日本小児循環器学会総会・学術集会

Congenital heart block is rare and can be associated with maternal autoantibody disease, structural heart disease, or in very rare cases inherited channelopathies. Prognosis is particularly guarded in patients with structural disease, with fetal demise, hydrops, and preterm delivery leading to a low overall survival. Heart block due to maternal autoantibody disease (specifically SSA/anti-Ro) affects fewer than 3% of those at risk, but consequences can be severe and include lifelong pacer dependence, cardiomyopathy, and fetal or postnatal death. Newer data suggests that evolving heart block in these fetuses may be amenable to in-utero therapy and intensive monitoring in mid-gestation for high-titer pregnancies is currently under investigation. Distinguishing heart block from pseudo-block and atrial bigeminy is crucial and evaluation for SSA+/anti-Ro and structural disease should be undertaken promptly whenever heart block is suspected.