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Prolonged His-Q interval in chronic bifascicular block. Relation to impending complete heart block.

Although prolonged infra-His conduction time in bifascicular block is suspected of denoting trifascicular disease, adquate documentation is lacking concerning the correlation between lengthened His-Q interval (H-Q) and the risk of development of complete heart block (CHB). H-Q in conducted sinus beats in patients with bifascicular block associated with Mobitz II or intermittent CHB represents the approximation of maximal H-Q prolongation prior to onset of trifascicular block. To assess this relationship between prolongation of H-Q and trifascicular block, His bundle electrocardiography (HBE) was performed in 50 patients with chronic bifascicular block exhibiting Mobitz II block or transient CHB. Mobitz II or episodic CHB was shown in all patients: within two days prior to HBE in 45/50 patients; in 39/50 patients during HBE; and following HBE in five patients. In 49/50 patients H-Q was prolonged (greater than 55 msec) and in 47 this interval was substantially lengthened (65 msec or greater). Since marked H-Q prolongation in conducted sinus beats was documented in nearly all patients with bifascicular block associated with intermittent complete trifascicular block, we conclude that a considerably lengthened H-Q interval in bifascicular block is not only a usual prerequisite but strong evidence, for impending complete heart block.

Adult↗

Ro (SS-A) antibody and antigen in a patient with congenital complete heart block.

Congenital complete heart block is closely associated with the presence of anti-Ro (SS-A) autoantibodies. Quantitative solid-phase assays for Ro (SS-A) autoantigen and autoantibody have established the presence of Ro (SS-A) in cardiac tissues and have been used to evaluate an informative pedigree. The propositus we describe here had complete congenital heart block and showed anti-Ro (SS-A) binding of 13-fold less than his normal HLA-identical twin sister. Both had identical titers of antinuclear antibody. These data support the hypothesis that anti-Ro (SS-A) may be directly involved in the pathogenesis of congenital complete heart block.

Adult↗

Maternal autoantibodies and congenital heart block: no evidence for the existence of a unique heart block-associated anti-Ro/SS-A autoantibody profile.

One of the rare examples of the transfer of autoimmune disease from mother to (unborn) child is the neonatal lupus syndrome. This syndrome comprises the development of fetal heart disease (congenital heart block) or neonatal skin rash and is specifically associated with maternal anti-Ro/SS-A autoantibodies. Previous studies have suggested that especially maternal autoantibody reactivity against the 52 kDa protein of the Ro/SS-A antigen and/or against the La/SS-B antigen is responsible for the development of congenital heart block (CHB). To determine the CHB-associated antibody response in more detail, we analysed the presence of autoantibodies in sera from mothers of children with isolated heart block. All 14 mothers of children with congenital heart block were positive for anti-Ro/SS-A antibodies. Remarkably, their antibody profile, including recognition of different Ro/SS-A proteins and autoantibody levels against these proteins, did not differ from anti-Ro/SS-A positive mothers of healthy children. In contrast, all 8 anti-Ro/SS-A negative mothers had children with acquired heart block. We conclude from our data that maternal anti-Ro/SS-A antibodies are essential for CHB but that fine analysis of this autoantibody response does not predict the occurrence of CHB.

Adult↗

Ro and La antigens and maternal anti-La idiotype on the surface of myocardial fibres in congenital heart block.

Congenital complete heart block (CCHB) is a rare but potentially fatal disease of infants born to mothers with autoimmune disease where maternal autoantibodies to Ro (SS-A) are thought to cross the placenta and damage fetal cardiac tissue. We have adopted a novel approach to demonstrate the localization and specificity of maternal autoantibodies deposited in fetal heart. We raised an anti-idiotype against maternal anti-La antibodies, which reacted strongly with the surface immunoglobulin on the myocardial fibres from a CCHB heart but not a control fetal heart of the same age. Maternal immunoglobulin eluted from the CCHB heart reacted with La (SS-B) by ELISA. Using monoclonal and affinity-purified antibodies to La and affinity-purified anti-Ro antibodies, both antigens were identified on the surface of the fibres of the affected heart. Surface co-expression of immunoglobulin, complement and Class II antigen, consistent with a local immune response, was also found. This is the first definitive demonstration of Ro and La antigens and specific maternal anti-La antibody and idiotype on the surface of myocardial fibres in CCHB. It suggests that induction of Ro and La antigens on the surface of myocardial fibres during fetal development may be critical in the localization of the specific autoantibodies and subsequent evolution of congenital complete heart block.

Antibodies, Antinuclear↗

Temporary endoepicardial atrioventricular sequential pacing for complete heart block following complex surgery for congenital heart disease.

Complete heart block following intracardiac surgical repair for complex congenital heart disease is not uncommon. In the presence of ventricular dysfunction, ventricular pacing alone may not improve the cardiac output. We report the feasibility and efficacy of endoepicardial atrioventricular sequential pacing in a case of postoperative complete heart block.

Cardiac Pacing, Artificial↗

Mesothelioma of the atrioventricular node and congenital complete heart block.

Congenital complete heart block (CCHB) has heterogeneous etiologies. It may occur as an isolated entity with no associated congenital cardiac malformations and is difficult to diagnose even with modern technology. Mesothelioma of the atrioventricular (AV) node is a benign tumor that causes CCHB. The occurrence of this tumor is rare, and a definitive diagnosis of the entity is made only at autopsy. The conduction disturbance caused by the mesothelioma is potentially treatable and should be considered in the differential diagnosis of CCHB. We present a case report of a 38-year-old female with CCHB caused by such a tumor. Clinical diagnosis and treatment of CCHB are reviewed.

Adult↗

Treatment of heart block due to sarcoid heart disease.

The role of glucocorticosteroid therapy for myocardial sarcoidosis is not well defined. This report shows the effect of prednisone therapy on atrioventricular (AV) conduction in a patient with myocardial sarcoidosis and AV block. On three separate occasions AV block was documented prior to prednisone therapy. On the first two occasions the patient had first and second degree AV block which by His bundle electrogram initially was shown to be in the AV node. On the third occasion the patient developed complete heart block. On each occasion treatment with prednisone resulted in improved AV conduction. The results indicate that prednisone therapy can be beneficial in the treatment of AV block due to myocardial sarcoidosis.

Adult↗

Use of dopamine hydrochloride during general anesthesia in the treatment of advanced atrioventricular heart block in four foals.

Heart block is a relatively common arrhythmia in the adult horse. It may be a normal physiologic phenomenon or it may have pathologic implication. Four foals in which advanced heart block developed during anesthesia were unresponsive to atropine sulfate and supportive treatment alone. Resolution of the heart blocks was achieved after the addition of dopamine hydrochloride to the therapeutic regimen.

Anesthesia, General↗

Ro52, Ro60 and La IgG autoantibody levels and Ro52 IgG subclass profiles longitudinally throughout pregnancy in congenital heart block risk pregnancies.

Congenital heart block occurs in fetuses of Ro/SSA and La/SSB positive women. To investigate the stability of maternal autoantibody levels during pregnancy, we followed Ro52, Ro60 and La autoantibody IgG level variation and Ro52 subclass profiles longitudinally in selected congenital heart block risk pregnancies. Serum samples were obtained from 12 Ro/La positive women diagnosed with a systemic rheumatic disease and followed on average 60 months (range two to 84) which included 13 pregnancies. Seven children were affected by neonatal lupus, whereof four developed complete congenital heart block. Serum was also collected from the babies at birth. Ro52, Ro60 and La IgG as well as subclass antibodies were analysed by ELISA using recombinant antigens. Six Ro/La negative rheumatic patients were included as controls for antibody levels during pregnancy. Ro52, Ro60 and La IgG levels decreased progressively from early to late pregnancy, significantly for Ro52 and Ro60 (P < 0.01). No peaks or persistent elevation of antibody levels were noted in any of the CHB risk pregnancies. Ro52 IgG1 antibody levels were significantly higher than IgG2 (P < 0.01), IgG3 (P < 0.01) and IgG4 (P < 0.05) levels in the mothers during pregnancy. Ro52 IgG1 and IgG4 levels decreased significantly from early to late pregnancy (P = 0.02), while levels of IgG2 and IgG3 were low and the decrease was not significant. All IgG subclasses were transferred to the children. We conclude that maternal levels of Ro52, Ro60 and La autoantibodies tended rather to decrease than to increase during pregnancy.

Adult↗