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At least 253 records · Page 14Linked to original sources
PubMed1985

Pacemaker management for acute onset of heart block in childhood.

Four children aged 2 to 15 years are described who had Stokes-Adams syndrome as the primary cardiac manifestation of complete heart block. All had mild, nonspecific antecedent symptoms without signs of congestive heart failure. Viral neutralizing antibody titers (coxsackievirus B1 and B2) rose significantly in two patients. Electrocardiography demonstrated complete heart block associated with one or more of the following: right bundle branch block, left anterior hemiblock, left bundle branch block, intraventricular conduction delay, and ventricular standstill. In addition, second-degree heart block was seen during the initial or resolving phases of illness. To prevent further life-threatening episodes of Stokes-Adams syndrome, temporary transvenous pacemakers were placed. Two patients with persisting complete heart block required permanent pacemakers. At last examination, the remaining two patients had sinus rhythm and normal conduction. Nonsurgical acquired complete heart block is unusual in childhood, and may be the only manifestation of myocarditis. Extreme bradycardia, which causes syncope, requires immediate temporary cardiac pacing; if the bradycardia persists, a permanent pacemaker should be implanted.

Acute Disease↗
PubMed1997

Perioperative management of children with third degree heart block undergoing pacemaker placement: a ten year review.

It has been suggested that children with third degree heart block require insertion of a temporary pacemaker prior to general anaesthesia. This recommendation needs to be reevaluated with the availability of noninvasive transcutaneous cardiac pacing. We undertook a retrospective ten-year chart review of anaesthesia in children with third degree heart block undergoing pacemaker insertion or revision. Forty-eight children with complete heart block underwent seventy anaesthetics of which fifty three were in children without pacemakers or with nonfunctioning pacemakers. One child had a temporary pacemaker placed preoperatively following asystole in the emergency room. In children who were not being paced, 60% had baseline heart rates less than 60 bpm. Complications seen in this study, including hypotension, would not have been prevented by temporary pacemaker placement. We conclude that there is no benefit to routine preoperative temporary pacing in children with third degree heart block.

Adolescent↗
PubMed1981

Maternal systemic lupus erythematosus associated with fetal congenital heart block. A case report.

Congenital heart block (CHB) is a rare cause of fetal bradycardia and, if misinterpreted as fetal distress, may lead to detrimental obstetric intervention. There is a definite association between systemic lupus erythematosus (SLE), as well as other connective tissue disorders in the mother and CHB in her offspring. A case of CHB in the child of a mother with asymptomatic SLE is reported and a useful diagnostic investigation, the sonar-atropine test, is described.

Adult↗
PubMed2006

Recurrent congenital heart block in neonatal lupus.

Congenital heart block (CHB) is the main complication of neonatal lupus (NL) and is strongly associated with the presence of anti-SSA/Ro and anti-SSB/La antibodies. The recurrence of CHB in subsequent pregnancies in mothers with these antibodies is uncommon, occurring in approximately 15% of cases. We describe here a case of recurrent CHB in a previously asymptomatic mother with Sjögren syndrome and discuss the current strategies for the prevention and treatment of CHB in NL.

Adult↗
PubMed1996

Placental and fetal cardiac laminin are targets for cross-reacting autoantibodies from mothers of children with congenital heart block.

The association of congenital heart block (CHB) with maternal autoantibodies to the Ro and La ribonucleoprotein antigens may be due to cross-reactions between maternal anti-La antibodies and fetal cardiac specific antigens. One of the major components of cardiac myocytes, laminin, is accessible for binding by maternal autoantibodies and we have previously reported cross-reactivity of mouse laminin with anti-La antibodies affinity purified from the sera of patients with primary Sjögren's syndrome. Affinity purified anti-La antibodies from ten women who had at some time given birth to a child with CHB were examined for cross-reactivity with human placental laminin, which shares structural similarities with cardiac laminin. All ten anti-La antibodies bound to the surface of cryosections of normal full term placental trophoblasts. Binding could be inhibited by pre-incubation of antibodies with either La or placental laminin. Eight anti-La antibodies also reacted with placental laminin by ELISA and La inhibited up to 82% of binding to laminin while laminin inhibited up to 85% of binding to La in a dose dependent manner. Eight anti-La antibodies also bound to the surface of fetal cardiac myocytes at 10.3 weeks of gestation and five showed lower levels of reactivity with the surface of fetal cardiac myocytes at 16.5 weeks of gestation. None showed any surface staining of normal adult heart. These data confirm the cross-reactivity of anti-La antibodies with laminin and may support a placental role in preventing the majority of potentially pathogenic antibodies from reaching the fetal circulation.

Autoantibodies↗
PubMed1997

Monozygotic twins discordant for congenital complete heart block.

