± 5.4 years. Pacing mode was atrioventricular in 55.6%, percentage of RV pacing was 97.9 ± 4.2% and paced QRS duration was 152.4 ± 20.1 ms. Overall, the majority (88.9%) were asymptomatic and did not use cardiovascular drugs. Longer time under PM (P= 0.013), or even under RV pacing (P= 0.005), higher age at study inclusion (P= 0.032) and lower left ventricular ejection fraction (LVEF) (P= 0.013) were associated with functional class II (NYHA) and / or drug use. B-natriuretic peptide values were normal in all tests. C reactive protein ultrasensitive, tumor necrosis factor alfa and interleukin-6 were increased in 66%, 34% and 13% tests, respectively. The mean walked distance in the sex minute walk test was 546.9 ± 76.2 meters (91.0 ± 12.5% of the predicted value). Mean scores of quality of life were 78.1 ± 17.7 for \"Physical Summary\" and 76.6 ± 17.1 for \"Mental Summary\" in the Short Form 36 (SF-36) and 77.4 ± 18.5 for \"Physical Summary\" and 77.7 ± 21.6 for \"Psychosocial Summary\", of Child Health Questionnaire - Parent Form 50 (CHQ-PF50). Decreased LVEF was detected in 39.7% and increased left ventricular end-diastolic diameter (LVDD) in 22.2% of subjects. Higher age at the first PM implant was negatively correlated with lower LVEF (r= -0.302; P= 0.016); paced QRS duration (r= 0.447; P= 0.002) and time under RV pacing (r= 0.416; P= 0.007) were positively correlated with LVDD. Ventricular dyssynchrony was detected in 60.3 % of individuals. Intra-left ventricular electromechanical delay was 86.5 ± 56.9 ms and interventricular was 141.9 ± 88 ms. However, ventricular dyssynchrony was not correlated with the studied variables. Autoantibodies anti-SSA/Ro were detected in 18 (32.1%) mothers. There was association between age at PM implant (P= 0.032) and use of ventricular PM at the time of the study (P= 0.022) and presence of anti-SSA/Ro. Multivariate linear regression showed significant correlation between age at PM implant with LVEF (P= 0.016); and paced QRS duration (P= 0.005) and time under RV pacing (P= 0.014) with LVDD. Conclusions: Clinical and laboratorial manifestations of heart failure presented low prevalence in this population. On the other hand, the prevalence of left ventricular remodeling was high. Functional capacity was adequate, as well as quality of life, in both physical and emotional aspects. Higher age at first PM implant, longer time under pacing and wider paced QRS duration were independent factors of ventricular remodeling and/or manifestation of heart failure

Introdução: O bloqueio atrioventricular congênito isolado (BAVCi) é raro e tem múltiplas apresentações clínicas. O implante de marca-passo cardíaco permanente (MP) é o tratamento de escolha, resultando em evolução clínica satisfatória para a maioria dos casos, porém, aproximadamente 10% deles apresentam remodelamento ventricular e insuficiência cardíaca grave. Objetivos: Estudar a evolução tardia de crianças e adultos jovens com BAVCi e estimulação crônica do ventrículo direito (VD), visando determinar: a prevalência de sinais clínicos e laboratoriais de insuficiência cardíaca e de remodelamento ventricular; a capacidade funcional; a qualidade de vida e fatores preditores de alterações clínicas, funcionais ou ecocardiográficas. Métodos: Estudo transversal realizado em coorte de portadores de BAVCi e MP implantado antes de 21 anos de idade com estimulação no VD há mais de um ano. Todos os indivíduos foram submetidos a avaliação clínica e laboratorial, da capacidade funcional, da qualidade de vida e a ecocardiograma. Mães e sujeitos da pesquisa foram investigados para doenças reumatológicas. Os dados foram armazenados no sistema REDCap (Research Electronic Data Capture) e analisados pelos programas SAS (Statistical Analysis System), SPSS (Statistical Package for the Social Sciences) e R Studio. A análise dos dados incluiu: análise univariada para pesquisa de associações entre variáveis preditoras e desfechos, coeficiente de correlação de Pearson e modelo de regressão linear multivariado. Resultados: De março/2010 a dezembro/2013, foram avaliados 63 indivíduos, 68% do sexo feminino, com idade de 1 a 40 anos, com MP por 13,4 ± 6,5 anos e estimulação do VD por 10,0 ± 5,4 anos. O modo de estimulação era atrioventricular em 55,6%, o percentual de estimulação de VD de 97,9 ± 4,2% e a duração do complexo QRS estimulado de 152,4 ± 20,1 ms. A maioria (88,9%) era assintomática e não utilizava medicamentos de ação cardiovascular. Maior tempo de MP (P= 0,013), maior tempo de estimulação do VD (P= 0,005), maior idade na inclusão no estudo (P= 0,032) ou menor fração de ejeção do ventrículo (FEVE) (P= 0,013) associaram-se com a presença de classe funcional II (NYHA) e/ou uso medicamentos. Os valores do peptídeo natriurético tipo B foram normais em todos os exames laboratoriais, mas houve alteração da proteína C reativa ultrassensível, fator de necrose tumoral alfa e interleucina-6 em 66%, 34% e 13% exames, respectivamente. A distância média percorrida no teste de caminha de seis minutos foi de 546,9 ± 76,2 metros (91,0 ± 12,5% do valor predito). Os escores médios da qualidade de vida foram 78,1 ± 17,7 para o \"Sumário Físico\" e 76,6 ± 17,1 para o \"Sumário Mental\" do questionário Short Form 36 (SF-36) e de 77,4 ± 18,5, para o \"Sumário Físico\" e de 77,7 ± 21,6 para o \"Sumário Psicossocial\", do Child Health Questionnaire - Parent Form 50 (CHQ-PF50). Diminuição da FEVE foi detectada em 39,7% e aumento do diâmetro diastólico do ventrículo esquerdo (DDVE) em 22,2% dos indivíduos. A idade mais avançada no