Abstract
Aims
Autoimmune congenital heart block (CHB) is a severe manifestation of neonatal lupus syndrome. There is lack of consensus regarding treatment of pregnant women with anti-SSA/SSB autoantibodies. To evaluate the effectiveness of the combined protocol therapy (oral steroid, plasmapheresis, and IVIG) for the CHB treatment.
Methods and results
All cases of CHB from 2000 to 2020 were retrospectively enrolled. All the patients underwent foetal echocardiography for the evaluation of CHB, ventricular rate and main related foetal complications: cardiomegaly, pericardial and pleural effusion, foetal hydrops, dilated cardiomyopathy, and heart failure. Moreover, postnatal major adverse cardiovascular, such as death and pacemaker implantation, were recorded. For statistical analysis, the population was divided into two cohorts: a protocol group receiving in utero combined therapy and a control group receiving other therapies or not treated. Among 252 pregnancies with anti-SSA/SSB antibodies, 36 developed CHB. At birth, complete CHB treated with protocol therapies showed a significantly higher ventricular rate (P = 0.042), a significant reduction in intrauterine or postnatal mortality (P = 0.018), and a lower rate of pacemaker implantation (P = 0.049).
Conclusions
The combined treatment protocol has proven effective in improving foetal and neonatal short- and long-term survival.