Perinatal management of neonatal congenital complete atrioventricular block: a survey of 5 cases / 中国新生儿科杂志

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.

Unusual ventricular activation produced by temporary transvenous cardiac pacing: electrovectorcardiographic findings / Activación ventricular inusual producida por estimulación cardíaca transvenosa temporal: hallazgos electrovectorcardiográficos

Abstract Complete heart block (CHB) results from dysfunction of the cardiac conduction system, which results in complete electrical dissociation. The ventricular escape rhythm can have its origin anywhere from the atrioventricular node to the bundle branch-Purkinje system. CHB typically results in bradycardia, hypotension, fatigue, hemodynamic instability, syncope, or even Stokes-Adams syndrome. Escape rhythm originating above the bifurcation of the His bundle (HB) produces narrow QRSs with relatively rapid heart rate (HR) (except in cases of His system disease). We present a middle-aged man with an HR of 34 bpm, progressive fatigue, in whom a temporary pacemaker was implanted in the subtricuspid region. The post-intervention electrocardiogram had unusual features.

Resumen El bloqueo cardíaco completo (BCC) resulta de la disfunción del sistema de conducción cardíaco, lo que ocasiona una disociación eléctrica completa entre aurículas y ventrículos. El ritmo de escape resultante puede tener su origen en cualquier lugar desde el nodo auriculoventricular hasta el sistema His Purkinje. El BCC generalmente produce bradicardia, hipotensión, fatiga, inestabilidad hemodinámica, síncope o incluso el síndrome de Stokes-Adams. El ritmo de escape que se origina por encima de la bifurcación del haz de His produce intervalos QRS estrechos con frecuencia cardíaca no muy lenta (excepto en casos de enfermedad del sistema Hisiano). Presentamos a un hombre de mediana edad con una frecuencia cardíaca de 34 lpm, fatiga progresiva, en el que se implantó un marcapasos temporario en la región subtricuspídea. El electrocardiograma resultante a la intervención presentó características inusuales.

Bloqueo auriculoventricular completo y asistolia en un niño con ataxia-telangiectasia / Complete heart block and asystole in a child with ataxia-telangiectasia

La ataxia-telangiectasia es una entidad caracterizada por un cuadro de ataxia cerebelosa progresiva, telangiectasias, defectos inmunológicos y una mayor tendencia al desarrollo de tumores malignos. La mutación genética responsable (ataxia-telangiectasia mutada) parece jugar un papel importante en la función celular normal y el remodelado cardiovascular. Se describe la aparición de una arritmia maligna en un paciente de 14 años con un diagnóstico de ataxia-telangiectasia, en remisión completa de linfoma no Hodgkin B de alto grado. Consultó en el Servicio de Urgencias Pediátricas por episodios de presíncope, y se observó, al ingresar, bloqueo auriculoventricular completo que evolucionó hacia asistolia, por lo que requirió la colocación de un marcapasos definitivo. Las dosis acumuladas de fármacos cardiotóxicos recibidos fueron de bajo riesgo. Sin embargo, es posible que esta enfermedad degenerativa crónica afecte con el tiempo al tejido de citoconducción. En la bibliografía revisada, no existen o se desconocen reportes previos de arritmias malignas en pacientes con ataxia-telangiectasia.

Ataxia-telangiectasia is a disorder characterized by cerebellar ataxia, telangiectasia, immunodeficiency, and increased predisposition to cancer susceptibility. Mutations in the ataxia telangiectasia mutated gene seem to play an important role in normal cell function and in cardiovascular remodeling. We report a case of a 14-year-old boy with ataxia-telangiectasia and high-grade B-non-Hodgkin lymphoma who remained in continuous complete remission after chemotherapy and who was admitted into our Emergency Room presenting with episodes of presyncope. At admission he presented a complete atrioventricular block that evolved into asystole and required placement of a pacemaker. Cumulative cardiotoxic drugs received were at low risk. However, it is possible that this chronic degenerative disease may affect the cardiac conduction system over time. In the reviewed literature there are no or unknown reports of ataxia-telangiectasia with malignant cardiac arrhythmias.

Surgical Implantation of Endocardial Lead for Adult Congenital Atrioventricular Block Combined with Obstructed Bilateral Subclavian Vein / 日本心臓血管外科学会雑誌

<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>

Temporary Transcutaneous Pacing in a Low Birth Weight Preterm Neonate with Congenital Complete Atrioventricular Block: A Case Report

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.

