Pregnancy-related aortic complications in women with bicuspid aortic valve.

OBJECTIVES: To describe the aortic-related risks associated with pregnancy in women with bicuspid aortic valve (BAV) and to evaluate changes in aortic diameter in pregnancy. METHODS: Prospective observational study of patients with BAV from a single-site registry of pregnant women with structural heart disease between 2013 and 2020. Cardiac, obstetric and neonatal outcomes were studied. An assessment of aortic dimensions was performed during pregnancy by two-dimensional echocardiography. Aortic diameters were measured at the annulus, root, sinotubular junction and maximum ascending aorta diameter, and the largest diameter was used. Measurements of the aorta were made using the end-diastolic leading edge-to-leading edge convention. RESULTS: Forty-three women (32.9 years, IQR 29.6-35.3) with BAV were included: 9 (20.9%) had repaired aortic coarctation; 23 (53.5%) had moderate or severe aortic valve disease; 5 (11.6%) had a bioprosthetic aortic valve; and 2 (4.7%) had a mechanical prosthetic aortic valve. Twenty (47.0%) were nulliparous. The mean aortic diameter in the first trimester was 38.5 (SD 4.9) mm, and that in the third trimester was 38.4 (SD 4.8) mm. Forty (93.0%) women had an aortic diameter of <45 mm; 3 (7.0%) had 45-50 mm; and none had >50 mm. Three women (6.9%) with BAV presented cardiovascular complications during pregnancy or the postpartum period (two prosthetic thrombosis and one heart failure). No aortic complications were reported. There was a small but significant increase in aortic diameter during pregnancy (third trimester vs first trimester, 0.52 (SD 1.08) mm; p=0.03). Obstetric complications appeared in seven (16.3%) of pregnancies, and there were no maternal deaths. Vaginal non-instrumental delivery was performed in 21 (51.2%) out of 41 cases. There were no neonatal deaths, and the mean newborn weight was 3130 g (95% CI 2652 to 3380). CONCLUSIONS: Pregnancy in BAV women had a low rate of cardiac complications with no aortic complications observed in a small study group. Neither aortic dissection nor need for aortic surgery was reported. A low but significant aortic growth was observed during pregnancy. Although requiring follow-up, the risk of aortic complications in pregnant women with BAV and aortic diameters of <45 mm at baseline is low.

Expected impact of immunomodulatory agents during pregnancy: A newborn's perspective.

The neonatal immune ontogeny begins during pregnancy to ensure that the neonate is well-suited for perinatal life. It prioritizes Th2/M2 and regulatory responses over Th/M1 activity to avoid excessive inflammatory responses and to ensure immune tolerance and homeostasis. Newborns also present increased Th17/Th22 responses providing effective anti-fungal immunity and mucosal protection. Intrauterine exposure to immune modulatory drugs with the placental transfer may influence the natural course of the fetal immune development. The vertical transfer of both biological therapy and small molecules begins during the first trimester through neonatal Fc receptor or placental diffusion, respectively, reaching its maximum transfer potential during the third trimester of pregnancy. Most of the biological therapy have a prolonged half-life in newborn's blood, being detectable in infants up to 12 months after birth (usually 6-9 months). The use of immunomodulators during pregnancy is gaining global interest. Current evidence mainly reports birth-related outcomes without exhaustive analysis of the on-target side effect on the perinatal immune system ontogeny, the infection risk, or the immune dysregulation. The present review will focus on: (1) the main characteristics of the perinatal immune system to understand its specific features and vulnerabilities to immune modulation; (2) the mechanisms of placental transfer of immunomodulators; and (3) the immune changes reported to date in newborns exposed to immunomodulators with emphasis on the current concerns and gaps in knowledge.

Multidisciplinary models for pregnancy care in patients with rheumatic diseases: Clinical experiences and experts opinion.

