BACKGROUND: Anti-SSA/Ro-associated third-degree congenital heart block is irreversible, prompting a search for early markers and effective therapy. METHODS AND RESULTS: One hundred twenty-seven pregnant women with anti-SSA/Ro antibodies were enrolled; 95 completed an evaluable course in 98 pregnancies. The protocol included fetal echocardiograms performed weekly from 16 to 26 weeks' gestation and biweekly from 26 to 34 weeks. PR intervals >150 ms were considered prolonged, consistent with first-degree block. Ninety-two fetuses had normal PR intervals. Neonatal lupus developed in 10 cases; 4 were neonatal lupus rash only. Three fetuses had third-degree block; none had a preceding abnormal PR interval, although in 2 fetuses >1 week elapsed between echocardiographic evaluations. Tricuspid regurgitation preceded third-degree block in 1 fetus, and an atrial echodensity preceded block in a second. Two fetuses had PR intervals >150 ms. Both were detected at or before 22 weeks, and each reversed within 1 week with 4 mg dexamethasone. The ECG of 1 additional newborn revealed a prolonged PR interval persistent at 3 years despite normal intervals throughout gestation. No first-degree block developed after a normal ECG at birth. Heart block occurred in 3 of 16 pregnancies (19%) in mothers with a previous child with congenital heart block and in 3 of 74 pregnancies (4%) in mothers without a previous child with congenital heart block or rash (P=0.067). CONCLUSIONS: Prolongation of the PR interval was uncommon and did not precede more advanced block. There was a trend toward more congenital heart block in fetuses of women with previously affected offspring than those without previously affected offspring. Advanced block and cardiomyopathy can occur within 1 week of a normal echocardiogram without initial first-degree block. Echodensities and moderate/severe tricuspid regurgitation merit attention as early signs of injury.
BACKGROUND: Anti-SSA/Ro-associated third-degree congenital heart block is irreversible, prompting a search for early markers and effective therapy. METHODS AND RESULTS: One hundred twenty-seven pregnant women with anti-SSA/Ro antibodies were enrolled; 95 completed an evaluable course in 98 pregnancies. The protocol included fetal echocardiograms performed weekly from 16 to 26 weeks' gestation and biweekly from 26 to 34 weeks. PR intervals >150 ms were considered prolonged, consistent with first-degree block. Ninety-two fetuses had normal PR intervals. Neonatal lupus developed in 10 cases; 4 were neonatal lupus rash only. Three fetuses had third-degree block; none had a preceding abnormal PR interval, although in 2 fetuses >1 week elapsed between echocardiographic evaluations. Tricuspid regurgitation preceded third-degree block in 1 fetus, and an atrial echodensity preceded block in a second. Two fetuses had PR intervals >150 ms. Both were detected at or before 22 weeks, and each reversed within 1 week with 4 mg dexamethasone. The ECG of 1 additional newborn revealed a prolonged PR interval persistent at 3 years despite normal intervals throughout gestation. No first-degree block developed after a normal ECG at birth. Heart block occurred in 3 of 16 pregnancies (19%) in mothers with a previous child with congenital heart block and in 3 of 74 pregnancies (4%) in mothers without a previous child with congenital heart block or rash (P=0.067). CONCLUSIONS: Prolongation of the PR interval was uncommon and did not precede more advanced block. There was a trend toward more congenital heart block in fetuses of women with previously affected offspring than those without previously affected offspring. Advanced block and cardiomyopathy can occur within 1 week of a normal echocardiogram without initial first-degree block. Echodensities and moderate/severe tricuspid regurgitation merit attention as early signs of injury.
Authors: Peter M Izmirly; Amit Saxena; Mimi Y Kim; Dan Wang; Sara K Sahl; Carolina Llanos; Deborah Friedman; Jill P Buyon Journal: Circulation Date: 2011-10-03 Impact factor: 29.690
Authors: Ruben J Acherman; Deborah M Friedman; Jill P Buyon; Joel Schwartz; William J Castillo; Robert C Rollins; William N Evans Journal: Prenat Diagn Date: 2010-08 Impact factor: 3.050
Authors: Carolina Llanos; Deborah M Friedman; Amit Saxena; Peter M Izmirly; Chung-E Tseng; Renata Dische; Rosanna G Abellar; Marc Halushka; Robert M Clancy; Jill P Buyon Journal: Rheumatology (Oxford) Date: 2012-02-03 Impact factor: 7.580