ABSTRACT
Objective:To study the diagnosis, treatment and outcome of congenital complete atrioventricular block (CCAVB) in neonates before, during and after delivery and to monitor their growth and development.Methods:From December 2017 to June 2021, clinical data of 5 neonates with CCAVB admitted to the neonatal department of our hospital were analyzed.Results:A total of 3 males and 2 females were included. The diagnosis was established at (24.6±3.6) weeks during the fetus period. The average birth weight was (2 493±551) g and the gestational age was (36.7±1.5) weeks. 4 mothers had autoimmune diseases, with positive antinuclear antibodies, SSA and Ro52. The cause of the remaining 1 neonate was undetermined. 4 neonates received isoproterenol treatment after birth, but the therapeutic effects were unsatisfying. The longest continuous isoproterenol infusion in 1 neonate lasted for 18 d. Permanent pacemakers were implanted during hospitalization in 3 neonates and 2 neonates were implanted within 1 year after birth. 1 neonate died of septic shock following pacemaker pocket infection at 26 d after birth. The other 4 neonates were followed up to 1-year-old after pacemaker implantation and their growth and development were otherwise normal.Conclusions:Most of the mothers of CCAVB neonates have autoimmune diseases. Fetal arrhythmias are often found in the second trimester during pregnancy, but most of the neonates can be delivered near full-term. The effects of drug therapy are poor and pacemakers are necessary.
ABSTRACT
<p>For A 38-year-old male diagnosed a congenital complete atrioventricular block in the neonatal period, epicardial lead and pacemaker was implanted through left thoracotomy. Although we tried to implant a pacemaker through the subclavian vein as an adult, it was unsuccessful because of obstruction of the bilateral subclavian vein. For this reason, we performed a pacemaker implantation with transatrial-endocardial lead through the right thoracotomy due to save the generator electric power. This is one of the useful techniques for cases with obstruction of the upper extremity vein.</p>
ABSTRACT
Congenital complete atrioventricular (AV) block is a rare neonatal disease. It is a passively acquired immune-mediated injury of the conduction system, triggered by transplacental passage of maternal anti-SSA/Ro and anti-SSB/La antibodies. Management of premature infants with symptomatic complete AV block is challenging. If medical treatment with a β-adrenergic agonist and inotropic drugs is not effective, early cardiac pacing should be considered. Here we report a case of congenital complete AV block in a low birth weight, preterm neonate, who was successfully treated with temporary transcutaneous pacing immediately after birth. Temporary transcutaneous pacing may be an option for the emergent management of a low birth weight preterm neonate with congenital complete AV block prior to permanent pacemaker implantation.
Subject(s)
Humans , Infant, Newborn , Infant, Newborn , Antibodies , Atrioventricular Block , Infant, Low Birth Weight , Infant, Premature , ParturitionABSTRACT
El bloqueo auriculoventricular es un retardo o interrupción del impulso eléctrico proveniente del nodo sinusal a nivel del nódulo auriculoventricular. Es congénito en uno de cada 20,000-25,000 nacidos vivos. Secundario a desarrollo embrionario anormal del nodo auriculoventricular asociado a anomalías cardiacas estructurales o por isoinmunización materna con anticuerpos que dan daño inmunológico irreversible del tejido cardiaco del feto, por inflamación y fibrosis. Los factores de mal pronóstico son: coexistencia con malformaciones cardiovasculares, insuficiencia cardiaca, frecuencia ventricular menor de 50 por min, bradicardia durante el sueño menos de 30 por min, marcapaso bajo o cambiante, QT prolongado. En asintomáticos, se recomienda seguimiento con monitoreo y ecocardiografía. Las indicaciones de marcapasos permanente son: ritmo de escape con complejos anchos, ectopia y disfunción ventricular, QT largo, cardiomegalia y auriculomegalia derecha.
