Use of intravenous gamma globulin and corticosteroids in the treatment of maternal autoantibody-mediated cardiomyopathy.

Abstract

OBJECTIVES:

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: Sara M Trucco, Edgar Jaeggi, Bettina Cuneo, Anita J Moon-Grady, Earl Silverman, Norman Silverman, Lisa K Hornberger
• Journal: Journal of the American College of Cardiology (J Am Coll Cardiol) Vol. 57 Issue 6 Pg. 715-23 (Feb 08 2011) ISSN: 1558-3597 [Electronic] United States
• PMID: 21292131 (Publication Type: Journal Article, Multicenter Study)
• Copyright: Copyright © 2011 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

Chemical References

• Adrenal Cortex Hormones
• Autoantibodies
• Immunoglobulins, Intravenous

Topics

• Adrenal Cortex Hormones (therapeutic use)
• Adult
• Autoantibodies
• Endocardial Fibroelastosis (congenital, drug therapy, immunology)
• Female
• Fetal Diseases (drug therapy, immunology)
• Histocompatibility, Maternal-Fetal
• Humans
• Immunoglobulins, Intravenous (therapeutic use)
• Infant, Newborn
• Infant, Newborn, Diseases (drug therapy, immunology)
• Pregnancy
• Retrospective Studies
• Young Adult