Subclinical maternal autoimmune disease leading to congenital high degree atrioventricular block: Case report and review of the literature.

Maternal autoimmune disease is the most common cause of congenital heart block (CHB), a rare illness characterized by fibrosis and calcification of the fetal atrioventricular (AV) node due to maternal autoantibodies anti-SSA/Ro and anti-SSB/La. We report the full autopsy and clinical information on a female neonate with high degree AV block and calcification in the AV node, atrial approaches to the AV node, and both right and left bundle branches, born to a 27-year-old female with subclinical autoimmune disease.

Tratamiento intervencionista con stent para el síndrome de vena cava superior con complicación por el uso de marcapasos en un lactante / Stent interventional treatment of superior vena cava syndrome with a pacemaker placement complication in an infant

Resumen Introducción: El marcapasos epicárdico es el método preferido en recién nacidos y niños que pesan menos de 10 kg. Sin embargo, su colocación cuenta con una alta incidencia de fracaso prematuro. Caso clínico: Se presenta el caso de un lactante que requirió la colocación de un marcapasos por bloqueo atrioventricular congénito diagnosticado prenatalmente. El paciente presentaba estenosis crítica de la vena cava superior como complicación secundaria a los cables del marcapasos. La resolución intervencionista mediante un stent en el sitio de estenosis corrigió el síndrome de vena cava superior de forma efectiva. Conclusiones: Este caso representa una alternativa clínicamente viable en el tratamiento del síndrome de vena cava superior, que puede emplearse después de otras terapias convencionales que hayan fallado.

Abstract Background: Epicardial pacemaker is the preferred method for newborns and children under 10 kg of weight. However, its placement presents a high rate of premature failure. Case report: The case of an infant requiring placement of pacemaker by congenital atrioventricular block with prenatal diagnosis is described. The patient showed critical stenosis of the superior vena cava as a complication. Interventional resolution using a stent at the site of stenosis corrected the superior vena cava syndrome effectively. Conclusions: This case represents a clinically viable alternative for the treatment of superior vena cava syndrome, which can be used after other conventional therapies have failed.

Implante de marcapasso em paciente com veia cava superior esquerda persistente / Pacemaker implant in patients with persistent left superior vena cava

A persistência da veia cava superior esquerda, embora rara, é a anomalia venosa mais encontrada no tórax, com prevalência de 0,3-0,5% na população geral. Em associação com a cardiopatia congênita, sua prevalência aumenta para 2,8-4,3%. Pacientes com cardiopatia congê- nita que necessitem de tratamento cirúrgico podem evoluir, durante a cirurgia, com bloqueio atrioventricular total e necessitar de implante de marcapasso. Apresentamos o caso de uma paciente com cardiopatia congênita, incluindo veia cava superior esquerda persistente, submetida a várias intervenções cirúrgicas, evoluindo com bloqueio atrioventricular total e necessitando de implante de marcapasso bicameral

The persistence of the left superior vena cava, though rare, is the most common venous anomaly in the chest, with a prevalence of 0.3%-0.5% in the general population. When combined with congenital heart disease, its prevalence increases to 2.8%-4.3%. Patients with congenital heart disease who require surgical treatment might evolve to a condition of complete heart block, and consequently require a pacemaker implant. We will present the case of a patient with congenital heart disease, including persistent left superior vena cava, which has been subjected to several surgical interventions, evolving with total ventricular atrial block and requiring a bicameral pacemaker implant

Avaliação da estimulação ventricular direita crônica em crianças e adultos jovens com bloqueio atrioventricular congênito isolado / Evaluation of chronic right ventricular pacing in children and young adults with isolated congenital complete atrioventricular block

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...

