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Spectrum and progression of conduction abnormalities in infants born to mothers with anti-SSA/Ro-SSB/La antibodies
A D Askanase
Department of Rheumatology and Medicine, Hospital for Joint Diseases, New York University School of Medicine, New York, New York, USA
D M Friedman
Division of Pediatric Cardiology, Department of Pediatrics, St Luke's-Roosevelt Hospital Center, New York, New York, USA
J Copel
Department of Obstetrics and Gynecology, Yale University School of Medicine, New Haven, Connecticut, USA
M R Dische
Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, New York, USA
A Dubin
Department of Pediatric Cardiology, Stanford University School of Medicine, Stanford, California, USA
T J Starc
Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, New York, USA
M C Katholi
Department of Rheumatology and Medicine, Hospital for Joint Diseases, New York University School of Medicine, New York, New York, USA
J P Buyon
Department of Rheumatology and Medicine, Hospital for Joint Diseases, New York University School of Medicine, New York, New York, USA; Department of Rheumatology, Room 1608, Hospital for Joint Diseases, 301 East 17th Street, New York, NY 10003, USA jill.buyon{at}med.nyu.edu
The classic cardiac manifestation of neonatal lupus is congenitalheart block, attributed to antibodymediated inflammation and subsequent fibrosis of the atrioventricular(AV) node. In consideringthe pathologic process of injury it may be that tissue damage results in a range of conduction abnormalities. Identification of less-advanced degrees of block or of fibrosis around the AV node without any conduction abnormality on EKG would support this pathologic model, and serve as a potential marker for treatment if the conduction defect could be shown to progress. To ascertain the spectrum of arrhythmias associated with maternal anti-SSA/Ro-SSB/La antibodies, records of all children enrolled in the Research Registry for Neonatal Lupus were reviewed. Of 187 children with congenital heart block whose mothers have anti-SSA/Ro-SSB/La antibodies, nine had a prolonged PR interval on EKG at birth, four of whom progressed to more advanced AV block. A child whose younger sibling had third degree block was diagnosed with first degree block at age 10 years at the time of surgery for a broken wrist. Two children diagnosed in utero with second degree block were treated with dexamethasone and reverted to normal sinus rhythm by birth, but ultimately progressed to third degree block. Four children had second degree block at birth: of these, two progressed to third degree block. Sinus bradycardia (< 100 bpm) was present in three (3.8%) of 78 fetuses for whom atrial rates were recorded by echocardiogram. Of 40 neonates for whom EKGs were available, the mean atrial rate was 137± 20 bpm (range 75–200). These data have important research and clinical implications. In contrast to the AV node, permanent sinoatrial nodal involvement is not clinically apparent. Perhaps many fetuses sustain mild inflammation, but resolution is variable, as suggested by the presence of incomplete AV block. Since subsequent progression of less-advanced degrees of block can occur, an EKG should be performed on all infants born to mothers with anti-SSA/Ro-SSB/La antibodies.
Key Words: SSA/Ro • SSB/La • congenital heart block • conduction abnormalities • neonatal lupus syndromes
Lupus, Vol. 11, No. 3,
145-151 (2002)
DOI: 10.1191/0961203302lu173oa
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