Clinical analysis of 73 pediatric patients with completely atrioventricular conduction block / 中华实用儿科临床杂志

Objective To explore the causes,clinical characteristics and prognosis of children′s completely at-rioventricular block(CAVB).Methods The clinical data of 73 patients with CAVB were analyzed retrospectively from January 2004 to December 201 3 at the Cardiology Department,Nanjing Children′s Hospital Affiliated to Nanjing Medi-cal University.Within those 73 patients,34 patients were male and the others were female,from 3 months old to 1 2.5 years old,the mean age of 6 years.Results There were 21 congenital CAVB patients and 52 acquired CAVB patients with myocarditis undergoing ventricular septal defect (VSD)closure operation.All congenital CAVB patients were re-fractory to drug therapy.Electrocardiogram and echocardiogram were performed in 1 9 cases without clinical symptoms during follow -up,but 2 cases had permanent pacemaker implanted.Among 27 fulminant myocarditis,Adams -Stokes attacks were found in 1 5 cases,3 cases had Adams -Stokes attack in 1 5 cases with sequelae of myocarditis,and 2 out of 6 cases undergoing VSD closure operation had Adams -Stokes attack,and other 4 cases without clinical symptoms were followed up periodically.The acquired CAVB patients were given energy composition and intravenous megavitamin C. The cases with fulminant myocarditis were given adrenal cortical hormone and intravenous gamma globulins simulta-neously.A total of 27 acquired CAVB patients were implanted temporary pacemaker and 5 with permanent pacemaker. Among 52 acquired CAVB patients,31 cases were cured,9 cases were improved,1 1 cases were ineffective,and 1 case died.Conclusions Most congenital CAVB children without clinical symptoms need clinical follow -ups.Myocarditis is a major cause of acquired CAVB.The CAVB prognosis caused by fulminant myocarditis may be related to antimely im-planting the temporary pacemaker timely.Permanent pacemaker should be implanted in patients who have no response to drug therapy with frequent Adams -Stokes or heart failure.

Pathological Substratum for a Case of Fulminant Myocarditis Treated with Extracorporeal Membrane Oxygenation and Subsequent Heart Transplantation

Fulminant myocarditis has been defined as the clinical manifestation of cardiac inflammation with rapid-onset heart failure and cardiogenic shock. We report on the case of a 23-yr-old woman with pathology-proven fulminant lymphocytic myocarditis presenting shock with elevated cardiac troponin I and ST segments in V1-2, following sustained ventricular tachycardia and a complete atrioventricular block. About 55 min of intensive cardio-pulmonary resuscitation, with extracorporeal membrane oxygenation support, bridged the patient to orthotopic heart transplantation. The explanted heart revealed diffuse lymphocytic infiltration and myocyte necrosis in all four cardiac chamber walls. Aggressive mechanical circulatory support may be an essential bridge for recovery or even transplantation in patients with fulminant myocarditis with shock.

Transient Complete Atrioventricular Block in a Preterm Neonate with Congenital Myotonic Dystrophy: Case Report

Congenital myotonic dystrophy (CMD) is an inherited neuromuscular disorder with cardiac rhythm abnormalities that may occur as a child grows. No report has described complete atrioventricular (AV) block detected in a neonate with CMD. We report a floppy infant of 31(+4) weeks gestation with complete AV block at birth, who was diagnosed with CMD by Southern analysis. She recovered from complete AV block 32 hr after temporary transcutaneous pacing was applied. To the best our knowledge, this is the first recorded case of a complete AV block accompanied by CMD during the neonatal period. When a newborn has a complete AV block, the physician should consider the possibility of the CMD and conduct a careful physical examination.

Bloqueo AV completo congénito: Revisión y presentación de un caso / Congenital complete av block: Review and case report

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.

A Case of Complete Heart Block in a Patient with Myasthenia Gravis Associated with Thymoma / 대한내과학회지

Myasthenia gravis is an autoimmune disorder characterized by antibodies against acetylcholine receptors in skeletal muscle. Myocardial involvement can present as myocarditis, ventricular tachycardia, heart failure and sudden death. However, advanced heart block is a very rare symptom. We report the case of a 69-year-old male who experienced dizziness and ptosis for one-month prior. He was diagnosed with myasthenia gravis and thymoma accompanied by complete atrioventricular block. The dizziness disappeared after implantation of a permanent pacemaker and the advanced heart block was resolved after surgical removal of the thymoma.

A Case of Pacemaker Implantation in Premature Newborn with Congenital Complete Atrioventricular Block

The congenital complete atrioventricular block(CCAVB) is a rare disease, which is the most serious complication of neonatal lupus erythematosus. Newborn with CCAVB are at risk of diminished cardiac output and the subsequent development of congestive heart failure. Transplacental steroid and beta-adrenergic agonist therapies are useful for the first and second degree atrioventricular block. But those therapies are usually not effective for complete atrioventricular block. If the fetus has a complete atrioventricular block, delivery should be considered unless other delivery contraindications. In this situation, early pacemaker implantation surgery can improve the survival rate of patients. We report one case of premature newborn with congenital complete atrioventricular block who is successfully recovered by pacemaker implantation.

