摘要
Abstract The present report describes a case of complete atrioventricular block (CAVB) diagnosed at 25 weeks of gestation in a pregnant woman with Sjögren's syndrome and positive anti-Ro/SSA antibodies. Fluorinated steroids (dexamethasone and betamethasone) and terbuline were used to increase the fetal heart rate, but the fetal heart block was not reversible, and the administration of drugs was discontinued due to maternal collateral effects. Follow-up fetal echocardiograms were performed, and the fetus evolved with pericardial effusion, presence of fibroelastosis in the right ventricle, and ventricular dysfunction. Interruption of pregnancy by cesarean section was indicated at 34 weeks of gestation, and a cardiac pacemaker was implanted in the male newborn immediately after birth. Therapy for fetuses with CAVB is controversial mainly regarding the use or not of corticosteroids; however, monitoring of the atrioventricular interval by fetal echocardiography should be performed in fetuses from pregnant women with positive autoantibodies anti-Ro/SSA and/or anti-La/SSB to prevent the progression to CAVB.
Resumo Este relato descreve um caso de bloqueio atrioventricular completo (BAVC) diagnosticado com 25 semanas de gestação em uma mulher com síndrome de Sjögren e anticorpos anti-Ro/SSA positivos. Esteroides fluoretados (dexametasona e betametasona) e terbulina foram utilizados para aumentar a frequência cardíaca fetal, mas o bloqueio cardíaco fetal não foi reversível, e a administração dos medicamentos foi interrompida devido a efeitos colaterais maternos. Ecocardiogramas fetais de acompanhamento foram realizados, e o feto evoluiu com derrame pericárdico, presença de fibroelastose no ventrículo direito, e disfunção ventricular. A interrupção da gravidez por cesariana foi indicada com 34 semanas, e um marca-passo cardíaco foi implantado no recém-nascido do sexo masculino imediatamente após o nascimento. A terapia para fetos com BAVC é controversa, principalmente no que diz respeito ao uso ou não de corticosteroides; no entanto, o monitoramento do intervalo atrioventricular pela ecocardiografia fetal deve ser feito em fetos de mulheres grávidas com autoanticorpos positivos anti-Ro/SSA e/ou anti-La/SSB para impedir a progressão para o BAVC.
Subject(s)
Humans , Female , Pregnancy , Adult , Pregnancy Complications, Cardiovascular , Sjogren's Syndrome/complications , Heart Block/congenital , Prenatal Care , Autoantibodies/blood , Heart Rate, Fetal/physiology , Ultrasonography, Prenatal摘要
Abstract: One of the most common pathologies attributed to lupus neonatal refers to atrioventricular (AV) congenital block, which diagnosis can be made between 16 and 30 weeks of gestation due to persistent fetal bradycardia. The development of this disease is mostly related to maternal anti-Ro/SSA and anti-Smith autoantibodies. Currently, there are a number of alternatives for prenatal and postnatal treatment, with some controversy about their viability. The placement of a permanent pacemaker is presented as one of the most appropriate procedures currently, even with the risks awarded. This case report describes the placement of a permanent pacemaker to a two-month-old newborn with high maternal contents of anti-Ro/SSA and anti-Smith nuclear autoantibodies, with a favorable outcome.(AU)
Resumen: Una de las patologías más comunes atribuidas al lupus neonatal se refiere al bloqueo congénito atrioventricular (AV), diagnóstico que se puede realizar entre 16 y 30 semanas de gestación debido a bradicardia fetal persistente. El desarrollo de esta enfermedad se relaciona principalmente con los anticuerpos anti-Ro/SSA materno y anti-Smith. Actualmente, existen varias alternativas para el tratamiento prenatal y postnatal, con cierta controversia sobre su viabilidad. La colocación de un marcapasos permanente se presenta como uno de los procedimientos más adecuados actualmente, incluso con los riesgos adjudicados. Este relato de caso describe la colocación de un marcapasos permanente en un recién nacido de dos meses con alto contenido materno de autoanticuerpos anti-Ro/SSA y anti-Smith, con un resultado favorable.(AU)
