Case report/Caso clínico 318 Acta Obstet Ginecol Port 2015;9(4):318-321 *MD, Obstetrics and Gynecology Resident, Maternal-fetal Department, Maternidade Dr. Alfredo da Costa - Centro Hospitalar de Lisboa Central, Lisboa, Portugal **MD, Consultant, Pediatric Cardiology Department, Hospital de Santa Marta - Centro Hospitalar de Lisboa Central, Lisboa, Portugal ***MD, Consultant in Internal Medicine, Maternidade Dr. Alfredo da Costa - Centro Hospitalar de Lisboa Central, Lisboa, Portugal ****MD, PhD, Consultant, Maternal-fetal Department, Maternidade Dr. Alfredo da Costa - Centro Hospitalar de Lisboa Central; NOVA Medical School, Universidade Nova de Lisboa, Portugal INTRODUCTION C ongenital complete atrioventricular block (AVB) without cardiac malformation occurs in 1-15000 to 1-20000 pregnancies 1,2 . It is a permanent, practical- ly irreversible and potentially fatal complication 3 . In more than 90% of cases results from neonatal lupus ery- thematosus through transplacental passage of antibo- dies anti-SSA/Ro and/or anti-SSB/La 2 . Maternal administration of high-dose oral fluorinated steroids may reverse or inhibit progression of first and second degree AVB. However, in the presence of a third degree AVB the therapeutical results have been inconsistent. The authors present a case of congenital complete AVB and a review of the literature. CASE REPORT A 34-year-old primiparous woman was referred to our Maternity at the 24 th week of gestation, due to fetal bradycardia detected on a routine ultrasound performed two weeks earlier. The previous pregnancy resulted in a healthy child. Her medical history was irrelevant. Our initial eva- luation included a fetal ultrasound and echocardiogram and maternal autoantibody screening, with emphasis on testing anti-nuclear antibody (ANA), anti double- -stranded DNA antibody (anti-dsDNA) and anti-ex- tractable nuclear antigens (ENA), particularly anti- -SSA/Ro and anti-SSB/La (Table I). Fetal ultrasound revealed normal fetal growth (25 th percentile) with nor- mal umbilical and mean cerebral artery doppler scans. Abstract Introduction: Congenital complete atrioventricular block (AVB) without cardiac malformation is a rare and potentially fatal condition. In most cases it is associated with maternal systemic lupus erythematosus through transplacental passage of antibodies anti-SSA/Ro and/or anti-SSB/La. Antenatal fluorinated-steroids have been successful in reversing first and second degree congenital AVB but inconsistent in third degree block. Case Report: The authors report a case of fetal bradycardia diagnosed at 24 weeks of gestation. The fetal echocardiogram revealed a second/third degree AVB without structural heart disease. Maternal anti-SSA/Ro antibodies were detected. There was no blockage improvement with maternal oral fluorinated-steroids. An elective cesarean section was performed at term with the delivery of a healthy girl that required an epicardical pacemaker on the 8 th day of life. Conclusion: In this case, treatment with maternal fluorinated corticosteroids was not effective in preventing progression of the heart block. Keywords: Congenital heart block; Pregnancy; anti-SSA/Ro antibodies; Anti-SSB/La antibodies; Fluorinated corticosteroids Congenital complete atrioventricular block – case report and review of the literature Bloqueio aurículo-ventricular completo congénito – caso clínico e revisão da literatura Ana Catarina Massa*, Isabel Freitas**, Augusta Borges***, Fátima Serrano**** Maternidade Dr. Alfredo da Costa e Hospital de Santa Marta - Centro Hospitalar de Lisboa Central