ABSTRACT
Congenital complete heart block (CCHB) is a rare cardiac disorder in neonates and maternal lupus is the most common cause. More rarely, CCHB can be associated with congenitally corrected transposition of the great arteries. In this case, the neonate was born via emergency caesarean section due to bradycardia. Post-natal electrocardiogram and echocardiography confirmed the diagnosis. A pacemaker was inserted and the baby was kept under follow-up.
ABSTRACT
@#Neonatal lupus is a passively acquired autoimmune disease that occurs in children of mothers with anti-Ro/SSA and/or anti-La/SSB antibodies. [1-4] The most serious complication in the neonate is complete heart block. [3-8] This is a case report of a newborn female presenting with persistent bradycardia detected in utero. The diagnosis was confirmed by maternal anti-Ro/SSA and/or anti-La/SSB antibodies and in utero detection of fetal heart block on echocardiogram. Therapeutic management involved placement of a permanent pacemaker.
ABSTRACT
Complete heart block comprises complete absence of AV conduction - none of the supraventricular impulses are conducted to the ventricles. Perfusing rhythm is maintained by a junctional or ventricular escape rhythm. Typically, the patient will have severe bradycardia with independent atrial and ventricular rates. The incidence is 1 in 15,000 to 20,000 live births. Authors present the case report of a primigravida, aged 30 years who presented with amenorrhoea of 35 weeks. She was diagnosed as complete heart block by cardiologist. She had no history of syncopal attacks during childhood or antenatal period. She had a heart rate of 50-52 bpm. ECG showed ventricular escape rhythm with narrow QRS complexes. Echocardiography showed no structural defects. Her elective LSCS was done at 39 weeks after consultation with cardiologist. Patient underwent temporary pacemaker insertion just prior to surgery and she delivered a healthy female baby of weight 3 kg with Apgar 9/10 at 5 mins. She remained asymptomatic during the postoperative period and pacemaker was removed after 30 hours. She was discharged under satisfactory condition on day 6. Patient was counselled for follow up with cardiologist. This case lays emphasis on proper history taking, vitals monitoring, early recognition of undiagnosed cardiac disorders and team work for good maternal and fetal outcome.
ABSTRACT
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, PrenatalABSTRACT
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/congenitalABSTRACT
Sjogren's syndrome is one of the most common autoimmune disorders and has a female predominance. Maternal circulating autoantibodies such as anti-Ro/SSA and anti-La/SSB antibodies can cause congenital heart block of fetus, and in severe case, emergency pacemaker implantation may be needed for neonate. Therefore, it is very important to understand maternal and fetal condition and pay attention to the status of the neonate during delivery. In this paper, we present a case of patient with Sjogren's syndrome who underwent cesarean section under spinal anesthesia.
Subject(s)
Female , Humans , Infant, Newborn , Pregnancy , Anesthesia , Anesthesia, Spinal , Antibodies , Autoantibodies , Cesarean Section , Emergencies , Fetus , Heart Block , Sjogren's SyndromeABSTRACT
ObjectiveTo explore the pathogenesis, clinical manifestations, diagnosis, and treatment of autoanti-body-associated congenital heart block.MethodsThe clinical data of one child with autoantibody-associated congenital heart block was retrospectively analyzed.ResultsIn 24 week gestation, fetal bradycardia had been found by routine fetal echocar-diography. After birth, the anti-SSA/Ro antibodies and anti-SSB/La antibodies were positive in both infant and her mother. The diagnosis of autoantibody-associated congenital heart block was conifrmed. Intravenous immunoglobulin at 1 g/kg was adminis-trated. At 6 months follow-up, the electrocardiogram suggested type I second degree atrioventricular block.ConclusionIn the fetus or neonates found to have bradycardia and excluded the cardiac structural abnormalities, the autoimmune antibody should been tested and early intervention should been promoted.
ABSTRACT
Congenital Heart Block (CHB) is the most serious complication of neonatal lupus erythematosus. Transplasental transfer of maternal anti-SSA/Ro or anti-SSB/La antibodies around 12th week of gestation is associated with development of CHB. This may lead to inflammation, fibrosis and scarring of fetal conduction system in the early second trimester. Different degrees of atrioventricular (AV) block may be seen in the affected fetus. First and second-degree AV blocks may change in severity; however, third degree AV block is irreversible. CHB is mostly diagnosed between 18- 24th weeks of gestation. Even if most of the mothers carrying autoantibodies of several rheumatic diseases such as systemic lupus erythematosus or Sjogren’s syndrome are not aware of their diseases until their children are born with CHB, it is recommended that antibody-positive mothers or the mothers having babies with neonatal lupus erythematosus should be referred for close fetal echocardiographic surveillance beginning from the early second trimester. Although their utility is still controversial, various therapeutic regimes such as sympathomimetic, plasmapheresis, steroids, intravenous immunoglobulin, digoxin, diuretic and in utero pacing have been used for intrauterine treatment of CHB. Aggressive medical treatment is coupled with pacing in infants who do not respond to medical therapy alone.
ABSTRACT
This review summarizes the epidemiology, etiology, clinical manifestation, treatment, follow-up of neonatal lupus erythematosus with focus on new discoveries on the etiology of the disease in recent years including anti-SSA/Ro and anti-SSB/La antibodies, serotonin (5-hydroxytryptamine 4), apoptosis of cardiac cells, calcium channels, maternal micro-chimera, genetic variants, to improve clinician awareness of the disease.
ABSTRACT
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.