Subject(s)
Humans , Infant , Pacemaker, Artificial , Lupus Nephritis/congenital , Bradycardia/congenital , Heart Block/congenital摘要
Abstract Background: Few studies have characterized the surgical outcomes following epicardial pacemaker implantation in neonates with congenital complete atrioventricular block (CCAVB). Objective: This study sought to assess the long-term outcomes of a minimally invasive epicardial approach using a subxiphoid access for pacemaker implantation in neonates. Methods: Between July 2002 and February 2015, 16 consecutive neonates underwent epicardial pacemaker implantation due to CCAVB. Among these, 12 (75.0%) had congenital heart defects associated with CCAVB. The patients had a mean age of 4.7 ± 5.3 days and nine (56.3%) were female. Bipolar steroid-eluting epicardial leads were implanted in all patients through a minimally invasive subxiphoid approach and fixed on the diaphragmatic ventricular surface. The pulse generator was placed in an epigastric submuscular position. Results: All procedures were successful, with no perioperative complications or early deaths. Mean operating time was 90.2 ± 16.8 minutes. None of the patients displayed pacing or sensing dysfunction, and all parameters remained stable throughout the follow-up period of 4.1 ± 3.9 years. Three children underwent pulse generator replacement due to normal battery depletion at 4.0, 7.2, and 9.0 years of age without the need of ventricular lead replacement. There were two deaths at 12 and 325 days after pacemaker implantation due to bleeding from thrombolytic use and progressive refractory heart failure, respectively. Conclusion: Epicardial pacemaker implantation through a subxiphoid approach in neonates with CCAVB is technically feasible and associated with excellent surgical outcomes and pacing lead longevity.
Resumo Fundamento: Há poucos estudos caracterizando os desfechos cirúrgicos após implante de marca-passo em neonatos com bloqueio atrioventricular total congênito (BAVTC). Objetivos: Este estudo procurou avaliar os resultados a longo prazo de uma abordagem epicárdica minimamente invasiva com utilização de acesso subxifoide para implante de marca-passo em neonatos. Métodos: Entre julho de 2002 a fevereiro de 2015, 16 neonatos consecutivos foram submetidos a implante de marca-passo epicárdico devido a BAVTC. Entre eles, 12 (75,0%) apresentavam defeitos cardíacos congênitos associados ao BAVTC. Os pacientes tinham uma média de idade de 4,5 ± 5,3 dias e nove (56,3%) eram do sexo feminino. Cabo-eletrodo bipolar revestido com esteroide foi implantado em todos os pacientes através de uma abordagem subxifoide minimamente invasiva e fixado na superfície ventricular diafragmática. O gerador de pulsos foi alojado em uma posição epigástrica submuscular. Resultados: Todos os procedimentos foram realizados com sucesso, sem complicações intraoperatórias ou mortes. O tempo médio de duração das operações foi de 90,2 ± 16,8 minutos. Após seguimento médio de 4,1 ± 3,9 anos e máximo de 12,2 anos, não foram observadas complicações de loja do gerador de pulsos, aumento crônico do limiar de comando, fratura de cabo-eletrodo ou outros problemas relacionados ao sistema de estimulação cardíaca. Três crianças foram submetidas à troca do gerador de pulsos por depleção normal de bateria aos 4,0, 7,2 e 9,0 anos de idade, sem necessidade de troca do cabo-eletrodo ventricular. Houve duas mortes aos 12 e 325 dias após o implante do marca-passo devido a sangramento decorrente do uso de trombolítico e insuficiência cardíaca refratária progressiva, respectivamente. Conclusões: O implante de marca-passo epicárdico através de abordagem subxifoide em neonatos com BAVTC com alojamento epigástrico do gerador de pulsos é tecnicamente viável e associado a excelentes desfechos cirúrgicos e longevidade do cabo-eletrodo de estimulação.