OBJECTIVES: To describe different models of multidisciplinary pregnancy care for patients with inflammatory and autoimmune rheumatic diseases, and the steps to follow concerning their implementation. METHODS: A qualitative study was conducted including: (1) a comprehensive literature search in PUBMED focused on multidisciplinary care models; (2) structured interviews with seven rheumatologists from multidisciplinary pregnancy clinics for patients with inflammatory and autoimmune rheumatic diseases. Data were collected related to the hospitals, medical departments, populations cared for, and multidisciplinary care models (type, material, and human resources, professional requirements, objectives, referral criteria, agendas, protocols, responsibilities, decision-making, research and educational activities, multidisciplinary clinical sessions, initiation/start, planning, advantages/disadvantages, and barriers/facilitators for implementation); (3) a nominal meeting group in which the results of searches and interviews were analyzed and the recommendations for the implementation of the multidisciplinary care models defined. RESULTS: We analyzed seven models of multidisciplinary care in pregnancy, implemented 3-10 years ago, which can all be summarized by two different subtypes: parallel (patients are assessed the same day in the involved medical services) and preferential (patients are assessed on different days in the involved medical services) circuits. The implementation of a specific model results rather from an adaptation to the hospital's and professionals' circumstances. Correct planning and good harmony among professionals are key points to implementing a model. CONCLUSION: Different multidisciplinary care models have been implemented for patients with inflammatory and autoimmune rheumatic diseases during pregnancy. They pretend to improve care, system efficiency, and collaboration among specialists and should be carefully implemented.

Control del embarazo en pacientes con lupus eritematoso sistémico/síndrome antifosfolípido. parte 2: seguimiento del embarazo / Pregnancy control in patients with systemic lupus erythematosus/antiphospholipid syndrome. Part 2: Pregnancy follow-up

Objetivo: Las sociedades españolas de ginecología y obstetricia, de medicina interna y de reumatología han constituido un grupo de trabajo paritario para la elaboración de 3 documentos de consenso sobre el control del embarazo en mujeres con lupus eritematoso sistémico (LES) y síndrome antifosfolípido (SAF). Métodos: Cada una de las sociedades científicas implicadas propuso 5 representantes en base a su experiencia en el área del control del embarazo en pacientes con enfermedades autoinmunes. Las recomendaciones se elaboraron siguiendo la metodología Delphi. Resultados: En este segundo documento se incluyen las recomendaciones que abordan el manejo del embarazo normal y sus complicaciones en mujeres con LES/SAF. Se presentan las recomendaciones relacionadas con el seguimiento del embarazo, la actividad lúpica, el bloqueo cardíaco congénito, las manifestaciones trombóticas y obstétricas del SAF y los defectos de placentación. Conclusiones: Estas recomendaciones multidisciplinares se consideran herramientas en la toma de decisiones para los clínicos involucrados en la asistencia a pacientes con LES/SAF durante el embarazo.(AU)

Objective: In order to agree on the fundamental aspects related to the management of pregnancy in patients with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS), the Spanish Societies of Gynaecology and Obstetrics, Internal Medicine and Rheumatology set up a working group for the preparation of three consensus documents. Methods: Each of the Scientific Societies involved proposed five representatives based on their experience in the field of pregnancy control in patients with autoimmune diseases. The recommendations were developed following the Delphi methodology. Results: This second document contains the recommendations regarding the management of pregnancy in women with SLE and APS, including complications such as lupus activity, congenital heart block, thrombotic and obstetric manifestations of APS and placental vascular disease. Conclusions:These multidisciplinary recommendations are considered decision-making tools for clinicians involved in the care of patients with SLE/APS during pregnancy.(AU)

Pregnancy control in patients with systemic lupus erythematosus/antiphospholipid syndrome. Part 2: Pregnancy follow-up. / Control del embarazo en pacientes con lupus eritematoso sistémico/síndrome antifosfolípido. parte 2: seguimiento del embarazo.

