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At least 577 records · Page 32Linked to original sources
PubMed1980

Heterotopic epithelial replacement (so-called "mesothelioma") of the atrioventricular node, congenital heart block, and sudden death.

Heterotopic epithelial replacement (so-called "mesothelioma") of the atrioventricular node is a rare cause of congenital heart block and sudden death; only about 50 cases have been reported in the literature in predominantly female patients ranging in age from infancy to 86 years. The lesion is virtually impossible to diagnose clinically and can be recognized at postmortem only by histologic examination of the atrioventricular conduction tissue. A unique case is reported since the patient, a young, asymptomatic woman, was chosen by chance as the subject of an electrophysiologic study of bradycardia 6 years before her sudden death. The morphologic (light- and electron-microscopic) findings of the atrioventricular node studied at autopsy were supportive of heterotopic epithelial origin of the lesion. The disputed histogenesis is briefly discussed.

Adolescent↗
PubMed1985

Complete heart block and bowel infarction secondary to rheumatoid disease.

A case of complete heart block secondary to rheumatoid involvement of the conduction system is presented. The same patient died after infarction of the colon as a consequence of arterial spasm superimposed upon pre-existing rheumatoid changes in the mesenteric arteries. The coexistence of rheumatoid involvement of the heart and of the bowel vasculature has not previously been reported.

Arthritis, Rheumatoid↗
PubMed1977

Association of maternal systemic lupus erythematosus with congenital complete heart block.

Infants born to mothers with disseminated lupus erythematosus occasionally have transient manifestations of the maternal disease. In six infants with congenital heart block born to mothers with systemic lupus erythematosus we postulated a causative relation. In one of the infants a post-mortem study of the conduction system suggested faulty embryonic development of the atrioventricular node with an abnormally thick annulus fibrosus and the effects of early inflammatory changes. Two of the infants had a cardiomyopathy and three, associated congenital heart disease.

Adult↗
PubMedDate not supplied

Maternal collagen vascular disease associated with fetal heart block and degenerative changes of the atrioventricular valves.

We report the case of a fetus diagnosed at 24 weeks' gestation with complete heart block associated with maternal collagen vascular disease and Sjögren's antibody. Serial fetal echocardiograms noted increased echogenicity along the tricuspid and mitral valves without insufficiency. Postnatally, severe tricuspid insufficiency occurred following placement of an endocardial pacing lead, and severe mitral insufficiency occurred acutely at 2.5 months of age. Both valves were noted at surgery to have fibrotic and calcific chordae tendineae that had ruptured from their papillary muscles. An inflammatory reaction within the fetal heart related to transplacental passage of Sjögren's antibody was likely responsible.

Adult↗
PubMed1978

Ventricular overdrive suppression of idioventricular rhythm in patients with complete heart block.

Idioventricular rate, QRS width, site of block and responses to ventricular overdrive pacing were studied in 29 patients with chronic complete heart block in an attempt to distinguish patients liable to syncopal attacks. Ten patients were asymptomatic, 5 gave a history of presyncope and 14 of syncope. Although the mean idioventricular rate of the 10 patients in the narrow QRS group (40.0 per min) was significantly faster than that of the 19 patients in the wide QRS group (35.5 per min; P less than 0.02), the rate did not distinguish symptomatic patients in either the narrow or the wide QRS groups. His bundle studies of the site of block were also unhelpful. Overdrive right ventricular pacing at increasing rates was used to assess ventricular escape times which were not significantly different in symptomatic and asymptomatic wide QRS patients. There was, however, a significant difference in the product of maximum ventricular escape time X overdrive cycle length between symptomatic and asymptomatic narrow QRS patients after 30 s overdrive (3.850+/-1.670 vs 1.070+/-0.475; P less than 0.01); 60 s overdrive (5.020+/-2.170 vs 1.240+/-0.515; P less than 0.01) and 120 s overdrive (6.040+/-2.900 vs 1.460+/-0.275; P less than 0.01) which may have clinical predictive values.

