title: Use of intravenous gamma globulin and corticosteroids in the treatment of maternal autoantibody-mediated cardiomyopathy.
abstract: OBJECTIVE
  This study sought to evaluate the outcome of maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis following intravenous gamma globulin (IVIG) and corticosteroid therapy.
  
  
  BACKGROUND
  We have previously shown that 85% of fetuses and infants with maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis suffer demise or need for transplant. In an attempt to improve this outcome, in 1998, we began to empirically treat affected patients with IVIG and corticosteroids.
  
  
  METHODS
  We reviewed the clinical records and echocardiograms of 20 affected patients encountered in our institutions and treated with IVIG and corticosteroids from 1998 to 2009.
  
  
  RESULTS
  All 20 were initially referred at a median gestational age of 23 weeks (range 18 to 38 weeks). Nineteen mothers were anti-Ro antibody positive, 8 anti-La antibody positive, and 7 had clinical autoimmune disease. Endocardial fibroelastosis was seen in 16 and was not obvious in 4 others with reduced ventricular function, and 16 (80%) had reduced or borderline ventricular shortening fraction (≤30%) before or after birth. Eighteen had atrioventricular block at referral (16 in 3°). During pregnancy, maternal IVIG was given in 9 and dexamethasone in 17. After birth, 17 infants received IVIG (n = 14) and/or corticosteroids (n = 15). Twelve underwent pacemaker implantation. Four with hydrops at presentation died perinatally, despite initial improvement in function in 3. At a median follow-up of 2.9 years (1.1 to 9.8 years), 16 (80%) patients are currently alive with normal systolic ventricular function and 6 are not paced.
  
  
  CONCLUSIONS
  Treatment of maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis with IVIG and corticosteroids potentially improves the outcome of affected fetuses. Further studies are needed to determine the optimal dose and timing of IVIG administration.
authors.affiliation: Pediatric Cardiology, University of California, San Francisco, USA.
authors.forename: Sara M
authors.initials: SM
authors.lastname: Trucco
authors.forename: Edgar
authors.initials: E
authors.lastname: Jaeggi
authors.forename: Bettina
authors.initials: B
authors.lastname: Cuneo
authors.forename: Anita J
authors.initials: AJ
authors.lastname: Moon-Grady
authors.forename: Earl
authors.initials: E
authors.lastname: Silverman
authors.forename: Norman
authors.initials: N
authors.lastname: Silverman
authors.forename: Lisa K
authors.initials: LK
authors.lastname: Hornberger
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D001323:Autoantibodies; D004695:Endocardial Fibroelastosis / Q000151:congenital / Q000188:drug therapy* / Q000276:immunology; D005260:Female; D005315:Fetal Diseases / Q000188:drug therapy* / Q000276:immunology; D054502:Histocompatibility, Maternal-Fetal; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000188:drug therapy / Q000276:immunology; D011247:Pregnancy; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous