TY - JOUR
T1 - Prospective Evaluation of Fetuses With Autoimmune-Associated Congenital Heart Block Followed in the PR Interval and Dexamethasone Evaluation (PRIDE) Study
AU - Friedman, Deborah M.
AU - Kim, Mimi Y.
AU - Copel, Joshua A.
AU - Llanos, Carolina
AU - Davis, Claudine
AU - Buyon, Jill P.
N1 - Funding Information:
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.
PY - 2009/4/15
Y1 - 2009/4/15
N2 - 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.
AB - 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.
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U2 - 10.1016/j.amjcard.2008.12.027
DO - 10.1016/j.amjcard.2008.12.027
M3 - Article
C2 - 19361597
AN - SCOPUS:63749103526
SN - 0002-9149
VL - 103
SP - 1102
EP - 1106
JO - American Journal of Cardiology
JF - American Journal of Cardiology
IS - 8
ER -