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

Autor: Micaela Fredi, Laura Andreoli, Beatrice Bacco, Tiziana Bertero, Alessandra Bortoluzzi, Silvia Breda, Veronica Cappa, Fulvia Ceccarelli, Rolando Cimaz, Salvatore De Vita, Emma Di Poi, Elena Elefante, Franco Franceschini, Maria Gerosa, Marcello Govoni, Ariela Hoxha, Andrea Lojacono, Luca Marozio, Alessandro Mathieu, Pier Luigi Meroni, Antonina Minniti, Marta Mosca, Marina Muscarà, Melissa Padovan, Matteo Piga, Roberta Priori, Véronique Ramoni, Amelia Ruffatti, Chiara Tani, Marta Tonello, Laura Trespidi, Sonia Zatti, Stefano Calza, Angela Tincani, Antonio Brucato
Jazyk: angličtina
Rok vydání: 2019
Předmět:
0301 basic medicine
fluorinated steroids
congenital heart block
neonatal lupus
outcome
pregnancy
risk factors
therapy
Pediatrics
medicine.medical_specialty
lcsh:Diseases of the circulatory (Cardiovascular) system
anti SSA/Ro
Pleural effusion
030204 cardiovascular system & hematology
Cardiovascular Medicine
Congenital heart block
NO
03 medical and health sciences
Immune system
0302 clinical medicine
congenital heart block
neonatal lupus
outcome
pregnancy
risk factors
therapy
medicine
Neonatal lupus erythematosus
Original Research
lupus (SLE)
Pregnancy
Univariate analysis
Fetus
neonatal lupus erythematosus
business.industry
Obstetrics
General Commentary
Mortality rate
congenital heart block (CHB)
Retrospective cohort study
medicine.disease
Fluorinated steroids
030104 developmental biology
In utero
lcsh:RC666-701
Cardiology and Cardiovascular Medicine
business
Live birth
Zdroj: Frontiers in Cardiovascular Medicine, Vol 6 (2019)
Frontiers in Cardiovascular Medicine
Frontiers in Cardiovascular Medicine, Vol 7 (2020)
DOI: 10.3389/fcvm.2019.00011/full
Popis: Objective: 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.Method: 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) in utero or neonatal period (up to 27 days after birth).Result: Eighty-nine cases of CHB were observed in 85 women with 88 pregnancies that occurred between 1969 and 2017. CHB was mostly detected in utero (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 in utero, five perinatal, and two during childhood. By univariate analysis, factors associated with fetal death were pleural effusion (p = 0.005, OR > 100; CI 95% 2.88->100 and hydrops (p = 0.003, OR = 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.Conclusion: 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.
Databáze: OpenAIRE
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