OBJECTIVE: Isolated congenital complete heart block (CCHB) occurs in 1/20,000 live births. More than 85% of mothers giving birth to affected infants are anti-Ro antibody positive, but only approximately 1% of babies with anti-Ro-positive mothers develop CCHB. We studied 2 sets of monozygotic twins discordant for CCHB. METHODS: Monozygosity was determined using placental examination and DNA microsatellite analysis. HLA typing was performed. Autoantibody studies were performed using counterimmunoelectrophoresis, immunoblotting, Ro 52 and Ro 60 enzyme-linked immunosorbent assay (ELISA), and indirect immunofluorescence (IIF) on Ro 60- and Ro 52-transfected HEp-2 cells. RESULTS: Both sets of twins were monozygotic. They had similar birth weights. Twin 2 in the second set required a pacemaker at age 2 months. Both mothers were positive for anti-Ro 52 and anti-Ro 60 antibody, and neither had anti-La antibody on immunoblot. One set of twins was studied at birth. Similar titers of anti-Ro 52 and anti-Ro 60 antibody were found by IIF and ELISA. CONCLUSION: There are no previous well-documented reports of monozygotic twins discordant for CCHB. These cases demonstrate that there is still discordance in the development of CCHB despite identical genetics and environmental exposure to anti-Ro antibody.

Adult↗
PubMed1997

Congenital heart block: HLA differences between affected children and healthy siblings in four Finnish families.

Congenital heart block without intracardiac anatomic malformations is a potentially lethal disease affecting children and newborns. The mother often has an autoimmune disorder with autoantibodies against SS-A/Ro and/or SS-B/La antigens. However, only a minority of the children of these mothers develop complete heart block. It is believed that the maternal antibodies are pathogenic, but other immunological mechanisms such as cell-mediated injury cannot be excluded. Maternal cells may recognize fetal antigens adjacent to fetal HLA, and thus some children may be more susceptible to heart block than others, depending on their HLA genetics. The purpose of this study was to evaluate whether there are HLA differences between children with heart block and their healthy siblings. Six affected children in four families and their siblings were studied. MHC class I were typed serologically and class II and some non-HLA alleles were typed by DNA techniques. DQB1*03/04 were seen more often in the affected children than in the siblings. Some other differences were also seen in the other antigens of the MHC area.

Alleles↗
PubMed1984

Complete heart block in HLA B27 associated disease. Electrophysiological and clinical characteristics.

A genetic predisposition associated with HLA B27 for developing complete heart block with or without clinical or radiological signs of associated rheumatic disease has recently been found. In this electrophysiological study of 12 patients with spontaneous complete heart block and HLA B27 associated disease, of whom eight had ankylosing spondylitis, 10 had suprahisian second or third degree atrioventricular block (eight spontaneously and two during atrial pacing at rates below 90 impulses per minute) and one infrahisian block. One patient with narrow QRS complexes during complete heart block three months earlier had normal findings. Three patients also had sinus node malfunction and six had fascicular or bundle branch block. In HLA B27 associated disease the atrioventricular block seems to be preferentially located in the atrioventricular node, although the conduction system may be widely affected. The findings in this study indicate a further cause of high degree atrioventricular block with a predominantly suprahisian location in addition to acute inferior myocardial infarction, digitalis intoxication, and "congenital" heart block.

Adult↗
PubMed1985

Anti-Ro (SS-A) antibodies and congenital complete heart block.

Maternal anti-Ro (SS-A) antibodies have been implicated in the pathogenesis of congenital complete heart block. When congenital complete heart block occurs, SS-A antibodies are almost always present in maternal and fetal serum, although not all infants of SS-A antibody positive mothers are affected. We describe a situation in which antibodies to SS-A were present in high titre in both maternal and fetal serum, although there was no evidence of heart block in the infant. This is the first report of a normal infant in whom maternally derived SS-A antibodies have been documented in high titre.

Adult↗
PubMed1980

Postural heart block.

A patient presented with orthostatic dizziness and syncope caused by postural heart block. When the patient was supine, atrioventricular conduction was normal and he was asymptomatic; when he was standing he developed second degree type II block and symptoms. The left bundle-branch block on his electrocardiogram and intracardiac electrophysiological study findings suggest that this heart block occurred distal to the His bundle. Orthostatic symptoms are usually presumed to be secondary to an inappropriate distribution of intravascular volume or to autonomic nervous system abnormalities. As shown in this patient, these symptoms may be the result of orthostatic heart block. Ambulatory monitoring may be useful in patients with orthostatic neurological symptoms, particularly when conduction abnormalities are present on the electrocardiogram.

Dizziness↗
PubMed1985

Survival and follow-up after pacemaker implantation: a comparison of patients with sick sinus syndrome, complete heart block, and atrial fibrillation.

The number of patients receiving cardiac pacemakers for sick sinus syndrome (SSS) has increased considerably in recent years. The literature has suggested that patients with sick sinus syndrome have a shorter life expectancy with pacemaker therapy than patients with total heart block or atrial fibrillation. We studied the survival rate of 1,049 patients with complete heart block, 592 with sick sinus syndrome and 447 with atrial fibrillation. After 10 years we found a survival rate of 54.5% for patients paced for SSS, 34.4% for those with complete heart block, and 24.7% for those with atrial fibrillation (statistical significance: SSS--heart block: p less than 0.05; SSS--atrial fibrillation: p less than 0.01; heart block--atrial fibrillation: NS). Considering the calculated survival rates of a comparable normal population (i.e., 56.5%; 41.2%; 47.8%), the differences in survival expectancy are even more pronounced (SSS-normal: NS; heart block-normal p less than 0.05; atrial fibrillation-normal: p less than 0.05). For patients with sick sinus syndrome, the life expectancy parallels that of the general population, while that of patients with complete heart block or atrial fibrillation have a life expectancy that is considerably lower.

