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We describe a 26-year-old man with a predominantly distal myopathy and complete heart block. A muscle biopsy specimen demonstrated numerous vacuoles, which, by electron microscopy, contained membranous whorls. Filamentous inclusions characteristic of inclusion body myositis were not seen. It is important to be aware that life-threatening cardiac disease may occur in this setting.
A newborn male presenting with congenital complete heart block was found to have low levels of free plasma carnitine. Among the many complications of carnitine deficiency is a structural disruption of myocardial tissue. A deficiency of carnitine may be causally related to the presence of cardiac dysrhythmias in this patient on the basis of mechanical disturbance to the cardiac conducting system.
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Congenital quadricuspid aortic valve is very uncommon, and is often associated with other cardiac disorders, such as patent ductus, ventricular septal defect, pulmonary stenosis, mitral valve malformation, hypertrophic cardiomyopathy and coronary abnormalities. We report a patient with congenital quadricuspid aortic valve associated with congenital complete heart block. To our knowledge, this association has not been reported so far.
We report a case of complete fetal heart block in a 35-year-old Chinese woman known to be positive for anti-SSA/Ro and anti-SSB/La antibodies. She had fetal hydrops leading to intrauterine death in her first pregnancy Prophylactic intravenous immunoglobulin, given at 14 and 18 weeks' gestation, as well as oral dexamethasone, commenced at 24 weeks' gestation, allowed continuation of the pregnancy until 34 completed weeks of gestation. An external pacemaker was inserted in the baby on the first day of life. Two-and-a-half months later, a permanent pacemaker was inserted.
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In this work we demonstrated that IgG present in the sera of patients with congenital heart block (CHB) and their mothers could bind and activate beta adrenoceptors and muscarinic cholinergic receptors of neonatal heart. These antibodies were able to inhibit in a noncompetitive manner the binding of [3H]QNB and [3H]CGP to muscarinic cholinergic and beta adrenoceptors of purified neonatal rat myocardial membranes, respectively. Moreover, IgG from children with CHB and their mothers could modify biological effects mediated by these neurotransmitter receptors, i.e., heart contractility and cAMP production. Neither binding nor biological effects were obtained using adult instead of neonatal rat atria. Both reactivities (adrenergic and cholinergic) were independent of Ro/SS-A and La/SS-B antibodies and were absent in the sera of normal women of childbearing age and of normal children. It could be concluded that antibodies against cardiac neurotransmitter receptors may be another serum factor (or factors) to be considered in the pathophysiology of the development of CHB.
The authors describe the occurrence of type III heart block in a patient undergoing a transjugular intrahepatic portosystemic shunt recanalization with use of the AngioJet thrombectomy system.
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A patient whose slow-relaxing tendon reflexes returned to normal after cardiac pacing for complete heart block is reported. Since the patient was euthyroid, these observations add further weight to previous suggestions that intrinsic catecholaminergic mechanisms, as well as thyroid status, may play a role in the pathogenesis of slow-relaxing reflexes.
Radiation therapy is the current standard therapy for several malignant disorders, including Hodgkin's disease. Cardiac complications, pericardial disease in particular, may develop long after the treatment. However, conduction disorders have rarely been described. We report a patient with the Wolff-Parkinson-White syndrome who developed complete heart block 16 years after mediastinal radiation therapy.
BACKGROUND: Progressive familial heart block type I (PF-HBI) is a dominantly inherited cardiac bundle-branch conduction disorder that has been traced through nine generations of a large South African kindred. Similar conduction disorders have been reported elsewhere; however, the cause of these diseases is unknown. The aim of the present study was to determine by linkage analysis the approximate chromosomal position of the gene causing PFHBI, thereby allowing family-based diagnosis and the development of positional cloning strategies to identify the causative gene. METHODS AND RESULTS: Eighty-six members of three pedigrees, 39 members of which were affected with PFHBI, were genotyped at four linked polymorphic marker loci mapped to chromosome 19, bands q13.2-q13.3 (chromosome 19q13.2-13.3). Maximum two-point logarithm of the odds scores (which represent the logarithm of the odds ratio of detecting linkage versus nonlinkage) generated were 6.49 (theta = 0) for the kallikrein locus, 5.72 (theta = 0.01) for the myotonic dystrophy locus, 3.44 (theta = 0) for the creatine kinase muscle-type locus and 4.51 (theta = 0.10) for the apolipoprotein C2 locus. The maximum multipoint logarithm of the odds score was 11.6, with the 90% support interval positioning the PFHBI locus within a 10 cM distance centering on the kallikrein 1 locus. CONCLUSIONS: The gene for PFHBI maps to an area of approximately 10 cM on chromosome 19q13.2-13.3. There are several candidate genes in this interval; although a recombination event ruled out the myotonic dystrophy locus from direct involvement with PFHBI, the proximity of these two loci may be relevant to the observed cardiac abnormalities of myotonic dystrophy. The results provide a means of DNA-based diagnosis in the families studied and a foundation for cloning studies to identify the causative gene.
A case of digoxin overdose was described. Despite vigorous gastric lavage, the serum digoxin which was 0.8 ng/ml on admission rose to 7 ng/ml four hours later. The main side-effects were symptoms of nausea and anorexia and conduction defects including complete atrioventricular dissociation requiring pacemaker insertion. Opportunity was taken to study the site of heart block with His bundle electrography during the conduction disturbances. This showed that the site of block was situated above the His-Purkinje system in the atrioventricular (A-V) node.