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Connective-Tissue Disease, Antibodies to Ribonucleoprotein, and Congenital Heart Block

List of authors.
  • James S. Scott, M.D.,
  • Peter J. Maddison, M.D.,
  • Pamela V. Taylor, Ph.D.,
  • Eva Esscher, M.D.,
  • Olive Scott, M.D.,
  • and R. Paul Skinner, Ph.D.

Abstract

The relation between congenital heart block and maternal connective-tissue disease was studied by antibody screening of serum samples obtained in connection with 45 cases of isolated congenital complete heart block. Serum was available from 41 mothers (17 who had connective-tissue disease and 24 who were healthy) and 21 children. Thirty-four mothers had antibody to a soluble tissue ribonucleoprotein antigen called Ro(SS-A), which was identified by immunodiffusion. Anti-Ro(SS-A) was found in seven of eight serum samples collected from affected children when they were less than three months old but in none of 13 samples obtained when these children were older. It appears that maternal anti-Ro(SS-A) antibody crosses the placenta and is a marker for risk of congenital complete heart block; its absence from maternal serum suggests that a child is unlikely to be affected. Anti-Ro(SS-A) or a related antibody is probably involved in the pathogenesis of congenital complete heart block. (N Engl J Med 1983; 309:209–12.)

Funding and Disclosures

Supported in part by a grant (778) from the British Heart Foundation and a grant from the Swedish Life Assurance Association.

We are indebted to the Association of European Paediatric Cardiologists and Dr. Magnus Michaelson for access to information in the registry of cases of congenital heart block held in Uppsala; to Dr. R. Arnold, Dr. E. H. Back, Dr. I. Battye, Prof. A. G. M. Campbell, Dr.J. W. Jordan, Dr. M. Maclean, Dr. L. Tuuteri, and Dr. P. G. I. Stovin for providing information and obtaining specimens; and to Prof. R. H. Anderson and Dr. L. Gerlis for information and helpful suggestions about pathological aspects.

Author Affiliations

From the Department of Obstetrics and Gynaecology (Leeds Maternity Hospital), University of Leeds, and Killingbeck Hospital, Leeds, U.K.; the Arthritis Research Group, Royal National Hospital for Rheumatic Diseases, Bath, U.K.; and the Department of Paediatrics, University Hospital, Uppsala, Sweden. Address reprint requests to Prof. Scott at the Department of Obstetrics and Gynaecology (LMH), University of Leeds, 17 Springfield Mount, Leeds LS2 9NG, U.K.

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