Subject(s)
Humans , Male , Female , Infant, Newborn , Pacemaker, Artificial , Cardiac Pacing, Artificial/methods , Heart Block/congenital , Postoperative Complications , Radiography, Thoracic , Reproducibility of Results , Follow-Up Studies , Treatment Outcome , Minimally Invasive Surgical Procedures/methods , Electrodes, Implanted , Equipment Design , Operative Time , Heart Block/surgery , Intraoperative Complications , Medical Illustration摘要
Background: Isolated congenital atrioventricular block (CAVB) is a rare condition with multiple clinical outcomes. Ventricular remodeling can occur in approximately 10% of the patients after pacemaker (PM) implantation. Objectives: To assess the functional capacity of children and young adults with isolated CAVB and chronic pacing of the right ventricle (RV) and evaluate its correlation with predictors of ventricular remodeling. Methods: This cross-sectional study used a cohort of patients with isolated CAVB and RV pacing for over a year. The subjects underwent clinical and echocardiographic evaluation. Functional capacity was assessed using the six-minute walk test. Chi-square test, Fisher's exact test, and Pearson correlation coefficient were used, considering a significance level of 5%. Results: A total of 61 individuals were evaluated between March 2010 and December 2013, of which 67.2% were women, aged between 7 and 41 years, who were using PMs for 13.5 ± 6.3 years. The percentage of ventricular pacing was 97.9 ± 4.1%, and the duration of the paced QRS complex was 153.7 ± 19.1 ms. Majority of the subjects (95.1%) were asymptomatic and did not use any medication. The mean distance walked was 546.9 ± 76.2 meters and was strongly correlated with the predicted distance (r = 0.907, p = 0.001) but not with risk factors for ventricular remodeling. (Arq Bras Cardiol. 2014; [online].ahead print, PP.0-0) Conclusions: The functional capacity of isolated CAVB patients with chronic RV pacing was satisfactory but did not correlate with risk factors for ventricular remodeling. .
Fundamento: O bloqueio atrioventricular congênito isolado (BAVCi) é raro e tem múltiplas apresentações clínicas. O remodelamento ventricular pode ocorrer em cerca de 10% dos indivíduos após o implante de marca-passo. Objetivos: Avaliar a capacidade funcional de crianças e adultos jovens com BAVCi e estimulação crônica no ventrículo direito (VD) e pesquisar sua associação com fatores preditores de remodelamento ventricular. Métodos: Estudo transversal em coorte de indivíduos com BAVCi e estimulação no VD há mais de um ano. Os indivíduos foram submetidos a avaliação clínica e ecocardiográfica. A capacidade funcional foi avaliada pelo teste de caminhada de seis minutos. Foram empregados os testes qui-quadrado, exato de Fisher e coeficiente de correlação de Pearson, considerando o nível de significância de 5%. Resultados: De março de 2010 a dezembro de 2013, foram avaliados 61 indivíduos, 67,2% do sexo feminino, com 7-41 anos de idade e uso de MP há 13,5 ± 6,3 anos. O percentual de estimulação ventricular era 97,9 ± 4,1% e a duração do complexo QRS estimulado era de 153,7 ± 19,1 ms. A maioria (95,1%) era assintomática e não utilizava medicamentos. A distância média percorrida de 546,9 ± 76,2 m teve forte correlação com a distância predita (r = 0,907; p = 0,001) e não se associou com os fatores de risco de remodelamento estudados. Conclusões: A capacidade funcional de portadores de BAVTCi com estimulação crônica no VD foi satisfatória e não se correlacionou com os fatores de risco para remodelamento ventricular. .
