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 8th day of life.
Conclusion: In this case, treatment with maternal fluorinated corticosteroids was not effective in preventing progression of the heart block

Borges, A

Freitas, I

Massa, AC

Serrano, F

Repository of the Hospital Center of Central Lisbon

Bloqueio Aurículo-Ventricular Completo Congénito. Caso Clínico e Revisão da Literatura

Repositório do Centro Hospitalar de Lisboa Central, EPE

Case report/Caso clínico318 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 SantaMarta - Centro Hospitalar de Lisboa Central, Lisboa, Portugal***MD, Consultant in Internal Medicine, Maternidade Dr. Alfredo daCosta - Centro Hospitalar de Lisboa Central, Lisboa, Portugal****MD, PhD, Consultant, Maternal-fetal Department, MaternidadeDr. Alfredo da Costa - Centro Hospitalar de Lisboa Central; NOVAMedical School, Universidade Nova de Lisboa, PortugalINTRODUCTIONCongenital complete atrioventricular block (AVB)without cardiac malformation occurs in 1-15000to 1-20000 pregnancies1,2. It is a permanent, practical-ly irreversible and potentially fatal complication3. Inmore than 90% of cases results from neonatal lupus ery-thematosus through transplacental passage of antibo -dies anti-SSA/Ro and/or anti-SSB/La2.  Maternaladmi nistration of high-dose oral fluorinated steroidsmay reverse or inhibit progression of first and seconddegree AVB. However, in the presence of a third degreeAVB the therapeutical results have been inconsistent. The authors present a case of congenital completeAVB and a review of the literature.CASE REPORTA 34-year-old primiparous woman was referred to ourMaternity at the 24th week of gestation, due to fetalbradycardia detected on a routine ultrasound performedtwo weeks earlier. The previous pregnancy resulted ina healthy child.Her medical history was irrelevant. Our initial eva -luation included a fetal ultrasound and echocardiogramand maternal autoantibody screening, with emphasison 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 ultrasoundrevealed normal fetal growth (25th percentile) with nor-mal umbilical and mean cerebral artery doppler scans.AbstractIntroduction: Congenital complete atrioventricular block (AVB) without cardiac malformation is a rare and potentiallyfatal condition. In most cases it is associated with maternal systemic lupus erythematosus through transplacental passageof antibodies anti-SSA/Ro and/or anti-SSB/La. Antenatal fluorinated-steroids have been successful in reversing first andsecond 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 echocardiogramrevealed 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 performedat term with the delivery of a healthy girl that required an epicardical pacemaker on the 8th day of life.  Conclusion: In this case, treatment with maternal fluorinated corticosteroids was not effective in preventing progressionof the heart block. Keywords: Congenital heart block; Pregnancy; anti-SSA/Ro antibodies; Anti-SSB/La antibodies; Fluorinated corticosteroidsCongenital complete atrioventricular block – case report and review of the literatureBloqueio aurículo-ventricular completo congénito – caso clínico e revisão da literaturaAna Catarina Massa*, Isabel Freitas**, Augusta Borges***, Fátima Serrano****Maternidade Dr. Alfredo da Costa e Hospital de Santa Marta - Centro Hospitalar de Lisboa CentralAna Catarina Massa, Isabel Freitas, Augusta Borges, Fátima SerranoActa Obstet Ginecol Port 2015;9(4):318-321 319Fetal echocardiogram showed a sinus bradycardia withheart rates of 57-60 bpm without associated structuralheart defects, atrioventricular (AV) regurgitation orpericardial effusions. Maternal anti-SSA/Ro antibo -dies were detected.Fetal echocardiogram repeated one week later re-vealed an alternating