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(Circulation. 2009;119:1867-1872.)
© 2009 American Heart Association, Inc.

Congenital Heart Disease

Early Diagnosis and Treatment of Atrioventricular Block in the Fetus Exposed to Maternal Anti-SSA/Ro-SSB/La Antibodies

A Prospective, Observational, Fetal Kinetocardiogram–Based Study

A.J.J.T. Rein, MD; D. Mevorach, MD; Z. Perles, MD; S. Gavri, MD; M. Nadjari, MD; A. Nir, MD; U. Elchalal, MD

From the Department of Pediatric Cardiology (A.J.J.T.R., Z.P., S.G., A.N.), Rheumatology Unit (D.M.), Department of Medicine, and Department of Obstetrics and Gynecology (M.N., U.E.), Hadassah University Medical Center, Jerusalem, Israel.

Correspondence to Azaria J.J.T. Rein, MD, Head, Department of Pediatric Cardiology, Hadassah–Hebrew University Medical Center, Kiryat Hadassah, POB 12000, Jerusalem 91102, Israel. E-mail rein{at}huji.ac.il

Received February 14, 2008; accepted January 6, 2009.

Background— A fetus exposed to maternal anti-SSA/Ro or anti-SSB/La antibodies (or both) may develop complete atrioventricular block (AVB), which results in high prenatal and postnatal morbidity and mortality. Until recently, only high-grade AVB could be diagnosed in utero. The tissue velocity–based fetal kinetocardiogram (FKCG) enables accurate measurement of AV conduction time and diagnosis of low-grade AVB. In the present multicenter observational study, we used FKCG to detect first-degree AVB in fetuses at risk.

Methods and Results— FKCG was performed in 70 fetuses of 56 mothers who were positive for anti-SSA/Ro and/or anti-SSB/La. Fetuses were monitored with weekly FKCG from 13 to 24 weeks’ gestation, followed by monthly assessments until delivery in unaffected fetuses and weekly assessments in affected fetuses. AV conduction in 70 at-risk and 109 normal fetuses was compared. FKCG was obtained readily in all fetuses; 6 showed first-degree AVB (AV conduction time >2 z scores above normal mean) at 21 to 34 gestational weeks. Immediate maternal treatment with dexamethasone resulted in normalization of AV conduction in all affected fetuses within 3 to 14 days. AV conduction time in the remaining 64 untreated fetuses remained normal throughout gestation. The ECG PR interval immediately after birth was normal in all affected newborns. No child developed AVB or cardiomyopathy in the subsequent 1- to 6-year (median 4-year) follow-up.

Conclusions— The present findings suggest that an FKCG can detect first-degree AVB in the fetus exposed to maternal anti-SSA/Ro or anti-SSB/La antibodies (or both). Dexamethasone given on detection was associated with normalized AV conduction in fetuses with first-degree AVB. No fetus in the present study developed complete prenatal or postnatal AVB.

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CLINICAL PERSPECTIVE

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Clinical Summaries
Circulation 2009 119: 1843-1845. [Extract] [Full Text]