Subject(s)
Adolescent , Adult , Child , Female , Humans , Male , Young Adult , Atrioventricular Block/rehabilitation , Cardiac Pacing, Artificial/methods , Exercise Test , Heart Block/congenital , Pacemaker, Artificial , Ventricular Dysfunction, Right/rehabilitation , Atrioventricular Block/physiopathology , Cross-Sectional Studies , Echocardiography , Follow-Up Studies , Heart Block/physiopathology , Heart Block/rehabilitation , Prospective Studies , Risk Factors , Time Factors , Treatment Outcome , Ventricular Dysfunction, Right/physiopathology , Ventricular Remodeling/physiology , Walking/physiology摘要
Maternal connective tissue disorders such as Systemic Lupus Erythematosus [most common], Sjogren's syndrome, mixed connective tissue disorders may lead to the rare condition of complete congenital heart block in the neonate. Rare fetal syndromes such as myocarditis, 18p syndrome, mucopolysaccharidoses and mitochondrial diseases are other causes. The mortality rate of this condition is inversely propotional to the age of presentation being 6% in the neonatal age group. As the cardiac output in the neonate is heart rate dependent, it is crucial to maintain the heart rate in these patients. Pharamacological interventions with dopamine, isoprenaline, epinephrine and atropine are known for their variable response. Although permanent pacing is the most reliable mode of management, the access to it is often not readily available, especially in the developing countries. In such cases temporary pacing methods become lifesaving. Of all the modalities of temporary pacing [transcutaneous, transesophageal and transvenous] transcutaneous pacing is the most readily available and immediate mode. In this case report we present a two day old neonate with isolated complete congenital heart block and a resting heart rate of 50-55/min in immediate need of palliative surgery for trachea-esophageal fistula [TEF]. With pharmacological intervention the heart rate could only be raised to 75-80/min. The surgery was successfully carried out using transcutaneous pacing to maintain a heart rate of 100/min
Subject(s)
Humans , Male , Heart Block/congenital , Cardiac Pacing, Artificial , Heart Block/etiology摘要
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...
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...
Subject(s)
Humans , Female , Child, Preschool , Child , Adolescent , Young Adult , Atrioventricular Block/congenital , Heart Block/congenital , Heart Ventricles , Lupus Erythematosus, Systemic , Pediatrics , Quality of Life , Ventricular Dysfunction, Left , Ventricular Function , Ventricular Remodeling , Young Adult , Autoantibodies , Cross-Sectional Studies , Echocardiography , Pacemaker, Artificial/adverse effects摘要
Neonatal lupus erythematosus is an immune-mediated disease caused by transplacental passage of maternal autoantibodies, primarily anti-Ro [SSA] and anti-La [SSB]. The major clinical manifestations are congenital heart block, cutaneous lupus lesions, and hematologic problems. Hepatic, pulmonary, and neurological involvements are rare. We report a 5-day-old male neonate, born to a clinically asymptomatic mother, presenting with conjugated hyperbilirubinemia, cutaneous lupus lesions, congenital heart block, and thrombocytopenia. Both the neonate and his mother had high titers of antinuclear antibodies [1:640], anti-Ro [SSA], and anti-La [SSB] antibodies. The thrombocytopenia improved with prednisolone [2 mg/kg/day] for 14 days. The skin lupus rashes and bilirubin resolved 2 months later, and liver enzymes were completely normal by 6 months
Subject(s)
Humans , Male , Lupus Erythematosus, Systemic , Infant, Newborn , Hyperbilirubinemia , Heart Block/congenital , Thrombocytopenia , Antibodies, Antinuclear , Ribonucleoproteins , Lupus Erythematosus, Cutaneous , Prednisolone摘要
A síncope causada por bloqueio atrioventricular paroxístico, definido como bloqueio de segundo ou terceiro grau transitório, raramente é relatada em pacientes pediátricos sem cardiopatias congênitas...