second to third degree heart blockwith atrial rate of 120 bpm and ventricular rate of 58--60 bpm.The mother was started on oral dexametha-sone (4mg/day) on the 25th week of gestation, whichwas discontinued 6 weeks after, since there was no re-verse in the AV block. Prior to the institution of thefluorinated steroids, 75g oral glucose tolerance test wasperformed within normal range values. Regular fetal echocardiograms confirmed completeAV block without heart failure, pericardial effusionsor atrioventricular impairment. At the 31st week of ges-tation a fetal brain magnetic resonance imagingshowed normal brain, cerebellum and brain stem mor-phology, with no signs of intracranial hemorrhage orencephaloclastic lesions. A mild asymmetry of the la -teral ventricles was identified.At the 35th week of gestation there was a slight in-crease in maternal arterial blood pressure (140/90mmHg) without proteinuria. Fetal growth fell to 15thpercentile with normal umbilical and mean cerebralartery doppler scans. There was no fetal hydrops orsigns of heart failure throughout the entire pregnancy.Due to a fall in atrial and ventricular rates (110 bpmand <50 bpm, respectively) an elective cesarean sectionwas performed at 37 weeks of gestation. The newbornhad an Apgar score of 8/8 and weighted 2320g. Heartrate at birth was above 50 bpm. The newborn wastransferred immediately to our Pediatric CardiologyUnit. Electrocardiogram confirmed a complete AVBwith a stable heart rate of 40-50 bpm. On the 8th dayof life an epicardical pacemaker was implanted. Post-natal echocardiogram reaffirmed the absence of struc-tural heart defects or signs of heart failure. The new-born was discharged at the 20th day. Maternal blood pressure normalized in the postpar-tum period. TABLE I. AUTOANTIBODIES PANELBlood Test 25th week 33rd week 3 weeks postpartumANA Positive (1/640) Positive (1/640) Positive (1/320)Anti-dsDNA Negative Negative NegativeAnti-SSA/Ro Positive Positive PositiveAnti-SSB/La Negative Negative NegativeLupic anticoagulant Negative Negative NegativeAnti-cardiolipin IgG/IgM Negative/Negative Negative/Negative Uncertain/NegativeAnti-β2GP1 IgG/IgM Negative/Negative Negative/Negative Negative/NegativeReumathoid Factor Positive NegativeAnti-smooth muscle Negative Negative NegativeAnti-RNP Negative Negative NegativeC’3 1.37 (0.90 – 1.80) 1.70 (0.90 – 1.80) 1.17 (0.90 – 1.80)C’4 0.31 (0.10 – 0.40) 0.35 (0.10 – 0.40) 0.41 (0.10 – 0.40)Anti-mithocondria NegativeAnti-nucleosome Negative NegativeAnti-Scl-70 Negative NegativeAnti-centromere Negative NegativeAnti-histone NegativeC-ANCA / P-ANCA Negative/NegativeAnti-TPO 1.0 (<9)Anti-TG <0.9 (<4)ANA: anti-nuclear antibody; Anti-dsDNA: anti double-stranded DNA antibody; Anti-β2GP1:  anti beta2 glycoprotein 1 antibody; Anti-RNP:anti-ribonucleoprotein; Anti-Scl-70: anti topoisomerase I antibody; C-ANCA: Cytoplasmic antineutrophil cytoplasmic antibodies; P-ANCA:Perinuclear Anti-Neutrophil Cytoplasmic Antibodies; Anti-TPO: Anti-thyroid peroxidase antibody; Anti-TG: Anti-thyroglobulin antibodyCongenital complete atrioventricular block – case report and review of the literature320 Acta Obstet Ginecol Port 2015;9(4):318-321genital AVB or SLE-associated rash, concomitanttreatment with prednisone and gestational age under12 weeks were required. The authors concluded thatIV-Ig was ineffective in preventing congenital AVB orreducing maternal antibodies titers8,12. In the sameyear, Pisoni published a similar study with 24 pregnantwomen whose offspring in prior pregnancies deve -loped congenital AVB. Gestational age under 12 weeksand positive anti-SSA/Ro and/or anti-SSB/La weremandatory criteria for enrollment. Likewise, efficacyof IV-Ig in preventing congenital AVB was not esta -blished8,13.Hydroxychloroquine is the most recent line of in-vestigation for congenital AVB treatment. In 2010, aretrospective case-control study involving 50 childrenwhose mothers had SLE and documented anti--SSA/Ro and/or anti-SSB/La antibodies and 151controls found no significant differences in pregnan-cy outcomes between the group treated with hydro -xychloroquine and the control group. Nevertheless, theauthors suggested that hydroxychloroquine may de-crease the risk of developing neonatal cardiac SLE8,14.Currently available data does not demonstrate effi-cacy of any pharmacological treatment in preventing oravoiding progression of congenital heart block. Two--thirds of these children will require a pacemaker with-in the first year of life and some will develop car-diomyopathy1,2,9. After an affected pregnancy, the riskof another fetus with congenital AVB increases at least10-fold8.In our case fluorinated corticosteroids were not ef-fective in preventing progression to third degree AVB.The lack of prospective and randomized controlled tri-als that support a beneficial effect of steroids, IV-Ig orhydroxychloroquine for treatment of complete con-genital AVB requires a careful risk-benefit analysis be-fore initiating therapy.REFERENCES1. Eliasson H, Sonesson S-E, Sharland G, Granath F, SimpsonJ, Carvalho J, Jicinska H, Tomek V, Dangel J, Zielinsky P, Respon-dek-Liberska M, Freund M, Mellander M, Bartrons J, GardinerH. Isolated Atrioventricular Block in the Fetus: A Retrospective,Multinational, Multicenter Study of 175 Patients. Circulation2011;124:1929-1926. 2. Rein A, Mevorach D, Perles Z, Gavri S, Nadjari M, Nir, A,Elchalal U. Early Diagnosis and Trea-tment of AtrioventricularBlock in the Fetus Exposed to Maternal Anti-SSA/Ro-SSB/LaAntibo-dies: A Prospective, Observational, Fetal Kinetocardiogram– Based Study. Circulation 2009;119:867-1872. 3. Julkunen H, Kurki P, Kaaja R, Heikkilâ R, Immonen I, ChanE, Wallgren E, Friman C. Isolated Congenital Heart Block: Long-The child is now one year old has attended regularfollow up in a Pediatric Cardiology and is doing well.DISCUSSIONVirtually all mothers of fetuses with isolated AVB willhave a connective tissue disease - Systemic Lupus Ery-thematosus (SLE) or Sjögren Syndrome. Althoughsome may be completely asymptomatic4, the increasedrisk of developing a connective tissue disease requiresa tight surveillance. Anti-SSA/Ro and Anti-SSB/Laare the maternal autoantibodies most frequently asso-ciated with AVB3,5-9. These autoantibodies, cross theplacenta during the second trimester of pregnancy,bind to fetal cardiac myocytes that undergo apoptosiswith subsequent opsonization, altering calcium ho -meostasis and promoting a proinflammatory reactionthat may lead to conduction disturbances, myocardi-tis and arrhytmogenicity3-5,7,10. Intrauterine mortalityis higher when complete AVB is diagnosed before 20weeks of gestation, ventricular rate is below 50 bpm orwhen hydrops or impaired left ventricular function arepresent1. Once a complete AVB is established, pharmacologi -cal attempts at reversal have never been successful.However, first and second degree AVB may still be re-versible with in utero treatment, suggesting that severefibrosis has not yet occurred8. Various therapeutic ap-proaches have been reported, such as plasmapheresis,steroids, intravenous gamma globulin (IV-Ig), beta--adrenergic agents, cyclophosphamide, extracorpore-al immunoabsorption, azathioprine, hydroxychloro-quine and B cell depletion therapies. Most of them in-volve single case reports1,8.Several studies suggested that fluorinated steroids(betamethasone or dexamethasone), which cross theplacenta in their active form, would prevent progres-sion of the AVB or even promote its reduction2,4,5,11.So far, there is not enough evidence that supports be -ne fit in complete AVB. Side-effects on the fetus –oligohydramnios, fetal growth restriction and reducedcerebral growth – and on the mother – diabetes, hy-pertension and adrenal insufficiency1,5,10 – have to beconsidered before initiating steroid therapy.Few non-randomized, multi-center studies of ante-natal IV-Ig on AVB are available. In 2010 Friedmanpublished a study of 20 pregnant women with anti--SSA/Ro and/or anti-SSB/La antibodies treated withIV-Ig. To enroll in the study, a previous child with con-Ana Catarina Massa, Isabel Freitas, Augusta Borges, Fátima SerranoActa Obstet Ginecol Port 2015;9(4):318-321 321-Term Outcome of Mothers and Characterization of the ImmuneResponse to SS-A/Ro and to SS-B/La. Arthritis Rheum1993;36(11):1588-1598.4. Copel J, Buyon J, Kleinman C. Successful in utero therapy offetal heart block. Am J Obstet Gy-necol 1995;173:1384-1390.5. Mevorach D, Elchalal U, Rein A. Prevention of completeheart block in children of mothers with anti-SSA/Ro and anti--SSB/La autoantibodies: detection and treatment of first degreeatrioventri-cular block. Curr Opin Rheumatol 2009;21:478-482.6. Lee A, Coulter S, Erner S, Chu H. Cardiac ImmunglobulinDeposition in Congenital Heart Block Associated with MaternalAnti-Ro Autoantibodies. Am J Med 1987;83:793-796.7. Askanase AD, Friedman D, Copel J, Dische M, Dubin A,Starc T, Katholi M, Buyon J. Spectrum and progression of con-duction abnormalities in infants born to mothers with anti--SSA/Ro-SSB/La antibodies. Lupus 2002;11:145-151. 8. Gleicher N, Elkayam U. Preventing congenital neonatal heartblock in offspring of mothers with anti-SSA/Ro and SSB/La an-tibodies: A review of published literature and registered clinical tri-als. Autoimmun Rev 2013;12:1039-1045. 9. Friedman D, Kim M, Copel J, Llanos C, Davis C, Buyon J.Utility of Cardiac Monitoring in Fetu-ses at Risk for CongenitalHeart Block: The PR Interval and Dexamethasone Evaluation(PRIDE) Prospective Study. Circulation 2008;117:1485-1493. 10. Gardiner H, Belmar C, Pasquini L, Seale A, Thomas M,Dennes W, Taylor M, Kulinskaya E, Wimalasundera R. FetalECG: a novel predictor of atrioventricular block in anti-Ro positi-ve pre-gnancies. Heart 2007;93:1454-1460. 11. Raboisson M-J, Fouron J-C, Sonesson S-E, Nyman M,Proulx F, Gmache S. Fetal Doppler Echo-cardiographic Diagno-sis and Successful Steroid Therapy of Luciani-Wenckeback Phe-nomenon and Endocardial Fibroelastosis Related to MaternalAnti-Ro and Anti-La Antibodies. J Am Soc Echocardiog2005;375-380.12. Friedman D, Llanos C, Izmirly P, Brock B, Buron J, CopelJ, Cummiskey K, Dooley M, Foley J, Graves C, Hendershott C, Ka-tes R, Komissarova E, Miller M, Paré E, Phoon C, Prosen T, Reis-ner D, Ruderman E, Samuels P, Yu J, Kim M, Buyon J. Evaluationof fetuses in a study of intra-venous immunoglobulin as preventi-ve therapy for congenital heart block: Results of a multicenter, pros-pective, open-label clinical trial.Arthritis Rheum 2010;62(4):1138--1146.13. Pisoni C, Brucato A, Ruffati A, Espinosa G, Cervera R,Belmonte-Serrano M, Sánchez-Román J, Garcia-Hernández F,Tincani A, Bertero M, Doria A, Hughes G, Khamashta M. Failu-re of intra-venous immunoglobulin to prevent congenital heartblock: Findings of a multicenter, prospective, observational study.Arthritis Rheum 2010:62(4):1147-1152.14. Izmirly P, Kim M, Llanos C, Le P, Guerra M, Askanase A,Sakmon Jm Buyon J. Evaluation of the risk of anti-SSA/Ro-SSB/La antibody-associates cardiac manifestations of neonatal lu-pus in fetuses of mothers with systemic lupus erythematosus expo-sed to hydroxychloroquine. Ann Rheum Dis 2010:69(10):1827--1830.

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Bloqueio Aurículo-Ventricular Completo Congénito. Caso Clínico e Revisão da Literatura

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