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Concentration of autoantibodies to native 60-kd Ro/SS-A and denatured 52-kd Ro/SS-A in eluates from the heart of a child who died with congenital complete heart block.

OBJECTIVE: To determine the serologic specificity of acid eluates from tissues of a child who died with congenital complete heart block (CCHB). METHODS: Tissues were extracted, acid eluted, and the IgG and antibody titers determined on the eluates by enzyme-linked immunosorbent assay. RESULTS: Antibodies to native 60-kd and denatured 52-kd Ro/SS-A were found to be enriched only in the heart eluate, and not in the eluates from brain, kidney, and skin. CONCLUSION: These findings indicate a major role for anti-native 60-kd Ro/SS-A in the immunopathogenesis of CCHB.

Adult↗

Chronic progressive external ophthalmoplegia, pigmentary retinopathy, and heart block (Kearns-Sayre syndrome). Report of a case.

Chronic progressive external ophthalmoplegia associated with a pigmentary retinopathy and heart block was described by Kearns & Sayre in 1958. They suggested that the triad represented a syndrome. The cases recorded since then are reviewed and summarised, and several common features are noted. Little attention has previously been paid to the retinopathy. A similar case is presented, and the pigment changes, retinal function, annd fluorescein angiography are examined. There is no clinical evidence for the diagnosis of classical retinitis pigmentosa. The prognosis and aetiology of the condition are discussed.

Adolescent↗

[Tolerance to cardiotoxic effect of strophanthane K in experimental total heart block and electrocardiostimulation].

Tolerance to the cardiotoxic effect of strophanthin K was studied in narcotized dogs with experimental complete heart block (CHB) and ventricular electrostimulation. A threshold to the toxic arrhythmogenic effect of strophanthin K was significantly reduced as compared to that in the control group. As the electrocardiostimulation (ECS) frequency increased, the minimum arrhythmogenic dose of strophanthin K showed a growth, while not reaching a level for the sinus rhythm. It is concluded that the basic mechanisms of the arrhythmogenic effect of strophanthin K under the conditions studied are the trigger activity in dogs with ECS and the increased peacemaker's activity in dogs with CHB.

Animals↗

Implantation of a permanent transvenous pacing catheter in a horse with complete heart block and syncope.

A permanent, transvenous, atrioventricular, sequential pacing system was implanted successfully in a 7-year-old Quarter Horse gelding with complete heart block. The transvenous atrial and ventricular electrodes were placed in their respective positions in the heart, using real-time, 2-dimensional echocardiography. The horse was able to return successfully to competition as a show horse.

Animals↗

Congenital heart block immunogenetics. Evidence of an additional role of HLA class III antigens and independence of Ro autoantibodies.

Congenital complete heart block (CCHB) occurs in the offspring of mothers who have the HLA-A1;B8; DR3 haplotype and Ro (SS-A) autoantibodies. It has been shown that the presence of HLA-DR3 in mothers may facilitate Ro synthesis, but may not, by itself, be sufficient to induce CCHB in the offspring. However, maternal DR3 and Ro antibody seem to be independent factors associated with CCHB. Other HLA antigens, including class III (complement) genes, may also be necessary to induce CCHB in newborns since A1;B8; DR3 haplotypes, together with BfS and/or C4AQ0B1, are increased in Ro+ mothers of infants with CCHB compared with both controls and with Ro+ mothers whose offspring do not have CCHB. On the other hand, DR3 genes in a different HLA haplotype (i.e., B18; BfF1;DR3) are nonpathogenetic; this latter finding may be due to a DR3 gene's intrinsic difference or to the influence of neighboring genes. Also, a trend toward DR3 bias transmission is observed in DR3+ mothers who are also Ro+; fetal DR3 might protect the fetus against in utero death when Ro antibodies are present.

Antibodies, Antinuclear↗

Baroreflex control of coronary conductance in normotensive and renal hypertensive conscious dogs with complete heart block.

1. The autonomic and local control of the circumflex coronary bed evoked through acute rises in aortic pressure (aortic balloon inflation) was analysed in conscious, normotensive and hypertensive dogs in complete heart block with ventricles paced at 100 beats/min. 2. In normotensive dogs there was an initial rise (6-12 s) in circumflex conductance preceding a fall in conductance as aortic pressure increased and was sustained. The initial rise, but not the later fall, in circumflex conductance was partly due to a cholinoreceptor and partly due to alpha-adrenoreceptor mechanisms. Local constrictor mechanisms were responsible for the later fall in conductance. 3. The initial cholinoreceptor component of the rise in circumflex conductance is initiated through arterial baroreflex mechanisms. 4. The initial autonomic mechanisms regulating circumflex conductance appear to be absent in renal hypertension. 5. The absence of the transient autonomic rise in circumflex flow and conductance in response to rapid elevations in aortic pressure in certain forms of hypertension, e.g; during tachyarrhythmias and behavioural disturbances, may result in myocardial ischaemia when it is least appropriate.

Animals↗

A case of fetal complete heart block recorded by magnetocardiography, ultrasonography and direct fetal electrocardiography.

Fetal magnetocardiograms (FMCGs) were recorded in a case of fetal complete heart block (CHB) from the 30th to the 37th week of gestation using the multichannel SQUID system (Hitachi, Japan). M-mode ultrasonography and direct fetal electrocardiography using needle electrodes revealed fetal CHB. We identified independent fetal P-waves and QRS complexes in the FMCG recorded in the 32nd week of gestation when the fetal atriums were close to the FMCG sensor. We also recorded FMCG P-waves in the 37th week of gestation when the fetal heart was larger. Fetal heart position and size are important for obtaining a useful FMCG. To establish FMCG as a diagnostic tool of fetal arrhythmia, comparative studies with FECG are needed.

Adult↗

Carotid sinus hypersensitivity and complete heart block in Reiter's syndrome.

We describe a 42-year-old man with Reiter's syndrome who developed complete heart block only 5 weeks after the onset of his illness. He was also noted to have carotid sinus hypersensitivity and evidence of sinoatrial disease. There were no clinical signs or echocardiographic findings to suggest involvement of the aortic root or aortic valve. His arrhythmias were treated successfully with a physiological pacemaker. The combination of sinoatrial and atrioventricular node disease suggests that the inflammatory process in Reiter's syndrome can produce widespread involvement of the conducting system.

Adult↗

[A case of congenital complete heart block in a mother with anti-52 kD SS-A/Ro antibodies].

We report a case of congenital complete heart block (CCHB). A 38-year-old woman was admitted our hospital because of fetal bradycardia at 21 weeks 3 days of gestational age. She had no symptom of collagen disease. On admission, laboratory data showed positive anti-nuclear antibodies, anti-SS-A/Ro antibodies and anti-52 kD SS-A/Ro antibodies. But anti-60 kD SS-A/Ro and anti-SS-B/La antibodies were negative. Consequently anti-52 kD SS-A/Ro antibodies positive woman had an infant with CCHB. The baby was equipped with pacemaker at the age of 2 months. This report suggests that anti-52 kD SS-A/Ro antibodies may play an important role in the development of CCHB.

Adult↗

Complete heart block following a single dose of trazodone.

Forty minutes after receiving a single starting dose of trazodone, a patient developed complete heart block. The case illustrates that, despite the results of earlier studies, trazodone's effect on cardiac conduction may be severe in individuals at risk for conduction delay.

Aged↗