Dexamethasone Treatment for Congenital Heart Block (CHB) in Newborns With Lupus

CHB is an abnormal condition in which the heart beats slowly. This is a disease that is
strongly associated with maternal antibodies to SSA/Ro and SSB/La ribonucleoproteins. This
study hopes to clarify the causes of CHB and develop appropriate treatments. The study has
two parts.

The first part of the study will be prospective; it will determine if fluorinated steroids
given to women prior to birth improves the heart function and well-being of their newborns.
This part of the study will evaluate fetuses diagnosed in utero with CHB during the third
trimester of pregnancy. Diagnosis of CHB must occur at least 6 weeks before the baby is born
to allow for sufficient data collection. It will be the decision of the physician and the
mother as to whether a steroid will be administered. Fetuses will be evaluated before
delivery by electrocardiogram (ECG) to detect abnormal fluid collection and by ultrasound to
monitor heartbeat. After birth, newborns will be assessed for overall pumping strength of
the heart and for abnormal heartbeat. Blood will be drawn from the mother at the time of
enrollment and during delivery. Visits will occur over a span of approximately 5 months.

The second part of this study will be observational; the purpose is to identify classic
indicators of heartbeat dysfunction and heart injury in newborns with CHB. The goal of this
part of the study is to better understand the stages of heart injury, the role of anti-Ro/La
antibodies in CHB, and procedures that may reverse heart block. Mothers considered to be at
high risk for having a child with CHB will undergo weekly ECGs from 16 weeks into their
pregnancy until Week 24, then will have an ECG every other week from Week 24 through Week
34. There will be a total of 15 visits to conduct these ECGs. Blood will be drawn at the
first ECG visit and during delivery. Visits will occur over a span of 4 months.

For both parts of the study, babies will undergo ECGs after delivery and at one year of age.
Additional tests not related to the study may be ordered by the physician.

Inclusion Criteria for Prospective Part of Trial:

- Mother must have anti-Ro and/or anti-La antibody

- Fetal bradyarrhythmia (slow, abnormal heart rhythm)

Exclusion Criteria for Prospective Part of Trial:

- Fetal heart abnormalities that may cause newborn CHB and could account for
atrioventricular (AV) block

Inclusion Criteria for Observational Part of Trial:

- Mother must have anti-Ro and/or anti-La antibody

- Fetus must have documented normal heartbeat prior to the 16th week of pregnancy

- Fetus must have a structurally normal heart

- Mother must be enrolled during the 16th, 17th, or 18th week of pregnancy

Exclusion Criteria for Observational Part of Trial:

- Mother is taking more than 10 mg of prednisone per day