Also, kids presented with hydrops fetalis and / or EFE and / or cardiomyopathy have poor outcome.Some studies showed a genetic contribution to the autoimmune CHB.Among anti Ro/La positive women, older ones have higher possibility of having kids with heart block.Mortality rate in CHB increases with earlier deliveries.Kids with congenital heart block have higher chance to face health-related problems (such as infections) than other kids. Epidemiology
The congenital heart block occurs in 1 child in every 15,000 to 20,000 births.More than 90% of the cases are associated with autoimmune disease and transference of maternal autoantibodies.Without considering the gender, the age of diagnosis or the associated diseases, mortality rate is around 20%.
Plasmapheresis
Plasma exchange in women positive to anti Ro/La autoantibodies has not been studied thoroughly, but it is suggested to have and effect on the titer of the antibodies in the mothers serum and therefore might have a preventive role. Intravenous immunoglobulin
Using intravenous immunoglobulin showed some promising results in decreasing the possibility of having CHBs complications such as EFE and cardiomyopathy. Hydroxychloroquine
Hydroxychloroquine is relatively new approach, but it showed promising results in preventing the inflammation and other injuries result from it such as fibrosis.Apart from these medications, a pace maker might be needed in around two thirds of the cases, and a procedure might be required when the heart has structural abnormalities. Outcomes
The outcome of the congenital heart block varies a lot due to several factors, such as the associated diseases, severity of the atrioventricular block, maternal age...etc. In terms of the severity of the AV block, newborn kids with heart rate lower than 55 bpm have a negative outcome and higher chance to need pace-maker implantation, as well as kids with symptomatic bradycardia such as lower tolerance of exercises.Isolated CHB has a better prognosis than the one associated with other disorders, the presence of congenital cardiac abnormalities increases the mortality rate.