Autoimmune-Associated Congenital Heart Block: Demographics, Mortality, Morbidity and Recurrence Rates Obtained From a National Neonatal Lupus Registry ☆

http://www.arritmiaonline.com.br/files/bavt_em_rn.pdf

Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block: A single institution's experience of 30 years

Background— Untreated isolated fetal complete atrioventricular block (CAVB) has a significant mortality rate. A standardized treatment approach, including maternal dexamethasone at CAVB diagnosis and β-stimulation for fetal heart rates <55 bpm, has been used at our institutions since 1997. The study presents the impact of this approach. Methods and Results— Thirty-seven consecutive cases of fetal CAVB since 1990 were studied. Mean age at diagnosis was 25.6±5.2 gestational weeks. In 33 patients (92%), CAVB was associated with maternal anti-Ro/La autoantibodies. Patients were separated into those diagnosed between 1990 and 1996 (group 1; n=16) and those diagnosed between 1997 and 2003 (group 2; n=21). The 2 study groups were comparable in the clinical presentation at CAVB diagnosis but did differ in prenatal management (treated patients: group 1, 4/16; group 2, 18/21; P<0.0001). Overall, 22 fetuses were treated, 21 with dexamethasone and 9 with β-stimulation for a mean of 7.5±4.5 weeks. Live-birth and 1-yea...

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Transplacental fetal treatment improves the outcome of prenatally diagnosed complete atrioventricular block without structural heart disease

Perinatal outcome of fetal complete atrioventricular block: a multicenter experience.

Objective

To compare intervention with fluorinated glucocorticoids to the natural history of untreated congenital heart block (CHB) with respect to conduction abnormalities, associated effusions, ascites, and hydrops fetalis, and the requirement for a pacemaker.



Methods

Records of all mothers enrolled in the Research Registry for Neonatal Lupus were reviewed. The cohort includes 47 mothers whose sera contain anti-SSA/Ro or anti-SSB/La antibodies, and their 50 offspring with CHB, in whom at least 4 echocardiograms were performed after in utero diagnosis. In 28 pregnancies, mothers received dexamethasone 4–9 mg/day for 3–19 weeks or betamethasone 12–24 mg/week for >6 weeks (group A). In 22 pregnancies, fluorinated steroids were not used (group B).



Results

Third-degree block was present in 21 fetuses in group A and 18 fetuses in group B; none were reversible despite steroid treatment. Three fetuses in group A and 2 in group B progressed from second-degree block, alternating with third-degree block, to permanent third-degree block at birth and postnatally. Of 4 fetuses in group A with second-degree block at presentation, all reverted to first-degree block by birth; 2 remain so at age 4 years, 1 alternates between first-degree and second-degree block at 2 years, and the fourth is in second-degree block at age 4 years. Of 2 fetuses in group B with second-degree block at presentation, both progressed to permanent third-degree block postnatally. Initial echocardiographic evaluation revealed pericardial effusions in 13 group A versus 4 group B fetuses, pleural effusions in 2 group A versus 0 group B, ascites in 8 group A versus 0 group B (P < 0.007), hydrops fetalis in 8 group A versus 0 group B (P < 0.007), and intrauterine growth restriction in 1 group A versus 1 group B. Pericardial effusions resolved and reappeared in both groups. Steroid therapy was most effective in the resolution of pleural effusions (2 of 2), ascites (6 of 8), and hydrops fetalis (5 of 8). Oligohydramnios ensued in 9 group A and 2 group B fetuses. Although fetuses in group A had more complications at presentation than those in group B, there were no significant differences in the duration of pregnancy (35.7 weeks versus 37.0 weeks), the number of deaths (4 versus 1), final degree of heart block, or requirement for a pacemaker (14 versus 11).



Conclusion

While prospective trials are needed, these data suggest that fluorinated steroids should be considered for fetuses with incomplete block or hydropic changes. Serial echocardiograms are recommended to monitor fetal progress. It remains to be determined whether third-degree block is reversible if therapy is initiated immediately upon detection.

Comparison of treatment with fluorinated glucocorticoids to the natural history of autoantibody-associated congenital heart block: retrospective review of the research registry for neonatal lupus.

An experimental therapeutic regimen for congenital lupus erythematosus, which consists of treating the mother with high doses of dexamethasone and performing plasmapheresis (3 times per week), was developed. This regimen was administered to a woman whose pregnancy was complicated by the abrupt onset of fetal pericarditis, myocarditis, and complete heart block in the twenty-fourth week of gestation. The effectiveness of plasmapheresis was evidenced by a decrease in the very high titer of SS-B (La) antibodies in the mother. After 5 weeks of therapy, the cardiac disease was ameliorated. The infant tolerated delivery at week 31, and the heart block has persisted. The rationale for this therapy for fetal disease is discussed, with emphasis on 2 goals: to diminish the quantity of maternal antibody that is transmitted to the fetus, and to suppress the manifestations of fetal carditis with the use of glucocorticoids that are not inactivated by the placenta. Tentative recommendations for more intensive prenatal monitoring of maternal and fetal status for those at risk are outlined.

Intrauterine therapy for presumptive fetal myocarditis with acquired heart block due to systemic lupus erythematosus. Experience in a mother with a predominance of SS-B (La) antibodies.

Steven H. Swersky

Papers

Intrauterine therapy for presumptive fetal myocarditis with acquired heart block due to systemic lupus erythematosus. Experience in a mother with a predominance of SS-B (La) antibodies.