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The placental transference of maternal Ro\/La autoantibodies may damage the conduction tissues during fetal development leading to blocking of signal conduction at the atrioventricular (AV) node in an otherwise structurally normal heart. Irreversible complete AV block is the main cardiac manifestation of ACHB, but some babies may develop endocardial fibroelastosis, valvular insufficiency, and\/or frank cardiomyopathies with significantly reduced cardiac function requiring transplant. The severity of ACHB is illustrated by a global mortality rate of 20% and pacemaker rates of at least 64%, often within the first year of life. This review analyses the main complex and\/or unusual clinical situations associated with ACHB, including unusual maternal immunological profiles, infrequent maternal autoimmune diseases, cardiac damage unrelated to AV block, fetal invasive management, late complications after birth, risk of congenital heart block (CHB) in ovodonation and in\u00a0vitro fertilization techniques, the role of maternal features other than autoimmunity, the influence of the birth order or the risk of CHB in twins and triplets.<\/jats:p>","DOI":"10.1177\/0961203315624024","type":"journal-article","created":{"date-parts":[[2016,1,13]],"date-time":"2016-01-13T19:46:10Z","timestamp":1452714370000},"page":"116-128","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":44,"title":["Autoimmune congenital heart block: complex and unusual situations"],"prefix":"10.1177","volume":"25","author":[{"given":"P","family":"Brito-Zer\u00f3n","sequence":"first","affiliation":[{"name":"Josep Font Laboratory of Autoimmune Diseases, Department of Autoimmune Diseases, Hospital Cl\u00ednic, Institut d\u2019Investigacions Biom\u00e8diques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"PM","family":"Izmirly","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Medicine, New York University School of Medicine, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Ramos-Casals","sequence":"additional","affiliation":[{"name":"Josep Font Laboratory of Autoimmune Diseases, Department of Autoimmune Diseases, Hospital Cl\u00ednic, Institut d\u2019Investigacions Biom\u00e8diques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"JP","family":"Buyon","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Medicine, New York University School of Medicine, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"MA","family":"Khamashta","sequence":"additional","affiliation":[{"name":"Graham Hughes Lupus Research Laboratory, The Rayne Institute, Division of Women's Health, Kings College London St Thomas Hospital, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2016,1,13]]},"reference":[{"key":"bibr1-0961203315624024","doi-asserted-by":"crossref","unstructured":"Brito-Zer\u00f3n P, Izmirly PM, Ramos-Casals M, Buyon JP, Khamashta MA. 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