title: Plasmapheresis, intravenous immunoglobulins and bethametasone - a combined protocol to treat autoimmune congenital heart block: a prospective cohort study.
abstract: OBJECTIVE
  At the moment there are no standard guidelines for the treatment of autoimmune congenital heart block (CHB). We set out to carry out a prospective cohort study to evaluate the benefits, limits, and safety of a combined therapy protocol to treat antibody-related CHB.
  
  
  METHODS
  Twelve consecutive pregnant patients positive to anti-SSA/Ro ± anti-SSB/La antibodies in whom CHB was detected were prospectively evaluated from 2009 to 2014. The treatment protocol consisted of: weekly plasmapheresis, fortnightly intravenous immunoglobulins (IVIG), and daily 4 mg betamethasone from CHB detection until delivery; IVIG was administered to the neonates soon after birth.
  
  
  RESULTS
  At the time CHB was detected, six of the foetuses presented atrioventricular blocks of 2(nd) degree type and six of 3(rd) degree type. Two of the foetuses with a 2(nd) degree block reverted to a 1st degree block and one to a normal atrioventricular conduction. The condition was stable throughout the pregnancy in the other three cases of 2(nd) degree block. All six 3(rd) degree blocks were stable during pregnancy and confirmed at birth. After a mean of 37.6 months ± 19.6 SD post-birth, the infants with 1st, normal sinus rhythm, and 2(nd) degree blocks at birth were all found to be stable. During the follow-up (29 months ± 19.8 SD), pacemakers were implanted in three of the six infants with 3(rd) degree blocks.
  
  
  CONCLUSIONS
  This combined therapy seems to be effective and safe in treating 2(nd) degree CHB, while its efficacy in treating 3rd degree CHB remains to be established.
authors.affiliation: Rheumatology Unit, Department of Medicine, University of Padua, Italy. amelia.ruffatti@unipd.it.
authors.forename: Amelia
authors.initials: A
authors.lastname: Ruffatti
authors.affiliation: Paediatric Cardiology Unit, Department of Women's and Children's Health, University of Padua, Italy.
authors.forename: Alessia
authors.initials: A
authors.lastname: Cerutti
authors.affiliation: Rheumatology Unit, Department of Medicine, University of Padua, Italy.
authors.forename: Maria
authors.initials: M
authors.lastname: Favaro
authors.affiliation: Rheumatology Unit, Department of Medicine, University of Padua, Italy.
authors.forename: Teresa
authors.initials: T
authors.lastname: Del Ross
authors.affiliation: Rheumatology Unit, Department of Medicine, University of Padua, Italy.
authors.forename: Antonia
authors.initials: A
authors.lastname: Calligaro
authors.affiliation: Rheumatology Unit, Department of Medicine, University of Padua, Italy.
authors.forename: Ariela
authors.initials: A
authors.lastname: Hoxha
authors.affiliation: Apheresis Unit, Blood Transfusion Service, University Hospital of Padua, Italy.
authors.forename: Piero
authors.initials: P
authors.lastname: Marson
authors.affiliation: Cardiology Unit, Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Italy.
authors.forename: Loira
authors.initials: L
authors.lastname: Leoni
authors.affiliation: Paediatric Cardiology Unit, Department of Women's and Children's Health, University of Padua, Italy.
authors.forename: Ornella
authors.initials: O
authors.lastname: Milanesi
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood; D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis / Q000276:immunology / Q000628:therapy*; D001623:Betamethasone / Q000008:administration & dosage* / Q000009:adverse effects; D015415:Biomarkers / Q000097:blood; D003131:Combined Modality Therapy; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D015150:Echocardiography, Doppler; D005260:Female; D005865:Gestational Age; D005938:Glucocorticoids / Q000008:administration & dosage* / Q000009:adverse effects; D006327:Heart Block / Q000097:blood / Q000151:congenital* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage* / Q000009:adverse effects; D007231:Infant, Newborn; D008297:Male; D010956:Plasmapheresis / Q000009:adverse effects; D011247:Pregnancy; D011446:Prospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D016216:Ultrasonography, Prenatal / Q000379:methods
chemical_list: D000974:Antibodies, Antinuclear; D015415:Biomarkers; D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; C035356:SS-A antibodies; C035357:SS-B antibodies; D001623:Betamethasone