Síndrome de Kearns-Sayre: Relato de caso / Síndrome de Kearns-Sayre: Reporte de un caso / Kearns-Sayre syndrome: Case report

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.

Autotransplantation of genetically-altered mesenchymal stem cells in treatment of complete heart block in porcine / 第二军医大学学报

Objective: To transplant the autologous mesenchymal stem cells (MSCs) carrying human hyperpolarization activated cyclic nucleoside gated channel 2 (hHCN2) gene into the His-bundle in porcine model of complete heart block (CHB), so as to assess the possibility of establishing autologous biological pacing. Methods: We constructed the recombinant adenovirus containing hHCN2 gene to transfect the porcine MSCs. After autotransplantation into the Hie-bundle in CHB model, the genetically-altered MSCs were tested for their ability to provide a stable and catecholamine-responsive heart rhythm. Histological and immunofluorescence analyses were also performed for the myocardium of the injection site. Results: Recombinant adenovirus pAd. hHCN2 was successfully constructed. Porcine MSCs were transfected by the adenovirus. After autotransplantation, transgenic MSCs significantly enhanced the heart rates in porcine CHB model compared with the control group (P < 0.01), and the cardiac rhythms in the transgenic MSC group were catecholamine responsive. Tissues obtained from the transplanted heart sites showed that the MSCs survived in the myocardium and overexpressed hHCN2 protein. Conclusion: Transplantation of autologous genetically-altered MSCs into the His-bundle in porcine CHB model can serve as a short-term catecholamine-responsive biological pacemaker.

Bloqueo cardiaco fetal completo: reporte de dos casos y revisión de la literatura / Fetal complete atrioventricular block: two case report and literature review

Presentamos 2 casos de embarazos controlados en nuestro servicio con el diagnóstico de bloqueo aurículo-ventricular fetal. Este es un tipo de arritmia poco frecuente, relacionado con la presencia de anticuerpos antiribonucleoproteínas (Ro y La). El manejo es expectante en la mayoría de los casos ya que no existe forma de revertir el bloqueo; en caso de evidenciar una descompensación hemodinámica fetal, se pueden administrar corticoides como medida terapéutica con un éxito limitado. No existe contraindicación del parto vaginal y el uso de pH de cuero cabelludo y oximetría de pulso parecen ser métodos adecuados para la evaluación de la condición fetal intraparto. Recomendamos el enfoque multidisciplinario en esta patología para evitar intervenciones innecesarias, anticipar los riesgos fetales y obtener un mejor pronóstico postnatal.

Here we report the perinatal outcome of two patients with fetal complete atrioventricular (AV) block. This is an uncommon disease, related to the presence of autoantibodies against ribonucleoproteins (Ro and La). Management should be expectant in most cases because a treatment to revert the AV block is not available; when fetal hemodynamic problems are detected corticosteroids can be used, but with limited effectiveness. Vaginal delivery is allowed; fetal scalp pH and pulse oximetry are appropriate for intrapartum fetal surveillance. We recommend a multi disciplinary approach to avoid unnecessary interventions, anticipate fetal risk and obtain a better perinatal outcome.

Complete Atrioventricular Block-Induced Torsade de Pointes, Manifested by Epilepsy

Complete atrioventricular (AV) block is frequently regarded as a cause of informed syncopal attacks, even though the escape rhythm is maintained. Torsade de pointes (TdP) may be a significant complication of AV block associated with QT prolongation. Here, we report the case of a 42-year-old female who was referred to our hospital due to recurrent seizure-like attacks while taking anti-convulsant drugs at a psychiatric hospital. TdP with a long QT interval (corrected QT = 0.591 seconds) was observed on an electrocardiogram (ECG) taken in the emergency department. The patient's drug history revealed olanzapine as the suspicious agent. Even after the medication was stopped, however, the QT interval remained within an abnormal range and multiple episodes of TdP and related seizure-like symptoms were found via ECG monitoring. A permanent pacemaker was thus implanted, and the ventricular rate was set at over 80 beats/min. There was no recurrence of tachyarrhythmia or other symptoms.