Bloqueo aurículo-ventricular congénito completo y anticuerpos maternos anti-Ro positivos / Complete congenital heart block and maternal autoantibodies anti-Ro positives

El bloqueo cardíaco congénito (BCC) es una entidad poco frecuente que se produce por la lesión del tejido de conducción cardíaco, con una alteración secundaria de la transmisión de los impulsos auriculares a los ventrículos. Se puede asociar a cardiopatías estructurales y a enfermedades autoinmunes como el lupus eritematoso sistémico o a la presencia de autoanticuerpos anti-Ro y anti-La. El diagnóstico prenatal se realiza mediante ecografía y ecocardiografía al objetivar una bradicardia fetal mantenida; ambas técnicas permiten asimismo el seguimiento y la valoración de finalización de la gestación ante la presencia de signos de deterioro hemodinámico. El tratamiento médico intraútero se efectúa con fármacos inotrópicos y corticoides y una vez finalizada la gestación la terapia definitiva generalmente será la implantación de un marcapasos. Presentamos el caso de una gestante de 26 semanas que acude a urgencias por dolor abdominal. Durante la exploración se objetiva un latido fetal anómalo confirmando la existencia de un BCC. Como único hallazgo analítico materno alterado se encuentra la presencia de anticuerpos de origen autoinmune ANA y anti Ro positivos (AU)

Congenital heart block is a rare anomaly produced by an injury to the specialized cardiac conducting tissue, with secondary alterations in the transmission of stimuli from the atria to the ventricles. This defect can be associated with structural heart disease and maternal autoimmune diseases such as systemic lupus erythematosus syndrome or the presence of anti-Ro and anti-La autoantibodies. Prenatal diagnosis is given by echocardiography, which shows persistent fetal bradycardia, and allows detection of fetal hemodynamic deterioration, requiring finalization of pregnancy. Intrauterine therapy consists of sympathomimetics and steroid agents and a pacemaker can be implanted in the newborn as a definitive treatment at the end of the pregnancy. We report the case of an asymptomatic mother at week 26 of pregnancy who presented to the emergency department with abdominal pain. On examination, an anomalous fetal heart rate was detected and congenital heart block was confirmed. The only abnormal result of maternal blood test was the presence of positive anti-nuclear antibodies and anti-Ro autoantibodies (AU)

Búsqueda prenatal de bloqueo aurículo ventricular congénito en población de riesgo: experiencia Universidad Católica de Chile / Congenital heart block in risk population: Universidad Catolica de Chile experience

Congenital heart block is a fetal arrhythmia detected by routine prenatal ultrasound. Natural history and risk factors for this condition are known. Patients having Ro/La antibodies have 2 to 4 percent risk of affected child and recurrence of up to 25 percent. There are standarized protocols for measuring the PR interval by ultrasound, as well as reference curves for different gestational ages. The experience at Hospital Clínico de la Universidad Católica de Chile is reported, describing follow up of 37 patients over a period of more than 4 years. A protocol based on monthly monitoring of risk group was adopted.

El bloqueo aurículo ventricular congénito constituye una de las arritmias fetales detectables mediante ultrasonido prenatal de rutina. Se conoce su evolución natural y la población de riesgo de sufrir esta afección. En portadoras de anticuerpos Ro/La el riesgo de un hijo afectado es entre 2 y 4 por ciento, y la recurrencia de hasta un 25 por ciento. Existen protocolos estandarizados para medir el intervalo PR mediante ultrasonido, como también curvas de referencia para las distintas edades gestacionales. Reportamos la experiencia en el Hospital Clínico de la Universidad Católica de Chile, con un seguimiento de 37 pacientes en un periodo de más de 4 años. Se adoptó un protocolo de seguimiento mensual del grupo de riesgo.

Cardiac Resynchronization Therapy for Left Ventricular Dysfunction Induced by Chronic Right Ventricular Pacing in a Child

Cardiac resynchronization therapy (CRT) has been proven its value in adult patients with congestive heart failure of low ejection fraction and wide QRS duration. Contrast to adult patients, CRT has been rarely applied for young patients. We report on a 9-yr-old boy with progressive left ventricular (LV) dilatation and dysfunction following chronic VVI pacemaker therapy for congenital complete atrioventricular block associated with maternal anti-SSA/Ro and SSB/La antibody. His LV dysfunction was improved after epicardially established CRT.