on January 22, 2002
Published online before print January 22, 2002, doi: 10.1161/hc0702.104182
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Submitted on August 23, 2001
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. * To whom correspondence should be addressed. 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.
Revised on December 6, 2001
Accepted on December 19, 2001
Maternal Anti-Ro and Anti-La Antibody--Associated
Endocardial Fibroelastosis
Key words: antibodies • cardiomyopathy • echocardiography • heart block • immune system
This article has been cited by other articles:
 |
|
 |
|
  J. Frankovich, C. Sandborg, P. Barnes, S. Hintz, and E. Chakravarty Neonatal Lupus and Related Autoimmune Disorders of Infants NeoReviews, May 1, 2008; 9(5): e206 - e217. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J.P. Buyon and R.M. Clancy Dying right to live longer: positing apoptosis as a link between maternal autoantibodies and congenital heart block Lupus, February 1, 2008; 17(2): 86 - 90. [Abstract] [PDF]
|
|
|
|
 |
|
 L. K. Hornberger and K. Collins New insights into fetal atrioventricular block using fetal magnetocardiography. J. Am. Coll. Cardiol., January 1, 2008; 51(1): 85 - 86. [Full Text] [PDF]
|
|
|
|
 |
|
 H. M Gardiner, C. Belmar, L. Pasquini, A. Seale, M. Thomas, W. Dennes, M. J O Taylor, E. Kulinskaya, and R. Wimalasundera Fetal ECG: a novel predictor of atrioventricular block in anti-Ro positive pregnancies Heart, November 1, 2007; 93(11): 1454 - 1460. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. G. Tzioufas and H. M. Moutsopoulos Predicting autoimmune congenital heart block: is it feasible and how? Rheumatology, August 1, 2007; 46(8): 1221 - 1222. [Full Text] [PDF]
|
|
|
|
|
|
E. Villain, N. Coastedoat-Chalumeau, E. Marijon, Y. Boudjemline, J.-C. Piette, and D. Bonnet Presentation and Prognosis of Complete Atrioventricular Block in Childhood, According to Maternal Antibody Status J. Am. Coll. Cardiol., October 17, 2006; 48(8): 1682 - 1687. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Tincani, C. B. Rebaioli, M. Taglietti, and Y. Shoenfeld Heart involvement in systemic lupus erythematosus, anti-phospholipid syndrome and neonatal lupus Rheumatology, October 1, 2006; 45(suppl_4): iv8 - iv13. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
N Costedoat-Chalumeau, S Georgin-Lavialle, Z Amoura, and J-C Piette Anti-SSA/Ro and anti-SSB/La antibody-mediated congenital heart block Lupus, September 1, 2005; 14(9): 660 - 664. [Abstract] [PDF]
|
|
|
|
|
|
Y Maeno, W Himeno, A Saito, S Hiraishi, O Hirose, M Ikuma, N Inamura, M Kawataki, A Mizukami, M Ota, et al. Clinical course of fetal congenital atrioventricular block in the Japanese population: a multicentre experience Heart, August 1, 2005; 91(8): 1075 - 1079. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 E. T. Jaeggi, J.-C. Fouron, E. D. Silverman, G. Ryan, J. Smallhorn, and L. K. Hornberger Transplacental Fetal Treatment Improves the Outcome of Prenatally Diagnosed Complete Atrioventricular Block Without Structural Heart Disease Circulation, September 21, 2004; 110(12): 1542 - 1548. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J P Buyon, A Rupel, and R M Clancy Neonatal lupus syndromes Lupus, September 1, 2004; 13(9): 705 - 712. [Abstract] [PDF]
|
|
|
|
|
|
V Fesslova, S Mannarino, P Salice, C Boschetto, L Trespidi, B Acaia, F Mosca, R Cimaz, and P L Meroni Neonatal lupus: fetal myocarditis progressing to atrioventricular block in triplets Lupus, October 1, 2003; 12(10): 775 - 778. [Abstract] [PDF]
|
|
|
|
 |
|
 R. M. Clancy, C. B. Backer, X. Yin, R. P. Kapur, Y. Molad, and J. P. Buyon Cytokine Polymorphisms and Histologic Expression in Autopsy Studies: Contribution of TNF-{alpha} and TGF-{beta}1 to the Pathogenesis of Autoimmune-Associated Congenital Heart Block J. Immunol., September 15, 2003; 171(6): 3253 - 3261. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E Rosenthal Classification of congenital complete heart block: autoantibody-associated or isolated? Lupus, June 1, 2003; 12(6): 425 - 426. [PDF]
|
|
|
|
|
|
L. E. Nield, E. D. Silverman, J. F. Smallhorn, G. P. Taylor, J. Brendan, M. Mullen, L. N. Benson, and L. K. Hornberger Endocardial fibroelastosis associated with maternal anti-Ro and anti-La antibodies in the absence of atrioventricular block J. Am. Coll. Cardiol., August 21, 2002; 40(4): 796 - 802. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Burch Heart failure in the young Heart, August 1, 2002; 88(2): 198 - 202. [Full Text] [PDF]
|
|