Adolescent↗
PubMed1992

Progression of first degree heart block to high-grade second degree block during spinal anaesthesia.

A case is presented in which a patient with pre-existing first degree heart block developed high-grade second degree heart block during spinal anaesthesia. Progression of the block was associated with blockade of cardiac sympathetic neurons induced by spinal anaesthesia. This suggests that patients with pre-existing heart block may be at increased risk for development of higher grade block during spinal anaesthesia.

Adult↗
PubMed1978

Trifascicular and complete heart block with anterior myocardial infarction. Electrocardiographic patterns and long-term medical management.

A 45-year-old man exhibited various patterns of delay in trifascicular conduction, culminating in complete heart block during acute myocardial infarction. A temporary pacemaker was utilized for the short period of heart block. Delay in ventricular conduction subsided, and the patient refused implantation of a permanent pacemaker. Ventricular irritability, which was detected eight months later, was suppressed with administration of digitalis, guided by testing with acetylstrophanthidin. Neither heart block nor delay in ventricular conduction recurred during 43 months after the infarction. In certain patients with heart block complicating anterior myocardial infarction, long-term prognosis may be dictated by coronary and myocardial residual function, rather than by the hazard of recurrent heart block.

Bundle-Branch Block↗
PubMed2005

Autoantibodies against the serotoninergic 5-HT4 receptor and congenital heart block: a reassessment.

The specificity of autoantibodies against the serotoninergic 5-HT4 receptor in congenital heart block has led to conflicting observations. In order to clarify the situation, a collaborative effort was undertaken to discover the reasons for these discrepancies and to reassess the importance of such autoantibodies by making use of the Research Registry for Neonatal Lupus. Sera from 128 patients (101 anti-SSA/Ro52 positive mothers among which 74 have children with congenital heart block (CHB), 9 anti-SSA/Ro52 negative patients of which 1 had a child with heart block and 18 healthy donors) were assessed in a single blind test using an ELISA coated with a 5-HT4 receptor-derived peptide. Discrepancies between previous observations in our two groups could be ascribed to small differences in the set up of the assay. Of the 75 sera from mothers of children with CHB, 12 were reactive with the 5-HT4 peptide. Four sera among which three were from 35 Ro52 negative mothers without affected children as well as 2 in the 18 controls were positive. Interestingly, in 1 mother with an isolated child with CHB but who had no detectable anti-SSA/Ro52 antibodies and 1 mother with a child with a structural heart block and no detectable antibodies to any component of SSA/Ro, reactivity with the 5-HT4 receptor was noted. While 5-HT4 receptor autoantibodies do not have the predictive value of anti-Ro52 autoantibodies, the presence of these antibodies in a minor subset of mothers whose children have CHB suggests an additional risk factor which may contribute to the pathogenesis of disease.

Amino Acid Sequence↗
PubMed1979

Congenitally complete heart block with an interrupted Mönckeberg sling.

The conducting tissues are described in a case of congenitally corrected transposition. The patient was known to have suffered from congenital complete atrioventricular block. In the heart, both anterior and posterior nodes and bundles were found to coexist. The two bundles shared a common bifurcation. Neither of the two nodes connected with their respective bundles. The different morphology of the two sites of fibrous interruption may be explained by differences in embryonic development.

Atrioventricular Node↗
PubMed1983

Complete heart block in rheumatoid arthritis.

We report 8 cases of complete heart block (CHB) occurring in patients with rheumatoid arthritis and review 20 similar patients previously reported. Complete heart block occurs generally in patients with established erosive nodular rheumatoid disease. It usually appears to be sudden and permanent, but progression from minor conduction delays is not uncommon. The characteristic histopathological finding is a rheumatoid granuloma in or near the AV node or bundle of His. If syncope or Stokes-Adams attacks occur, the treatment of choice is the insertion of a permanent pacemaker. The prognosis is good provided no other cardiac lesions occur, whether pericardial, valvular, or myocardial.

Adult↗
PubMed1983

[Congenital heart block in a newborn infant born to a mother with lupus erythematosus].

We report a case of a newborn with a congenital heart-block. Clinical picture began with bradycardia and heart failure from birth. The mother had a systemic lupus erythematosus in remission during pregnancy suffering a reactivation after delivery. This report deals about the greater incidence of congenital heart-block in the lupus-mother's newborns and we expose some etiopathogenic hypothesis. Also we point-out the easy clinical and electrocardiographic diagnosis that shows a third degree heart-block with atrioventricular dissociation and atrial frequency two or three times greater than ventricular. It's established the differential diagnosis among neonatal bradycardias and congenital heart-blocks. The treatment with isoproterenol was ineffective. A pacemaker was need.

Female↗