The atrioventricular block is a delay or interruption of the electrical impulse from the sinusal node, to level of the auriculoventricular nodule is a congenital (BAVC), in one of every 20,000-25,000 born alive. Secondary to an abnormal embryonic development of the node AV, associated with structural cardiac abnormalities or for maternal is immunization with antibodies that cause immunological irreversible damage in the fetal heart tissue, by inflammation and subsequent fibrosis. The factors of worst prognosis are: Coexistence with cardiovascular malformations, heart failure, ventricular frequency below 50 per minute, bradycardia less than 30 per minute during sleep, pacemaker under or changing, long QT. In asymptomatic patient, it is recommended monitoring and follow-up with echocardiography. Indications for permanent pacemaker are: escape rhythm with wide complex, ectopy and ventricular dysfunction, long QT, cardiomegaly and right atrial dilatation.
ABSTRACT
A síndrome de Kearns-Sayre é uma mitocondriopatia caracterizada por disfunções multiorgânicas que classicamente se desenvolve antes dos vinte anos de idade. Os critérios diagnósticos mais aceitos na literatura são a tríade: oftalmoplegia externa progressiva, retinopatia pigmentar e distúrbio de condução cardíaco. O prognóstico da SKS está relacionado ao número de tecidos acometidos, bem como a gravidade das alterações. Neste artigo relata-se caso de um paciente de 18 anos que apresentou características clínicas compatíveis com a síndrome de Kearns-Sayre.
Kearns-Sayre syndrome The Kearns-Sayre syndrome (KSS) is a mitochondriopathies characterized by multiorgan dysfunction that typically develops before the age of twenty. The diagnostic criteria most widely accepted in the literature are a triad: progressive external ophthalmoplegia, pigmentary retinopathy and disorders of cardiac conduction. KSS prognosis is related to the number of tissues affected and the severity of the alterations. In this article we report on a patient who presented 18 clinical features consistent with the Kearns-Sayre syndrome.
Síndrome de Kearns-Sayre El síndrome de Kearns-Sayre (SKS) es una mitocondriopatía caracterizada por disfunciones multiorgánicas que clásicamente se desarrolla antes de los veinte años de edad. Los criterios de diagnóstico más ampliamente aceptados en la literatura son una tríada: oftalmoplejía externa progresiva, retinopatía pigmentaria y trastornos de la conducción cardíaca. El pronóstico del SKS está relacionado con el número de los tejidos afectados y la severidad de las alteraciones. En este artículo, se presenta un caso de un joven de 18 años que presentaba características clínicas compatibles con el síndrome de Kearns-Sayre.
ABSTRACT
Neonatal Lupus Erythematosus (NLE) is a disease of the newborn defined by presence of maternal autoantibodies and characteristic clinical features (congenital complete atrioventricular block, skin lesion, etc.). Although the autoantibodies often are not associated with clinical disease in the mother, NLE is likely the result of fetal or neonatal tissue damage caused by maternally transmitted IgG autoantibodies. The majority of morbidity and mortality of NLE results from congenital complete atrioventricular block. Most of the neonates with NLE have maternally transmitted anti-SSA/Ro antibodies. Anti-SSB/La antibodies are found in 40 to 50 percent of patients. Anti-SSA/Ro antibodies are the most important autoantibodies in the pathophysiology of NLE. Noncardiac manifestations of neonatal lupus are transient, resolving at about 6 months of life, coinciding with the disappearance of maternal autoantibodies from neonatal circulation. Congenital complete atrioventricular block is irreversible, and permanent pacemaker insertion is needed. We report a case of NLE with congenital complete atrioventricular block with literature reviews.
Subject(s)
Humans , Infant, Newborn , Antibodies , Atrioventricular Block , Autoantibodies , Immunoglobulin G , Mortality , Mothers , SkinABSTRACT
Congenital complete atrioventicular block is uncommon which has a heterogenous etiology. According to recent studies, about one-third of the mothers of the patients who were diagnosed as congenital complete atrioventricular block, had symptoms or signs of connective tissue disease. Even though the outlook is usually regarded as favorable, the mortality is highest in the neonatal period, much lower during childhood and adolescence and increases slowly later in life. In the neonatal period the predominant indication for pacing was heart failure. It is difficult to make a prognosis in the individual patient. A slow, fixed or decreasing venticular rate neonatally and a prolonged QT interval seem to be bad prognostic sign. We report a case of congenital complete atrioventricular block who was diagnosed by fetal echocardiography. Her mother was asymptomatic, but had positive serologic test to anti-Ro antibody and anti-La antibody. We report a case of congenital complete atrioventricular block with a brief review of related literatures.