A case of complete atrioventricular block persisting for 5 days in a patient with variant angina / 대한내과학회지

Variant angina is more common in Asian people, including Koreans. Variant angina has a broad spectrum of clinical manifestations, from intermittent non-exertional pain to syncope and sudden cardiac death. Complete atrioventricular block is a major cause of syncope in patients with variant angina. The complete atrioventricular block related to variant angina is usually transient and easily terminated immediately after the restoration of coronary blood flow. We experienced a case of variant angina combined with prolonged complete atrioventricular block. The complete atrioventricular block persisted after the restoration of coronary blood flow, but normal sinus rhythm was restored spontaneously 5 days later. Here, we report this rare case

Bloqueo auriculoventricular paroxístico desencadenado por estímulo vagal con síncope recurrente / Paroxysmal Atrioventricular Block by the Triggering of Vagal Stimulus with Recurrent Syncopal Episodes

El bloqueo auriculoventricular completo (BAVC) paroxístico con ECG de reposo normal es una entidad poco frecuente. Se presenta el caso de una paciente de 34 años que cursaba el segundo mes de embarazo y consultó por cuadros sincopales recurrentes. Luego de ser evaluada con ECG, eco-Doppler cardíaco, tilt test, estudio electroencefalográfico, TAC y RM de cerebro se descartó cardiopatía de base y se interpretó que se trataba de síncope neurocardiogénico. Por persistencia de los síntomas se realizó un Holter de 5 días hasta que presentó un nuevo síncope y se arribó al diagnóstico de BAVC paroxístico. Se indicó un marcapasos definitivo VDD y seis meses después persistía asintomática.

Paroxysmal complete atrioventricular block (CAVB) with normal electrocardiogram at rest is uncommon. A 34 year-old two month-pregnant woman consulted for recurrent syncope. After screening assessment with electrocardiogram, cardiac echo-Doppler, tilt test, electroencephalogram, brain computerized tomography and magnetic resonance imaging, cardiopathy was ruled out and a diagnosis of neurocardiogenic syncope was made. As symptoms persisted, a 5 day-Holter monitoring was performed until she presented a new episode of syncope and paroxysmal CAVB was diagnosed. A definite VDD pacemaker was implanted and six months later she remained free of symptoms.

A case of KearnsSayre syndrome complicated with complete AV block / 대한내과학회지

KearnsSayre syndrome (KSS) is a mitochondrial DNA disorder characterized by the onset before age 20 years, progressive external opthalomoplegia, atypical retinal pigmentation and cardiac conduction disturbance. This report describes a 24-year-old woman who experienced syncope due to complete atrioventricular block complicated by KSS. At 12 years old, she was diagnosed KSS. The consecutive change of EKG shows typical progression of cardiac conduction disturbance of KSS. She was successfully treated with implantation of a pacemaker.

A Case of Hyperthyroidism with Complete Atrioventricular Block and Cardiac Arrest / 대한내분비학회지

The cardiovascular manifestations of hyperthyroidism are sinus tachycardia, paroxysmal supraventricular tachycardia, atrial fibrillation, atrial flutter, atrioventricular block, bundle branch block, angina pectoris, heart failure, and cardiomyopathy. Though complete atrioventricular block is rare, it is important to recognize it in clinical practice because of the possibility of cardiac arrest. We experienced a 47-year-old female patient who had hyperthyroidism with complete atrioventricular block and developed cardiac arrest despite the initiation of an antithyroid drug. We could resuscitate successfully and inserted a temporary pacemaker, but eventually a permanent pacemaker was needed to prevent the recurrence of cardiac arrest. We report a rare case of complete atrioventricular block and cardiac arrest associated with hyperthyroidism with the review of references.

A case of hyperthyroidism with complete atrioventricular block / 대한내과학회지

The importance of cardiovascular system involvement in hyperthyroidism has been recognized for many years. Many electrocardiographic abnormalities have been described in hyperthyroidism, including sinus tachycardia, atrial fibrillation, and atrial or ventricular extrasystoles and the increase in resting heart rate are characteristics of hyperthyroidism. In the middle aged or elderly patients, often with mild but prolonged elevation of plasma thyroid hormone, symptoms and sign of heart failure and complicating atrial fibrillation may eliminate the classical clinical feature and mask the more classical endocrine manifestation of the disease. Though rare in hyperthyroidism, the development of heart block is important to recognize in clinical practice. The mechanism of variable delayed conduction (from 1st degree to complete heart block) in hyperthyroidism is not well documented until now. We experienced 67-year-old female patient who complained of loss of body weight. After work-up with thyroid function test and electrocardiogrpahy, we found that she had hyperthyroidism with complete atrioventricular block. We started antithyroid drug and continued to monitor electrocardiophy. We are going to give the radioactive iodine theraphy and in the case of symptomatic atrioventricular block, we will make a insertion of pacemaker.