primeiro implante de MP se correlacionou negativamente com menor FEVE (r= -0,302; P= 0,016); a duração do complexo QRS estimulado (r= 0,447; P= 0,002) e maior tempo sob estimulação do VD (r= 0,416; P= 0,007) se correlacionaram positivamente ao aumento do DDVE. Foi detectada dissincronia ventricular em 60,3% dos indivíduos. O retardo da ativação eletromecânica intraventricular esquerda foi de 86,5 ± 56,9 ms e interventricular, de 141,9 ± 88 ms. Contudo, não houve correlação com os fatores estudados. Autoanticorpos anti-Ro/SSA foram detectados em 18 (32,1%) mães, com associação entre idade no momento do implante do MP (P= 0,032) e uso de MP ventricular no momento do estudo (P= 0,022). A regressão linear multivariada confirmou a correlação entre a idade no momento do implante do MP com a FEVE (P= 0,016), da duração do complexo QRS estimulado (P= 0,004) e do tempo sob estimulação do VD (P= 0,014) com o DDVE. Conclusões: A prevalência de manifestações clínicas e laboratoriais de insuficiência cardíaca foi baixa; por outro lado, a de remodelamento ventricular esquerdo foi elevada. A capacidade funcional foi satisfatória, assim como a qualidade de vida, nos aspectos físicos e emocionais. Idade mais avançada no primeiro implante de MP, maior tempo sob estimulação cardíaca e complexo QRS estimulado mais alargado foram fatores independentes de remodelamento ventricular e/ou de manifestação de insuficiência cardíaca / Introduction: Isolated congenital atrioventricular block (iCAVB) is a rare condition with multiple clinical presentations. Permanent cardiac pacing is the most effective therapy for this population resulting in satisfactory long-term outcomes. However, approximately 10% of patients may have ventricular remodeling and severe heart failure. Objectives: To study the long-term effects of chronic right ventricular (RV) pacing in children and young adults with iCAVB in order to determine: prevalence of clinical and laboratory signs of heart failure and ventricular remodeling, functional capacity, quality of life and predictors of clinical, functional or echocardiographic abnormalities. Methods: Cross-sectional study of a cohort of iCAVB patients with <= 21 years old at initial pacemaker (PM) implantation and single or dual-chamber pacing in a unique RV site for a minimum of one year. All subjects underwent clinical and laboratory assessment, functional capacity, quality of life and echocardiogram. Mothers and research subjects were investigated for rheumatic diseases. Data were stored in REDCap (Research Electronic Data Capture) system and analyzed by SAS (Statistical Analysis System), SPSS (Statistical Package for the Social Sciences) and R Studio programs. Data analysis included: univariate analysis for associations between predictor variables and outcomes, Pearson correlation coefficient and linear regression multivariate model. Results: Between March/2010 and December/2013, we evaluated 63 subjects aged 1-40 years old, 68% female, under PM for 13.4 ± 6.5 years and under RV pacing for 10.0 ± 5.4 years. Pacing mode was atrioventricular in 55.6%, percentage of RV pacing was 97.9 ± 4.2% and paced QRS duration was 152.4 ± 20.1 ms. Overall, the majority (88.9%) were asymptomatic and did not use cardiovascular drugs. Longer time under PM (P= 0.013), or even under RV pacing (P= 0.005), higher age at study inclusion (P= 0.032) and lower left ventricular ejection fraction (LVEF) (P= 0.013) were associated with functional class II (NYHA) and / or drug use. B-natriuretic peptide values were normal in all tests. C reactive protein ultrasensitive, tumor necrosis factor alfa and interleukin-6 were increased in 66%, 34% and 13% tests, respectively. The mean walked distance in the sex minute walk test was 546.9 ± 76.2 meters (91.0 ± 12.5% of the predicted value). Mean scores of quality of life were 78.1 ± 17.7 for \"Physical Summary\" and 76.6 ± 17.1 for \"Mental Summary\" in the Short Form 36 (SF-36) and 77.4 ± 18.5 for \"Physical Summary\" and 77.7 ± 21.6 for \"Psychosocial Summary\", of Child Health Questionnaire - Parent Form 50 (CHQ-PF50). Decreased LVEF was detected in 39.7% and increased left ventricular end-diastolic diameter (LVDD) in 22.2% of subjects. Higher age at the first PM implant was negatively correlated with lower LVEF (r= -0.302; P= 0.016); paced QRS duration (r= 0.447; P= 0.002) and time under RV pacing (r= 0.416; P= 0.007) were positively correlated with LVDD. Ventricular dyssynchrony was detected in 60.3 % of individuals. Intra-left ventricular electromechanical delay was 86.5 ± 56.9 ms and interventricular was 141.9 ± 88 ms. However, ventricular dyssynchrony was not correlated with the studied variables. Autoantibodies anti-SSA/Ro were detected in 18 (32.1%) mothers. There was association between age at PM implant (P= 0.032) and use of ventricular PM at the time of the study (P= 0.022) and presence of anti-SSA/Ro. Multivariate linear regression showed significant correlation between age at PM implant with LVEF (P= 0.016); and paced QRS duration (P= 0.005) and time under RV pacing (P= 0.014) with LVDD. Conclusions: Clinical and laboratorial manifestations of heart failure presented low prevalence in this population. On the other hand, the prevalence of left ventricular remodeling was high. Functional capacity was adequate, as well as quality of life, in both physical and emotional aspects. Higher age at first PM implant, longer time under pacing and wider paced QRS duration were independent factors of ventricular remodeling and/or manifestation of heart failure