Congenital Complete Heart Block and Connective-Tissue Disease

Abstract

In 1901 the first description of congenital complete heart block was reported as a syndrome of slow pulse, syncopal attacks, and sudden death, often occurring in families.¹ Michaëlsson and Engle's cooperative study of 599 infants and children with congenital complete heart block was published in 1972.² The cause was obscure; speculations included tumors, fetal endocarditis, endocardial fibroelastosis, interstitial myocarditis, linkage to congenital heart defects, and genetic influences. In 1945 Plant and Steven described a case of congenital complete heart block in which the mother had systemic lupus erythematosus, but they did not ascribe a causative relationship.³ By 1977, the association . . .