The Italian Registry of Autoimmune Congenital Heart Block (Lu.Ne Registry): Report of the First Year of Activity

Micaela Fredi¹, Laura Andreoli², Tiziana Bertero³, Alesandra Bortoluzzi⁴, Silvia Breda⁵, Veronica Cappa⁶, Fulvia Ceccarelli⁷, Rolando Cimaz⁸, Salvatore De Vita⁹, Emma Di Poi¹⁰, Franco Franceschini¹¹, Maria Gerosa¹², Marcello Govoni⁴, Ariela Hoxha¹³, Andrea Lojacono¹⁴, Luca Marozio¹⁵, Alessandro Mathieu¹⁶, Antonina Minniti¹⁷, Marina Muscarà¹⁸, Melissa Padovan⁴, Matteo Piga¹⁶, Roberta Priori⁷, Véronique Ramoni¹⁹, Amelia Ruffatti¹³, Marta Tonello¹³, Sonia Zatti²⁰, Stefano Calza⁶, Antonio Brucato⁵ and Angela Tincani², ¹Department of Rheumatology and Clinical Immunology, Rheumatology and Clinical Immunology, Rheumatology and Clinical Immunology, Spedali Civili of Brescia, Brescia, Italy, ²Rheumatology and Clinical Immunology, Spedali Civili and University of Brescia, Brescia, Italy, ³Ospedale Mauriziano, Torino, Italy, ⁴UOC of Rheumatology, University Hospital S. Anna, Cona Ferrara, Italy, ⁵Struttura Complessa Medicina Interna, ASST Papa Giovanni XXIII, Bergamo, Italy, ⁶Unit of Biostatistics and Biomathematics & Unit of Bioinformatics, Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy, ⁷UO Complessa Reumatologia, Policlinico Umberto I Università Sapienza di Roma, Rome, Italy, ⁸Pediatric Rheumatology, Anna Meyer Children's Hospital-University of Firenze, Florence, Italy, ⁹Rheumatology Clinic, Academic Hospital S. M. della Misericordia, Medical Area Department, University of Udine, Italy, Udine, Italy, ¹⁰Clinica di Reumatologia, Azienda Sanitaria Universitaria Integrata di Udine, Udine, Italy, ¹¹Rheumatology and Clinical Immunology, Spedali Civili of Brescia, Brescia, Italy, ¹²Istituto Ortopedico Gaetano Pini, University of Milan, Milano, Italy, ¹³Unità di Reumatologia, Dipartimento di Medicina-DIMED, Università di Padova., Padova, Italy, ¹⁴Obstetrics and Gynecology, Spedali Civili and University of Brescia, Brescia, Italy, ¹⁵Ginecologia e Ostetricia 1, Dipartimento di Scienze Chirurgiche, Università di Torino, Torino, Italy, ¹⁶Unit and Chair of Rheumatology, University Hospital of Cagliari, Cagliari, Italy, ¹⁷UO Complessa Reumatologia, Policlinico Umberto I Università Sapienza di Roma, Roma, Italy, ¹⁸Reumatologia ASST Ospedale Niguarda, Milano, Italy, ¹⁹Rheumatology, Rheumatology, IRCCS Policlinico San Matteo Foundation and University of Pavia, Pavia, Italy, ²⁰U.O. Ginecologia e Ostetricia ASST Spedali Civili, Brescia, Italy

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: autoantibodies, heart block and pregnancy

Session Information

Date: Monday, November 6, 2017

Title: Reproductive Issues in Rheumatic Disorders Poster

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose:

Neonatal Lupus (NL) is a rare syndrome caused by placental transfer of maternal anti-Ro/SSA and anti-La/SSB autoantibodies to the fetus. Cardiac manifestations may comprise autoimmune congenital heart block (CHB) and dilated cardiomiopathy (DCM). The prevalence of CHB has been estimated as 1-2% in anti-Ro/SSA-positive women while the recurrence rate is 16-19%. The rarity of these conditions requires the establishment of collaborative registries in order to improve our knowledge on the management of CHB. Here we report the preliminary data of the Italian Registry of the autoimmune congenital heart block, which was created in the frame of the Italian Society for Rheumatology in 2016.

Methods:

The aim was to collect retrospective and prospective pregnancies complicated with CHB in patients with anti-Ro/SSA and/or anti-La/SSB antibodies. The study was approved by the Ethic Committee of the Promoting Centre in July 2016 and it has been approved by other eight Italian Institutions. Data regarding demography, maternal treatment before and after CHB detection, neonatal outcome, maternal and neonatal follow-up were collected through an online electronic datasheet prepared in a Research Electronic Data Capture (REDCap) platform. Two-tailed Student’s t-test for continuous variables, Fisher’s exact test or Yates’s Chi squared test for categorical variables were applied. The statistical software Stata/SE14.2 was used.

Results:

Seventy-five pregnancies complicated with the detection of CHB were collected in 73 women. CHB was detected in utero in 68 cases (90.6%), 5(6.7%) neonatal cases, 2(2.7%) unknown. Demographic description of the cohort, degree of CHB and treatment are reported in the table. The death of the child was observed in 17 (23.2%) cases: 7 fetal-deaths,5 termination of pregnancy and 5 postnatal death. Maternal and fetal risk factors for fetal mortality were analyzed for possible associations. At univariate analysis, factors associated with death were hydrops (p=0.065) and pericardial effusion (p=0.025).

Conclusion:

The Lu.Ne registry is an ongoing project aiming at collecting all cases of CHB referred to Italian Rheumatology Centres. Our preliminary data confirmed that hydrops and pericardial effusion are risk factors for fetal death. Conversely, we found that the majority of the mothers (58%) whose pregnancy was affected by CHB had a formal diagnosis of systemic autoimmune disease. This is in contrast with other registries showing that usually CHB was incidentally detected in healthy women. Probably such discrepancy is related to the recruiting Centres all belonging to Rheumatology Society. The collection of CHB cases from Gynecological and Pediatric Cardiology Centres, planned in the next months, will complete our analysis

Disclosure: M. Fredi, None; L. Andreoli, None; T. Bertero, None; A. Bortoluzzi, None; S. Breda, None; V. Cappa, None; F. Ceccarelli, None; R. Cimaz, None; S. De Vita, None; E. Di Poi, None; F. Franceschini, None; M. Gerosa, None; M. Govoni, None; A. Hoxha, None; A. Lojacono, None; L. Marozio, None; A. Mathieu, None; A. Minniti, None; M. Muscarà, None; M. Padovan, None; M. Piga, None; R. Priori, None; V. Ramoni, None; A. Ruffatti, None; M. Tonello, None; S. Zatti, None; S. Calza, None; A. Brucato, None; A. Tincani, None.

To cite this abstract in AMA style:

Fredi M, Andreoli L, Bertero T, Bortoluzzi A, Breda S, Cappa V, Ceccarelli F, Cimaz R, De Vita S, Di Poi E, Franceschini F, Gerosa M, Govoni M, Hoxha A, Lojacono A, Marozio L, Mathieu A, Minniti A, Muscarà M, Padovan M, Piga M, Priori R, Ramoni V, Ruffatti A, Tonello M, Zatti S, Calza S, Brucato A, Tincani A. The Italian Registry of Autoimmune Congenital Heart Block (Lu.Ne Registry): Report of the First Year of Activity [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/the-italian-registry-of-autoimmune-congenital-heart-block-lu-ne-registry-report-of-the-first-year-of-activity/. Accessed .

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