Arrhythmias and conduction disturbance
Prospective Evaluation of Fetuses With Autoimmune-Associated Congenital Heart Block Followed in the PR Interval and Dexamethasone Evaluation (PRIDE) Study

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We evaluated the efficacy of dexamethasone (DEX) in anti-SSA/Ro–exposed fetuses newly diagnosed with congenital heart block. Previous use of DEX has been anecdotal with varying reports of therapeutic benefit. This was a multicenter, open-label, nonrandomized study involving 30 pregnancies treated with DEX (22 with third-degree block, 6 with second-degree block, 2 with first-degree block) and 10 untreated (9 with third-degree block, 1 with first-degree block). Initial median ventricular rates, age at diagnosis, and degree of cardiac dysfunction were similar between groups. Six deaths occurred in the DEX group. There was no reversal of third-degree block with therapy or spontaneously. In fetuses treated with DEX, 1/6 with second-degree block progressed to third-degree block and 3 remained in second-degree block (postnatally 1 paced, 2 progressed to third degree); 2 reverted to normal sinus rhythm (NSR; postnatally 1 progressed to second degree). DEX reversed the 2 fetuses with first-degree block to NSR by 7 days with no regression at discontinuation. Absent DEX, the 1 with first-degree block detected at 38 weeks had NSR at birth (overall stability or improvement in 4 of 8 in the DEX group vs 1 of 1 in the non-DEX group). Median gestational birth age was 37 weeks in the DEX group versus 38 weeks in the non-DEX group (p = 0.019). Prematurity and small size for gestational age were restricted to the DEX group. Pacemaker use and growth parameters at birth and 1 year were similar between groups. In conclusion, these data confirm the irreversibility of third-degree block and progression of second- to third-degree block despite DEX. A potential benefit of DEX in reversing first- or second-degree block was supported in rare cases but should be weighed against potential steroid side effects such as growth restriction.

Section snippets

Methods

Patients entered this prospective, multicenter, observational study from December 2000 to April 2006. A total of 40 pregnant women with anti-SSA/Ro antibodies (with/without anti-SSB/La antibodies) were enrolled from 33 centers across the United States by participating clinicians who included rheumatologists, pediatric cardiologists, and obstetricians. The study was approved by the IRB of the New York University School of Medicine (New York, New York). Written informed consent was obtained from

Results

Forty mothers identified with a fetus with CHB were enrolled. Maternal health status was as follows: 11 women were asymptomatic, 10 had SS, 6 had SLE, 1 had SLE/SS, 1 had discoid lupus, 10 had UAS, and 1 had rheumatoid arthritis. Recurrent fetal disease was common: 4 women (10%) had a previous offspring with CHB, and 5 women (13%) had a previous infant with the characteristic rash of neonatal lupus. All mothers with a previously affected child were in the DEX group.

Thirty of these mothers

Discussion

This component of the PRIDE study2 comprises prospective observational data obtained with the use of a structured protocol including serologic confirmation and echocardiographic oversight from 40 fetuses diagnosed with CHB. Confirming and extending published studies, once fetal third-degree block was detected, it was irreversible, regardless of treatment with DEX.1 Moreover, second-degree block progressed to third-degree block despite DEX treatment, as previously reported.10 This study also

Acknowledgment

We thank the following investigators for contributing to the PRIDE study: Matthew Allsede, MD, Ahmed Baschat, MD, Tara Becker, MD, T.M. Biancaniello, MD, Morton Borg, MD, Patrick Callahan, MD, Eric J. Carlson, MD, Edward Chien, MD, Kathryn Collins, MD, Bettina F. Cuneo, MD, William Cusick, MD, Curt DeGroff, MD, Mary Donofrio, MD, Robin Doroshow, MD, James F.X. Egan, MD, Mohammed Elkousy, MD, Llyod Feit, MD, Carlen Gomez, MD, Nina Gotteiner, MD, Julie Glickstein, MD, David Hirsch, MD, Eby

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This work was funded by Grant RO1 AR42455-01 (Maternal Autoantibodies: Pathogenesis of Neonatal Lupus) from the National Institutes of Health/National Institute of Arthritis and Musculoskeletal and Skin Diseases (Bethesda, Maryland) to Dr. Buyon, Contract NO1-AR-4-2220 (Research Registry for Neonatal Lupus) from the National Institutes of Health to Dr. Buyon, Grant 1-R01-AR46265 (the PRIDE study) from the National Institutes of Health/National Institute of Arthritis and Musculoskeletal and Skin Diseases to Dr. Buyon, and a generous gift from the Lee family, Brooklyn, New York.

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