Premium
Fetal congenital heart block
Author(s) -
Pennell C.,
Reid K. P.,
Dickson J. E.
Publication year - 2001
Publication title -
ultrasound in obstetrics and gynecology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.202
H-Index - 141
eISSN - 1469-0705
pISSN - 0960-7692
DOI - 10.1046/j.1469-0705.2001.abs19-7.x
Subject(s) - medicine , fetus , pregnancy , gestation , obstetrics , incidence (geometry) , fetal echocardiography , atrioventricular block , hydrops fetalis , pediatrics , prenatal diagnosis , genetics , physics , optics , biology
Purpose: To ascertain the incidence and outcome of all cases of prenatally diagnosed congenital heart block (CHB) referred to our institution during the decade 1990–2000. Methods: Cases were identified from the fetal medicine database permitting review of the clinical presentation, ultrasound findings, maternal immunological status, subsequent management and pregnancy outcome. Results: Eleven cases of CHB were identified during the period of review (100% ascertained for Western Australia) with a diagnosis at median gestation of 20 weeks (16.8–32). Fetal cardiac anatomy was normal in 64% of cases. Fetal hydrops was present at diagnosis in 27% with a further 33% subsequently developing hydrops. Only 18% of fetuses had major structural cardiac anomalies. The median gestation at delivery was 35 weeks (25–38) and there was a female gender bias (7 : 4). The mortality rate was 64% with three pregnancy terminations, one second trimester intrauterine fetal death, one neonatal death and two infant deaths. Maternal immunological abnormalities were common with 36% of the women having clinical autoimmune conditions. Seventy‐eight percent of women had SSA and/or SSB antibodies. Two of the six infants that survived the neonatal period had persistent abnormal autoantibodies during their first year of life. One infant with SSA and SSB died at one year of age from left ventricular failure believed to be of autoimmune origin. This infant was one of triplets, with only the female triplet being affected. Another fetus with SSA and SSB had thrombocytopenia at delivery. Postmortem assessment of five fetuses confirmed focal calcification at the atrioventricular junction associated with a lymphocytic infiltrate and subendocardial fibrosis. Eight subsequent pregnancies have occurred and none were complicated by CHB. Three women underwent prophylactic immunosuppression. Conclusions: Fetal congenital heart block is a rare condition with a strong association with autoimmune disease and poor outcome. The recurrence risk in this series is lower than previously reported.