Key result
Maternal autoantibody-associated endocardial fibroelastosis with congenital heart block has a very high mortality rate, with 85% of cases resulting in death or cardiac transplantation.
Observational (n=13)
Yes
Autoantibody-associated endocardial fibroelastosis in fetuses and children with congenital heart block carries an 85% mortality or transplant rate and can develop despite adequate ventricular pacing.
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High mortality warrants vigilant fetal monitoring in antibody-positive pregnancies; leaves open optimal prevention and therapy strategies.
Nield et al. (2002) conducted an observational in Endocardial fibroelastosis associated with congenital heart block (n=13). Maternal anti-Ro and anti-La antibodies was evaluated on Death or cardiac transplantation secondary to EFE. Maternal autoantibody-associated endocardial fibroelastosis with congenital heart block has a very high mortality rate, with 85% of cases resulting in death or cardiac transplantation.
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