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

22. Harrison TR: Harrison's Principles of Internal Medicine, ed 8, edited by Thorn GS. New York, McGraw-Hill, 1977, p 429 23. Hogg GR: Congenital acute lupus erythematosis associated with subendocardial fibroelastosis. Am J Clin Pathol 28: 648, 1957 24. Brigden W, Bywaters EGL, Lassof MH, Ross IP: The heart in systemic lupus erythematosus. Br Heart J 22: 1, 1960 25. Griffith GC, Vural IL: Acute...

A family is reported in which two siblings had congenital complete heart block with resultant congestive heart failure, the father and paternal grandfather show adult-onset conduction defects, and the mother has systemic lupus erythematosis. The interaction of heredity and environment is discussed in this context. A review of the literature on familial complete heart block suggests that so-call...