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Heart Block/complications , Heart Block/congenital , Heart Defects, Congenital/complications , Heart Defects, Congenital/diagnosis , Pacemaker, Artificial摘要
We describe case report of a 45 days old male baby with neonatal lupus erythematosus, who presented with 3rd degree congenital heart block and depigmented skin lesions on face and upper part of body. Diagnosis of the baby was confirmed by anti nuclear levels and skin biopsy.
Subject(s)
Antibodies, Antinuclear/analysis , Heart Block/congenital , Humans , Infant , Lupus Erythematosus, Cutaneous/complications , Male , Skin Pigmentation/immunologySubject(s)
Humans , Male , Female , Infant, Newborn , Heart Block/complications , Heart Block/congenital , Heart Block/epidemiology , Heart Block/immunology , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/therapy , Autoimmune Diseases/complications , Autoimmune Diseases/congenital摘要
Desenvolvemos um novo eletrodo para implante de marcapasso fetal, com o objetivo de se evitar a cirurgia intra-uterina a céu aberto. O eletrodo foi implantado por punção miocárdica a céu aberto nos corações de cinco fetos caprinos, para avaliação eletrofisiológica. A resistência foi de 1050,4e a onda R foi de 8,64mV. Os limiares de estimulação foram baixos, permanecendo relativamente constantes (>0,70 V) para larguras de pulso acima de 0,50 mseg, o que representa ótimo desempenho. O sistema de implante percutâneo de marcapasso fetal proposto poderá representar alternativa interessante para tratar o bloqueio átrio-ventricular total congênito, sem necessidade de exposição fetal / We have developed a new electrode for fetal pacing that avoids the need for intrauterine open surgical procedures. The electrode was implanted by myocardium puncture in five fetal goats for electrophysiological assessment. The stimulation resistance was 1050,4 and sensed fetal R wave of 8.64 mV. The acute stimulation thresholds were consistently low, remaining relatively constant (>0.70 V) at pulse widths greater than 0.5 msec, with excellent performance. The proposed percutaneous fetal pacing system may represent an interesting alternative to treat congenital complete heart block, with no need for fetal exposure...
Subject(s)
Animals , Heart Block/surgery , Heart Block/complications , Heart Block/congenital , Fetal Heart/surgery , Fetal Heart/physiopathology , Fetal Heart/metabolism , Arrhythmias, Cardiac , Disease Models, Animal , Goats , Hydrops Fetalis/surgery , Hydrops Fetalis/physiopathology , Pacemaker, Artificial摘要
CLINICAL PRESENTATION: Congenital heart block (CHB) in the absence of major structural abnormalities is associated with maternal antibodies to Ro (SS-A) and La (SS-B). CHB is most commonly diagnosed between 18 and 24 wk of gestation, and may be first, second or third degree (complete). Mortality approaches approximately 20%, and most surviving children require pacemakers. Affected infants may develop cardiomyopathy. Abnormalities in the skin, liver and blood of neonates are also associated with anti-Ro/La antibodies, and are usually self-limiting; these manifestations and CHB are collectively referred to as neonatal lupus syndromes (NLS). INVESTIGATION OF PATHOGENESIS: Recent studies demonstrate that Ro/La ribonucleoproteins appear on the surface of apoptotic fetal cardiocytes and are recognized by their cognate antibodies, promoting an inflammatory response. Mice immunized with Ro/La proteins have offspring with conduction abnormalities. In vitro, human serum and IgG with anti-Ro/La antibodies affect the conducting properties of isolated animal heart tissue. DIAGNOSTIC PROBLEMS: If fetal bradycardia is identified, a 2-dimensional and M-mode fetal echocardiographic and Doppler ultrasound should be obtained to determine whether there is an atrial arrhythmia or atrioventricular (AV) block, and to what degree, and whether there are major structural abnormalities of the heart. The mother's serum should be tested by ELISA for anti-Ro and/or anti-La antibodies. THERAPEUTIC OPTIONS: To date, only anecdotal and retrospective evidence guides in utero therapy of CHB. A prospective trial is currently underway to evaluate the efficacy of maternal oral dexamethasone in treating newly identified first, second or third degree block. Established third-degree block appears to be irreversible. Dexamethasone and sympathomimetics may be of some benefit in treating hydrops fetalis. In pregnant women with anti-Ro/La antibodies, prophylactic therapy is not indicated but serial echocardiographic analysis is strongly recommended, with emphasis on the mechanical PR interval to identify a reversible block. CONCLUSION: CHB occurs in approximately 1-5% of pregnancies in mothers with anti-Ro/La antibodies, independent of the mother's disease status, and in approximately 15-20% of pregnancies following the birth of a child with NLS. Treatment of CHB identified in utero is not established but guidelines are provided. Serial echocardiographic monitoring of high-risk pregnancies, using the mechanical PR interval to identify first degree block, may afford the earliest opportunities for therapeutic intervention.
