Congenital complete heart block can occur in sibs with and without associated congenital heart defects.' This has been associated with different histological changes, but the ultimate cause has remained obscure. Recently there have been reports of an association between congenital heart block and maternal systemic lupus erythematosus.24 This report concerns two sisters with congenital complete ...

Twenty patients with congenital complete heart block (CHB) were monitored with ECG tape recordings while awake and asleep. Episodes of marked ventricular slowing during sleep (R-R greater than 3000 msec., i.e. less than 20 beats/min) were noted in 35% (7/20). Most of these sudden R-R prolongations were 2:1 or 3:1 exit block of the junctional focus. Atrial and ventricular rate changes were mostl...

normal 0 false false false en-us x-none fa background : fahr's disease is a rare neurodegenerative disorder of unknown cause characterized by idiopathic basal ganglia calcification that is associated with neuropsychiatric and cognitive impairment. no case of fahr's disease with associated cardiac conduction disease has been described in the literature to date. the objective of this case report ...

Ellis-van Creveld syndrome is transmitted as an autosomal recessive trait. This syndrome is accompanied in 60% of cases with congenital heart disease, mostly single atrium or large ASD. Patients are mostly symptomatic, but in this rare case despite 68 years of age, the patient was free of symptoms except for complete heart block for which pacemaker was inserted