Adams-Stokes Syndrome↗
PubMed1985

Emphysema, cirrhosis, and heart block in a young patient with partial alpha 1 antitrypsin deficiency (PiMZ phenotype).

Severe lung disease and liver disease are not recognised features of the PiMZ phenotype, which is associated with alpha 1 antitrypsin deficiency. A 31 year old woman with this phenotype was found to have emphysema and complete heart block and showed evidence of hepatic cirrhosis, although her three sisters, all of whom had the same phenotype, were clinically normal. This case supports the possibility of a causal relation between the PiMZ phenotype and chronic lung and liver disease, but an association between alpha 1 antitrypsin deficiency and complete heart block could not be proved in this patient.

Adult↗
PubMed1982

Complete congenital heart block. Report of 35 cases.

Congenital complete atrioventricular block is uncommon, and the outlook is usually regarded as favourable. Thirty-five patients with congenital heart block are presented. There was no obvious sex difference and their ages ranged from 12 days to 85 years, though most were under the age of 20 years when first seen. Accompanying heart disease was noted in six, but presentation with symptoms in early infancy was a more serious risk factor in our experience. Permanent pacing was required in 21, three of whom were neonates. One of the 14 unpaced patients died unexpectedly at the age of 5 years. Long term supervision is necessary, as most will require permanent pacing before their 50th birthday.

Adolescent↗
PubMed1981

Symptomatic high grade heart block in Lyme disease.

We describe an unusual and remediable form of heart block which developed in the course of Lyme disease. The diagnosis of Lyme disease requires a careful clinical history pertaining to epidemiologic geography or a history of erythema chronicum migrans. In this case, a highly selective cardiac abnormality responded promptly to systemic steroids.

Adult↗
PubMed1984

Aortic valve endocarditis complicated by complete heart block.

A 34-year-old man with severe aortic incompetence caused by Streptococcus viridans developed severe central chest pain followed by complete heart block, multifocal ventricular extrasystoles, lengthening of the QTc and signs of cerebral emboli and pulmonary oedema. Early antibiotic therapy along with pacing, non-invasive investigations coupled with early surgery contributed significantly to the patient's survival.

Adult↗
PubMed1979

Congenital heart block and maternal systemic lupus erythematosus.

The association between infants with congenital heart block (CHB) and the presence or later development of maternal systemic lupus erythematosus or other connective-tissue disease (CTD) was reviewed in 67 cases. In 24 cases CHB was diagnosed at or before birth. Of nine necropsies on affected infants, seven showed endomyocardial fibrosis. The results suggest that one in three mothers who deliver babies with CHB have or will develop CTD. The association is probably explained by placental transfer of a maternal antibody. Awareness of the association may lead to prevention of the birth of children with CHB and better neonatal care of affected children.

Adolescent↗
PubMed2003

Cytokine polymorphisms and histologic expression in autopsy studies: contribution of TNF-alpha and TGF-beta 1 to the pathogenesis of autoimmune-associated congenital heart block.

Although Abs to SSA/Ro-SSB/La are necessary for the development of congenital heart block (CHB), the low frequency suggests that fetal factors are contributory. Because CHB involves a cascade from inflammation to scarring, polymorphisms of the TNF-alpha promoter region and codons 10 and 25 of the TGF-beta gene were evaluated in 88 children (40 CHB, 17 rash, 31 unaffected siblings) and 74 mothers from the Research Registry for Neonatal Lupus (NL). Cytokine expression was assessed in autopsy material from two fetuses with CHB. Significantly increased frequency of the -308A (high-producer) allele of TNF-alpha was observed in all NL groups compared with controls. In contrast, the TGF-beta polymorphism Leu(10) (associated with increased fibrosis) was significantly higher in CHB children (genotypic frequency 60%, allelic frequency 78%) than unaffected offspring (genotypic frequency 29%, p = 0.016; allelic frequency 56%, p = 0.011) and controls, while there were no significant differences between controls and other NL groups. For the TGF-beta polymorphism, Arg(25), there were no significant differences between NL groups and controls. In fetal CHB hearts, protein expression of TGF-beta, but not TNF-alpha, was demonstrated in septal regions, extracellularly in the fibrous matrix, and intracellularly in macrophage infiltrates. Age-matched fetal hearts from voluntary terminations expressed neither cytokine. TNF-alpha may be one of several factors that amplify susceptibility; however, the genetic studies, backed by the histological data, more convincingly link TGF-beta to the pathogenesis of CHB. This profibrosing cytokine and its secretion/activation circuitry may provide a novel direction for evaluating fetal factors in the development of a robust animal model of CHB as well as therapeutic strategies in humans.

Arginine↗
PubMed2000

Short- and long-term outcome of children with congenital complete heart block diagnosed in utero or as a newborn.

OBJECTIVES: Few data are available in the literature regarding the long-term outcome of newborns with congenital complete heart block (CHB). The aims of this retrospective study were to assess neonatal morbidity and mortality, incidences of dilated cardiomyopathy (DCM), and associated heart defects, and to establish prenatal and postnatal factors that might predict adverse outcome in children with CHB. DESIGN AND SETTING: The cohort includes 91 infants with CHB diagnosed in 5 tertiary centers in Finland between 1950 and 1998. PATIENTS: Maternal connective tissue disease was evident in 89% of the patients. At birth, the median gestational age was 37.1 weeks, and the median weight was 2969 g. Of the 91 infants, 60 (66%) were girls and 7 (8%) were twins. RESULTS: Incidences of perinatal morbidity and mortality were 58% and 7%, respectively. The total mortality of CHB was 16%; 11 of 15 (73%) died during the first 12 months. Cumulative probability of survival at 10 years old was 82%. Pacing as a newborn was indicated in 48 of 90 cases (53%), and 36 received pacemakers at older ages. Cardiac defects not causally related to CHB were found in 38 of 90 patients (42%), of whom 22 were operated on. DCM was found in 21 (23%), of whom 13 died. During the follow-up, among 75 survivors with a median age of 9 years, 54 (72%) are free from symptoms. Poor outcome defined as clinically or pathologically evident congestive DCM was associated with intrauterine hydrops, low fetal and neonatal heart rate, low birth weight, male sex, and neonatal problems attributable to prematurity or neonatal lupus. CONCLUSIONS: Despite early pacing, CHB carries high mortality during the first 12 months of life. High incidences of DCM and associated heart defects indicate close echocardiographic monitoring of all children with CHB.

Cardiac Pacing, Artificial↗
PubMed1994

Complete heart block induced by general anesthesia in a patient with suspected conduction system defect: a case report.

We report a rare incident of complete heart block occurring after induction of general anesthesia in an apparently fit patient. An external temporary pacemarker was first applied, which was then replaced by isoproterenol infusion, but permanent pacing was required postoperatively. The role of anesthesia as a precipitating factor was discussed. It is suggested that surgical patients suspicious of conduction system defect should be under continuous hemodynamic monitoring with temporary pacing at hand during perioperative period.

Anesthesia, General↗
PubMed1964

SURGICAL HEART BLOCK.

Explore the source record for details and available documents.

Cardiac Surgical Procedures↗
PubMed2006

Left ventricular epicardial VVI pacing for a congenital complete heart block with severe myocardial dysfunction: shall epicardial pacing wires be positioned left?

We present the case of a patient with a congenital complete heart block (CHB) who developed a severe dilated hypokinetic cardiomyopathy whilst paced with a right-sided epicardial wire inserted by an anterior approach. She dramatically and rapidly improved both clinically and echocardiographically, once a single pacing wire was inserted on the left ventricular (LV) wall towards the apex by left thoracotomy. Based upon recent literature, attention is drawn to the fact that left-inserted epicardial pacing wires should probably be considered for pediatric patients in whom atrio-ventricular or inter-ventricular pacing might not be possible to achieve, or else as a consistent approach for small patients requiring VVI epicardial pacing.

Cardiac Pacing, Artificial↗