Dror Mevorach1, Uriel Elchalal, Azaria J J T Rein. 1. Rheumatology Research Center, Hadassah-Hebrew University Medical Center, Kiryat Hadassah, Jerusalem, Israel. mevorachd@hadassah.org.il
Abstract
PURPOSE OF REVIEW: To describe the results of two recent prospective studies that may indicate how to monitor, diagnose, and treat fetuses with neonatal lupus manifesting with heart involvement and to summarize additional research reports regarding the pathophysiology and outcomes of this rare condition. RECENT FINDINGS: The PR Interval and Dexamethasone Evaluation study found 10 cases of neonatal lupus (10%) with three first-degree atrioventricular blocks (AVBs) and three complete heart blocks. The study included 98 pregnancies in 95 women with anti-SSA/Ro antibodies who completed weekly fetal Doppler echocardiogram-based evaluation. The authors concluded that they were unable to detect first-degree AVB before progression to complete heart block. A similar observational prospective study was performed in 70 fetuses of 56 mothers using tissue velocity fetal kinetocardiogram for measurement of PR prolongation. In this study, six fetuses (8.5%) showed first-degree AVB, and fast normalization of heart function was achieved through maternal treatment with fluorinated steroids. The authors concluded that fetal kinetocardiogram can detect first-degree AVB in the fetus exposed to maternal anti-SSA/Ro or anti-SSB/La antibodies or both and that fluorinated steroids given on detection were associated with normalized atrioventricular conduction in fetuses with first-degree AVB. SUMMARY: Echo Doppler seems a less reliable method for early detection of fetus first-degree AVB, and it is suggested that fetal kinetocardiogram or fetal electrocardiography are preferred. Although atrioventricular block reverses spontaneously in some fetuses, parents and treating physicians should consider immediate treatment with fluorinated steroids once a first-degree AVB is detected due to the high risk of rapid progression to complete blockage.
PURPOSE OF REVIEW: To describe the results of two recent prospective studies that may indicate how to monitor, diagnose, and treat fetuses with neonatal lupus manifesting with heart involvement and to summarize additional research reports regarding the pathophysiology and outcomes of this rare condition. RECENT FINDINGS: The PR Interval and Dexamethasone Evaluation study found 10 cases of neonatal lupus (10%) with three first-degree atrioventricular blocks (AVBs) and three complete heart blocks. The study included 98 pregnancies in 95 women with anti-SSA/Ro antibodies who completed weekly fetal Doppler echocardiogram-based evaluation. The authors concluded that they were unable to detect first-degree AVB before progression to complete heart block. A similar observational prospective study was performed in 70 fetuses of 56 mothers using tissue velocity fetal kinetocardiogram for measurement of PR prolongation. In this study, six fetuses (8.5%) showed first-degree AVB, and fast normalization of heart function was achieved through maternal treatment with fluorinated steroids. The authors concluded that fetal kinetocardiogram can detect first-degree AVB in the fetus exposed to maternal anti-SSA/Ro or anti-SSB/La antibodies or both and that fluorinated steroids given on detection were associated with normalized atrioventricular conduction in fetuses with first-degree AVB. SUMMARY: Echo Doppler seems a less reliable method for early detection of fetus first-degree AVB, and it is suggested that fetal kinetocardiogram or fetal electrocardiography are preferred. Although atrioventricular block reverses spontaneously in some fetuses, parents and treating physicians should consider immediate treatment with fluorinated steroids once a first-degree AVB is detected due to the high risk of rapid progression to complete blockage.