Subject(s)
Antibodies, Antinuclear/immunology , Autoimmune Diseases/immunology , Female , Heart Block/congenital , Humans , Infant, Newborn , Lupus Vulgaris/immunology , Pregnancy , Pregnancy Complications, Cardiovascular摘要
Se presenta un caso de bloqueo aurículo-ventricular congénito diagnosticado prenatalmente en un feto de 30 semanas. Se produce el parto por vía transpelviana, eutócico, obteniéndose recién nacido vivo con peso, talla y apgar normales. Se revisa la etiopatogenia del BAVC en relación con los daños autoinmunes al sistema fetal de conducción por anticuerpos IgG anti SSA/Ro y anti SSB/La derivados de madres que sufren enfermedades autoinmunes. Se discute el diagnóstico, cuadro clínico, tratamiento y pronóstico del BAVC. Se exponen malformaciones menores presentes en nuestro caso, no reportadas anteriormente en relación con el BAVC
Subject(s)
Humans , Adult , Female , Pregnancy , Infant, Newborn , Antibodies, Anti-Idiotypic , Autoimmune Diseases , Heart Block/congenital , Heart Block/drug therapy , Heart Block , Fetal Diseases , Heart Defects, Congenital , Immunoglobulin G , Ultrasonography, Prenatal摘要
Neonatal lupus erythematosus (NLE) is an auto-immune disease related to systemic lupus erythematosus (SLE). Unlike SLE it is not a spontaneous syndrome but rather an acquired one. In NLE the most common disease manifestations are a transient cutaneous lesion and cardiac conduction disturbances. The cutaneous lesions and other non-cardiac manifestations of NLE are transient and disappear about six months after birth, at the time when maternal antibodies disappear from the neonatal circulation. This fact suggests that maternal antibodies may cross the placenta leading to an inflamatory reaction in the fetal tissues. NLE is the principal cause of atria-ventricular block, when it is not associated with congenial birth defects. All the clinical studies to date correlate the heart block in NLE with the presence of certain types of circulating maternal antibodies, against the Ro/SSA nuclear proteins, in the serum of the newborn. In this paper we discuss animal models that have been developed by our and other groups to study the participation of the anti-Ro/SSA antibodies in the pathogenesis of the cardiac conduction blockades that occur in NLE.
Subject(s)
Animals , Antibodies, Antinuclear/immunology , Heart Block/congenital , Heart Block/immunology , Immunity, Maternally-Acquired , Lupus Erythematosus, Systemic/congenital , Lupus Erythematosus, Systemic/immunology , Autoantibodies/immunology , Disease Models, Animal , Syndrome摘要
Este estudio describe la historia natural y evolución de 67 pacientes con bloqueo auriculoventricular (BAV) completo congénito ingresados en el Instituto Nacional de Cardiología "Ignacio Chávez", México, D.F. de 1944 a 1998. Fueron 35 (52 por ciento) mujeres y 32 (47 por ciento) hombres, con un periodo de seguimiento medio de 93.7 ñ 104 meses, la mayoría de los pacientes tenían corazón estructuralmente sano (90 por ciento). Los síntomas más comunes fueron disnea y sincope. El electrocardiograma mostró una frecuencia ventricular media de 42.2 ñ 9 latidos/minuto en pacientes con sincope. El intervalo QTc promedio fue 403 ñ 60 ms. El 85.7 por ciento de los pacientes tuvieron un BAV suprahisiano. En 31 po ciento de los pacientes se implantó un marcapasos definitivo debido a sincope. La mortalidad general fue de 4.4 por ciento y las arritmias ventriculares malignas fueron las contribuyentes principales. Los factores de riesgo para mortalidad, identificados en este estudio, fueron ritmo de escape de la unión AV menor de 50 latidos/minuto, respuesta cronotrópica inapropiada durante el ejercicio, prolongación del intervalo R-R durante la noche, dilatación de cavidades cardiacas, fracción de eyección ventricular izquierda deprimida y prolongación del QTc. En todas éstas condiciones nosotros recomendamos la implantación de un marcapasos permanente
Subject(s)
Humans , Male , Female , Heart Block/congenital , Heart Block/diagnosis , Heart Block/therapy , Echocardiography , Electrocardiography , Incidence , Mexico/epidemiology , Pacemaker, Artificial , Disease Progression , Radiography, Thoracic , Retrospective Studies摘要
A síndrome do lúpus neonatal (LN) é uma entidade rara, caracterizada por bloqueio cardíaco congênito (BCC)e/ou lesões cutâneas e, eventualmente, associada a alterações hematológicas e hepáticas. Essa síndrome está estritamente relacionada com a passagem transplacentária de auto-anticorpos maternos, particularmente anti-Ro/SSa e anti-La/SSB, sendo considerada um modelo de auto imunidade adquirida passivamente. Aproximadamente, metade das mães são assintomáticas e o restante apresenta síndrome de Sjögren, lúpus eritematoso sistêmico e outras doenças do sistema conectivo. Essa síndrome é a principal causa de bloqueio cardíaco congênito isolado e determina alto índice de mortalidade. Não existe tratamento preventivo para a síndrome, sendo recomendado que a gestação de mães com anticorpos anti-Ro/SSA ou anti-La/SSB tenha acompanhamento de frequência cardíaca fetal rigoroso. Nos casos em que há evidências de inflamação do miocárdio concomitante está indicado o tratamento com corticóide sistêmico que não seja inativado pela placenta, na tentativa de reverter o quadro
Subject(s)
Humans , Infant, Newborn , Autoantibodies , Autoimmune Diseases/congenital , Autoimmunity , Heart Block/congenital , Heart Block/physiopathology , Lupus Vulgaris/diagnosis , Lupus Vulgaris/physiopathology , Lupus Vulgaris/therapy , Dexamethasone/therapeutic use , Hydrocortisone/therapeutic useSubject(s)
Humans , Pregnancy , Female , Echocardiography , Ultrasonography, Prenatal/methods , Heart Block/congenital , Cardiac Complexes, Premature , Aortic Valve Stenosis , Pulmonary Valve Stenosis , Heart Neoplasms , Heart Septal Defects, Atrial , Hypoplastic Left Heart Syndrome , Tachycardia , Tetralogy of Fallot摘要
El bloqueo cardíaco completo congénito es una alteración del sistema de conducción cardíaco a nivel del nodo aurículo-ventrícular. Es una entidad poco común y los pacientes que adolecen de la enfermedad suelen ser asintomáticos o bien tener sintomatología desde la vida fetal o en el nacimiento. Cuando desarrollan síntomas, estos pacientes necesitan ser tratados, en forma farmacológica o con el uso de marcapaso artificial. Reportamos los primeros 2 casos de bloqueo cardíaco completo congénito en niños tratados con la implantación de marcapaso permanente en Honduras