title: Autoimmune-associated congenital heart block: treatment of the mother with immunoadsorption.
abstract: Autoimmune-associated congenital heart block (CHB) is a rare complication of pregnancy in mothers with Anti-Ro/SSA antibodies (SSA-abs), resulting in fetal myocarditis, atrioventricular block, hydrops fetalis and/or intrauterine fetal death. As these antibodies are supposed to be directly involved in the pathogenesis of CHB, their removal should be associated with an improved clinical course. Extracorporeal immunoadsorption (IA) is the most efficient method to remove IgG-immunoglobulins like SSA-abs selectively. Two women with high titers of those auto-antibodies [mothers serum 615 and 612, respectively (normal range <3.0 IU/mL)] were treated with IA two to three times per gestation week in the outpatient department of the University of Rostock. In both patients, the mean removal of IgG (65 +/- 6%) to a target near 2.0 g/L after IA was successful. The SSA-abs were reduced from mean 328 +/- 138 and 247 +/- 105 pre IA to 88 +/- 124 and 98 +/- 42 post IA, respectively. One child received a pacemaker due to the persisting atrioventricular block grade III after birth. The second was unaffected. The removal of highly elevated SSA-antibodies by immunoadsorption is a possible treatment option in pregnant woman with high titers of those antibodies and/or a positive history of clinical complications. Further clinical studies are necessary.
authors.affiliation: Department of Nephrology, University of Rostock, Rostock, Germany. heiko.hickstein@med.uni-rostock.de
authors.forename: Heiko
authors.initials: H
authors.lastname: Hickstein
authors.forename: Thomas
authors.initials: T
authors.lastname: Külz
authors.forename: Renate
authors.initials: R
authors.lastname: Claus
authors.forename: Jan
authors.initials: J
authors.lastname: Stange
authors.forename: Reinhard
authors.initials: R
authors.lastname: Schmidt
mesh_terms: D000327:Adsorption; D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000628:therapy*; D018376:Cardiovascular Abnormalities / Q000517:prevention & control*; D005260:Female; D005333:Fetus / Q000276:immunology / Q000503:physiopathology; D019084:Fluorescent Antibody Technique, Indirect; D005858:Germany; D005865:Gestational Age; D006327:Heart Block / Q000151:congenital / Q000503:physiopathology / Q000517:prevention & control*; D006439:Hemodynamics; D006801:Humans; D007163:Immunosorbent Techniques; D011247:Pregnancy; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies