Published online before print January 22, 2002, doi: 10.1161/hc0702.104182
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(Circulation. 2002;105:843.)
© 2002 American Heart Association, Inc.
Clinical Investigation and Reports |
Maternal Anti-Ro and Anti-La Antibody–Associated Endocardial Fibroelastosis
From the Department of Paediatrics, Division of Cardiology (L.E.N., J.F.S., R.M.H., L.K.H.), Division of Pathology (G.P.T.), and Division of Rheumatology (E.D.S.), The Hospital for Sick Children, University of Toronto Faculty of Medicine, and Department of Obstetrics (P.G.S.) and Department of Pathology and Laboratory Medicine (J.B.M.M.), Mount Sinai Hospital, Toronto, Ontario, Canada; Department of Cardiology (N.H.S.), University of California, San Francisco, Calif; Department of Cardiology (J.P.F.), Isaak Walton Killiam Children’s Hospital, Halifax, Nova Scotia, Canada; Division of Pediatric Cardiology (Y.M.L.), Children’s Hospital of Pittsburgh, Pittsburgh, Pa; and Division of Paediatric Cardiology (D.G.H.), British Columbia’s Children’s Hospital, Vancouver, British Columbia, Canada.
Correspondence to Lisa K. Hornberger, MD, The Hospital for Sick Children, Division of Cardiology, 555 University Ave, Toronto, Ontario M5G 1X8, Canada. E-mail hornberg{at}sickkids.on.ca
Background— Maternal anti-Ro and anti-La antibodies are associated with congenital heart block (CHB). Although endocardial fibroelastosis (EFE) has been described in isolated cases of autoantibody-mediated CHB, the natural history and pathogenesis of this disease are poorly understood.
Methods and Results— We retrospectively reviewed the clinical history, echocardiography, and pathology of fetuses and children with EFE associated with CHB born to mothers positive for anti-Ro or anti-La antibodies at 5 centers. Thirteen patients were identified, 6 with a prenatal and 7 with a postnatal diagnosis. Six mothers were positive for anti-Ro and anti-La antibodies, and 7 were positive for anti-Ro antibodies only. Only 1 mother had autoimmune disease. Severe ventricular dysfunction was seen in all fetal and postnatal cases. Four fetal and 3 postnatal cases had EFE at initial presentation. However, 2 fetal and 4 postnatal cases developed EFE 6 to 12 weeks and 7 months to 5 years from CHB diagnosis, respectively, even despite ventricular pacing in 6 postnatal cases. Eleven (85%) either died (n=9) or underwent cardiac transplantation (n=2) secondary to the EFE. Pathologic assessment of the explanted heart, available in 10 cases, revealed moderate to severe EFE in 7 and mild EFE in 3 cases, predominantly involving the left ventricle. Immunohistochemistry in 4 cases (including 3 fetuses) demonstrated deposition of IgG in 4 and IgM in 3 and T-cell infiltrates in 3 cases, suggesting an immune response by the affected fetus or child.
Conclusions— EFE occurs in the presence of autoantibody-mediated CHB despite adequate ventricular pacing. Autoantibody-associated EFE has a very high mortality rate, whether developing in fetal or postnatal life.
Key Words: antibodies • cardiomyopathy • echocardiography • heart block • immune system
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