AUTHOR=Fredi Micaela , Andreoli Laura , Bacco Beatrice , Bertero Tiziana , Bortoluzzi Alessandra , Breda Silvia , Cappa Veronica , Ceccarelli Fulvia , Cimaz Rolando , De Vita Salvatore , Di Poi Emma , Elefante Elena , Franceschini Franco , Gerosa Maria , Govoni Marcello , Hoxha Ariela , Lojacono Andrea , Marozio Luca , Mathieu Alessandro , Meroni Pier Luigi , Minniti Antonina , Mosca Marta , Muscarà Marina , Padovan Melissa , Piga Matteo , Priori Roberta , Ramoni Véronique , Ruffatti Amelia , Tani Chiara , Tonello Marta , Trespidi Laura , Zatti Sonia , Calza Stefano , Tincani Angela , Brucato Antonio 

TITLE=First Report of the Italian Registry on Immune-Mediated Congenital Heart Block (Lu.Ne Registry)

JOURNAL=Frontiers in Cardiovascular Medicine

VOLUME=6

YEAR=2019

URL=https://www.frontiersin.org/journals/cardiovascular-medicine/articles/10.3389/fcvm.2019.00011

DOI=10.3389/fcvm.2019.00011

ISSN=2297-055X

ABSTRACT=<p><bold>Objective:</bold> Neonatal Lupus (NL) is a rare syndrome caused by placental transfer of maternal anti-SSA/Ro and anti-La/SSB autoantibodies to the fetus. The rarity of this condition requires the establishment of multidisciplinary registries in order to improve our knowledge.</p><p><bold>Method:</bold> Inclusion criteria in this retrospective study were the maternal confirmed positivity for anti-SSA/Ro and/or anti-SSB/La antibodies, and the presence of II or III degree congenital heart block (CHB) <italic>in utero</italic> or neonatal period (up to 27 days after birth).</p><p><bold>Result:</bold> Eighty-nine cases of CHB were observed in 85 women with 88 pregnancies that occurred between 1969 and 2017. CHB was mostly detected <italic>in utero</italic> (84 cases, 94.2%), while five cases were observed in the neonatal period. A permanent pacemaker was implanted in 51 of 73 children born alive (69.8), whereas global mortality rate was 25.8% (23 cases): 16 <italic>in utero</italic>, five perinatal, and two during childhood. By univariate analysis, factors associated with fetal death were pleural effusion (<italic>p</italic> = 0.005, OR &gt; 100; CI 95% 2.88-&gt;100 and hydrops (<italic>p</italic> = 0.003, <italic>OR</italic> = 14.09; CI 95% 2.01–122). Fluorinated steroids (FS) were administered in 71.4% pregnancies, and its use was not associated with better survival. Some centers treated all cases with fluorinated steroids and some centers did not treat any case. CHB was initially incomplete in 24 fetuses, and of them five cases of II degree block reverted to a lower degree block after treatments. Recurrence rate in subsequent pregnancies was 17.6% (3 out of 17). A prophylactic treatment was introduced in 10 of these 16 subsequent (58.8%) pregnancies, mostly with FS or high dose intravenous immunoglobulins.</p><p><bold>Conclusion:</bold> This is the first report from the Italian Registry of neonatal lupus/CHB. The live birth rate was nearly 80%, with nearly two thirds of the children requiring the implantation of a pacemaker. The management of fetuses diagnosed with CHB was heterogeneous across Italian Centers. The registry at present is mainly rheumatological, but involvement of pediatric cardiologists and gynecologists is planned.</p>