Abstract 1945: Preventive IVIG Therapy for Congenital Heart Block (PITCH) Study
Objective: The recurrence rate of anti-SSA/Ro associated congenital heart block (CHB) is 18%. Reversal of 3rd degree block has never been achieved. Based on potential reduction of maternal autoantibody titers as well as fetal inflammatory responses, IVIG was evaluated as a preventative therapy for CHB.
Methods: A multicenter open-label study with a power calculation based on Simon’s 2-stage optimal design was initiated. Enrollment criteria included: maternal anti-SSA/Ro antibody, a previous child with CHB/rash, </= 20 mg prednisone, < 12 weeks pregnant. IVIG (400mg/kg) was given every 3 weeks from 12 to 24 weeks of gestation. The primary outcome was the development of 2nd or 3rd degree CHB.
Results: Twenty mothers completed the IVIG protocol before reaching the stopping rule of 3 advanced CHB. CHB was detected at 19, 20 and 25 weeks; none was preceded by an abnormal PR interval. One of these mothers had two previous children with CHB. One child without CHB developed a transient rash consistent with neonatal lupus. Fourteen children have no manifestations of neonatal lupus and 2 fetuses remain unaffected at ≥ 30 weeks of gestation. No significant changes in maternal antibody titers to SSA/Ro, SSB/La, or Ro52 were detected over the course of therapy or at delivery. There were no safety issues.
Conclusions: IVIG at doses consistent with replacement does not prevent the recurrence of CHB or reduce maternal antibody titers. Having established safety with this protocol and feasibility of patient enrollment, subsequent studies should address the efficacy of IVIG at higher doses.