Health Outcomes of 215 Mothers of Children With Autoimmune Congenital Heart Block: Analysis of the French Neonatal Lupus Syndrome Registry.

Objective: Transplacental passage of maternal anti-SSA and anti-SSB antibodies, potentially associated with maternal autoimmune diseases, can cause neonatal lupus syndrome. Given the paucity of data in this setting, we report short- and long-term outcomes of mothers of offspring with congenital heart block (CHB).

Methods: This retrospective study included anti-SSA/SSB antibody-positive mothers of fetuses with high-degree CHB and focused on their health status before pregnancy, at CHB diagnosis, and thereafter.

Pregnancy-associated pelvic vein thrombosis: Insights from a multicenter case series.

Background: Pelvic vein thrombosis (PVT) is a rare complication of pregnancy that can lead to life-threatening complications, such as pulmonary embolism (PE).

Objective: To describe characteristics of PVT and its treatment in pregnancy in the province of Quebec, Canada.

Patients/methods: We developed a province-wide case series of PVT in pregnancy including four tertiary care centers and the Registry of Rare Diseases of the Groupe d'Étude en Médecine Obstétricale du Québec.

Routine repeated echocardiographic monitoring of fetuses exposed to maternal anti-SSA antibodies: time to question the dogma.

In around 1% of exposed pregnancies, anti-Ro/SSA and anti-La/SSB antibodies lead to congenital heart block, the main feature of neonatal lupus syndrome. As such, echocardiographic screening to detect congenital heart block, done every other week from 16 weeks to at least 24 weeks gestation, is widely recommended for anti-SSA-positive pregnant women. Such screening is now routinely done in many centres worldwide.

Effect of in utero hydroxychloroquine exposure on the development of cutaneous neonatal lupus erythematosus.

Objective: Cutaneous neonatal lupus (cNL) occurs in possibly 5%-16% of anti-Ro±anti-La antibody-exposed infants. Data suggest in utero exposure to hydroxychloroquine (HCQ) may prevent cardiac NL. The aim was to assess whether in utero exposure to HCQ decreases the risk of cNL and/or delays onset.

Association of Natural Killer Cell Ligand Polymorphism HLA-C Asn80Lys With the Development of Anti-SSA/Ro-Associated Congenital Heart Block.

Objective: Fetal exposure to maternal anti-SSA/Ro antibodies is necessary but not sufficient for the development of autoimmune congenital heart block (CHB), suggesting that other factors, such as fetal genetic predisposition, are important. Given the previously described association between major histocompatibility complex alleles and CHB risk, we undertook the present study to test the hypothesis that a variant form of HLA-C Asn80Lys, which binds with high affinity to an inhibitory killer cell immunoglobulin-like receptor (KIR) and thus renders natural killer (NK) cells incapable of restricting inflammation, contributes to the development of CHB.

Methods: Members of 192 pedigrees in the US and Europe (194 cases of CHB, 91 unaffected siblings, 152 fathers, 167 mothers) and 1,073 out-of-study controls were genotyped on the Immunochip single-nucleotide polymorphism microarray.

Incidence, risk factors, and mortality of neonatal and late-onset dilated cardiomyopathy associated with cardiac neonatal lupus.

Background: Dilated cardiomyopathy (DCM), a well-known complication of cardiac neonatal lupus, is associated with high mortality rate. Its risk factors remain unclear.

Methods: We analyzed occurrence of postnatal DCM among children with high-degree congenital heart block (CHB) and mothers with anti-SSA and/or anti-SSB antibodies.

Description of 214 cases of autoimmune congenital heart block: Results of the French neonatal lupus syndrome.

Background: Cardiac neonatal lupus syndrome is due to anti-SSA or SSB antibodies and mainly includes congenital heart block (CHB) and dilated cardiomyopathy (DCM). Its optimal management is still debated. We report a large series of autoimmune high degree CHB.

Catastrophic antiphospholipid syndrome and pregnancy: an experience of 13 cases.

Objective: Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening disease caused by the onset of rapidly progressive and widespread small-vessel thromboses in the presence of aPLs. The aim of this study was to examine pregnancy-related CAPS.

Methods: Retrospective series of 13 patients with pregnancy-related CAPS with special focus on the follow-up.