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ObjectiveIt is still a matter of debate whether low-dose acetylsalicylic acid (LDASA) should be prescribed to all patients with SLE during pregnancy. This study aimed at investigating the impact of LDASA on pregnancy outcomes in patients with SLE without history of renal involvement and without antiphospholipid antibodies (aPL).MethodsThis is a retrospective analysis of prospectively monitored pregnancies at seven rheumatology centres. Previous/current renal involvement and aPL positivity were the exclusion criteria. Adverse pregnancy outcome (APO) is the composite outcome of the study and included proteinuric pre-eclampsia, preterm delivery Results216 pregnancies in 187 patients were included; 82 pregnancies (38.0%) were exposed to LDASA treatment. No differences in terms of age at conception, disease duration, clinical manifestations, comorbidities and disease flare during pregnancy were observed between patients taking LDASA and those who did not take LDASA during pregnancy. APO was observed in 65 cases (30.1%), including 13 cases (6.1%) of pre-eclampsia. The incidence of all complications was similar in the two groups. However, it is interesting to note that pre-eclampsia had lower frequency in patients taking LDASA versus those not taking LDASA (2.4% vs 8.3%, p=0.14).ConclusionsIn pregnant patients with SLE without renal involvement and were aPL-negative, there is a low risk of severe obstetric complications, such as early pre-eclampsia. LDASA treatment does not provide a statistically significant advantage over these complications. However, a careful individual risk–benefit balance is warranted.

Absent pulmonary valve syndrome is a rare congenital heart disease characterized by absent or rudimentary pulmonary valve leaflets and hypoplastic pulmonary annulus. The most common variant, associated with tetralogy of Fallot, implies dilatation of pulmonary branches and usually absent ductus arteriosus. Rarely, absent pulmonary valve occurs with intact ventricular septum: pulmonary branches are normally sized or mildly dilated and ductus arteriosus is usually patent. The rarest type is associated with intact ventricular septum and tricuspid atresia. A close connection has been raised between pulmonary regurgitation and development of tricuspid valve. We describe a case of prenatal diagnosed absent pulmonary valve with intact ventricular septum and patent ductus arteriosus in which severe pulmonary regurgitation caused reversible functional atresia of tricuspid valve. Postnatally, satisfactory biventricular circulation was obtained with inotropic support for a few days while ductus arteriosus closed spontaneously. At a 3-year follow up the child is asymptomatic without therapy.

Objectives To determine whether disease remission or low disease activity state at the beginning of pregnancy in SLE patients is associated with better pregnancy outcome. Methods Pregnancies in SLE patients prospectively monitored by pregnancy clinics at four rheumatology centres were enrolled. Patient demographics and clinical information were collected at baseline (pregnancy visit before 8 weeks of gestation) including whether patients were in remission according to the Definition of Remission in SLE (DORIS) criteria and and/or Lupus Low Disease Activity State (LLDAS). Univariate and multivariate analysis were performed to determine predictors of disease flare and adverse pregnancy outcomes (APOs) including preeclampsia, preterm delivery, small for gestational age infant, intrauterine growth restriction and intrauterine fetal death. Results A total of 347 pregnancies were observed in 281 SLE patients. Excluding early pregnancy losses, 212 pregnancies (69.7%) occurred in patients who were in remission at baseline, 33 (10.9%) in patients in LLDAS, and the remainder in active patients. Seventy-three flares (24%) were observed during pregnancy or puerperium, and 105 (34.5%) APOs occurred. Multivariate analysis revealed that patients in disease remission or taking HCQ were less likely to have disease flare, while a history of LN increased the risk. The risk of APOs was increased in patients with shorter disease duration, while being on HCQ resulted a protective variable. An almost significant association between complete remission and a decreased risk of APOs was observed. Conclusions Prenatal planning with a firm treat-to-target goal of disease remission is an important strategy to reduce the risk of disease flares and severe obstetric complications in SLE pregnancies.

Background The attainment of remission and low disease activity (LLDAS) in SLE is associated with better outcomes. This study was aimed to test whether disease remission and LLDAS at the beginning of pregnancy are associated with better pregnancy outcomes. Methods A total of 85 pregnancies in 61 SLE patients prospectively followed at a single center were considered; 10 pregnancies ended in spontaneous abortion during the first trimester and were excluded from the analysis. Definitions of remission (according to DORIS criteria) and LLDAS were applied at the first pregnancy visit (7–8 weeks of gestational age). Disease flare was defined according to the SELENA-SLEDAI flare index. Obstetric complications included pretermprelabour rupture of membranes, preeclampsia, preterm delivery, SGA infant, IUGR, intrauterine fetal death (IUFD) and gestational diabetes. Results Characteristics of the cohort are detailed in table 1. In twenty-one cases (28%) we observed flare during pregnancy or puerperium. Two severe flares and 19 mild-moderate flares were recorded. Obstetric complications were observed in 31 cases (41%). The risk of disease flare during pregnancy was significantly lower in patients in LLDAS at the beginning of pregnancy with respect to active patients (OR 12.47, p Conclusions These data confirm that disease remission is one important predictor of pregnancy outcomes in SLE. While serology seems not to have a substantial role, a residual disease activity at conception might impact on pregnancy outcomes. Available definitions of remission and LLDAS could be valid treatment targets in the family planning perspective.

Uterine anomalies result from the failure of complete fusion of the Müllerian ducts during embryogenesis. A unicornuate uterus with a rudimentary horn is the rarest anomaly and results from the failure of one of the Müllerian ducts to develop completely and an incomplete fusion with the contralateral side. Diagnosis and surgical management of a 5-week ectopic pregnancy in a non-communicating rudimentary horn in an 18-year-old nulliparous woman in whom this congenital uterine anomaly was previously unknown are described., Highlights • The incidence varies from 1 per 76000 pregnancies to 1 per 140000. • Ultrasound sensitivity ranges from 33% to 14% in non-symptomatic cases. • The major risk is a rupture during the second or third trimester. • After diagnosis, immediate laparoscopic surgery is most commonly recommended. • Ultrasound during early obstetric scan is the key for a correct management.