OBJECTIVE: In order to agree on the fundamental aspects related to the management of pregnancy in patients with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS), the Spanish Societies of Gynaecology and Obstetrics, Internal Medicine and Rheumatology set up a working group for the preparation of three consensus documents. METHODS: Each of the Scientific Societies involved proposed five representatives based on their experience in the field of pregnancy control in patients with autoimmune diseases. The recommendations were developed following the Delphi methodology. RESULTS: This second document contains the recommendations regarding the management of pregnancy in women with SLE and APS, including complications such as lupus activity, congenital heart block, thrombotic and obstetric manifestations of APS and placental vascular disease. CONCLUSIONS: These multidisciplinary recommendations are considered decision-making tools for clinicians involved in the care of patients with SLE/APS during pregnancy.

Pregnancy control in Patients with Systemic Lupus Erythematosus/Antiphospholipid Syndrome. Part 3: Childbirth. Puerperium. Breastfeeding Contraception. Newborn. / Control del embarazo en pacientes con lupus eritematoso sistémico/síndrome antifosfolípido. Parte 3. Parto. Puerperio. Lactancia. Anticoncepción. Recién nacido.

OBJECTIVE: In order to agree on the fundamental aspects related to the management of pregnancy in patients with systemic lupus erythematosus and antiphospholipid syndrome, the Spanish Societies of Gynaecology and Obstetrics, Internal Medicine and Rheumatology have set up a working group for the preparation of three consensus documents. METHODS: Each of the Scientific Societies involved proposed five representatives based on their experience in the field of pregnancy control in patients with autoimmune diseases. The recommendations were developed following the Delphi methodology. RESULTS: This third document contains the recommendations regarding the management of delivery, puerperium and lactation, including medication use during these periods and the care of the newborn. In addition, a section on contraception is included. CONCLUSIONS: These multidisciplinary recommendations are considered decision-making tools for clinicians involved in the care of patients with systemic lupus erythematosus/antiphospholipid syndrome during pregnancy.

Intravenous immunoglobulin as monotherapy for myasthenia gravis during pregnancy.

INTRODUCTION: Pregnant women with myasthenia gravis (MG) are at increased risk of complications and adverse outcomes, including the teratogenic effects of many drugs used to treat MG women of childbearing age. The effectiveness of intravenous immunoglobulins (IVIg) on other autoimmune mediated diseases has been extensively reported in recent years, although little is known about the role of IVIg in the treatment of MG during pregnancy. We designed this study to determine the effectiveness of IVIg as monotherapy during pregnancy for women with MG. MATERIAL AND METHODS: Five pregnant MG patients (mean age at delivery 36.4years, SD 5.8, range 29.4-45.2) were studied in 2013-14. Their treatment was switched to monthly IVIg cycles 2months before the pregnancy. Follow-up included monthly neurological QMG throughout the pregnancy and postpartum, obstetrical monitoring during monthly visits in the first two trimesters of the pregnancy, fortnightly visits between week 32 and week 36, and weekly visits after 36weeks, and neonatal follow-up after delivery. RESULTS: We observed no exacerbations during pregnancy, delivery or post-partum. The mean QMG score at baseline (before pregnancy) was 7.4 points in five women with generalized forms of MG. The maximum mean value reached during pregnancy was 8.6 points. The mean pregnancy duration was 38 w+5 d. No infant with transient neonatal myasthenia gravis. CONCLUSIONS: These results suggest that monotherapy with IVIg during pregnancy in MG patients could be promising, although confirmation is required in studies with larger populations.

Predictors of obstetric complications in women with heart disease.

OBJECTIVES: The objective of this study is to evaluate obstetric outcomes in women with heart disease and determine whether current multidisciplinary management approaches adversely affect the mother, the neonate, or both. Also to compare the accuracy of several risk scores (RS) including the modified World Health Organization classification (mWHO) and CARPREG to predict obstetric and neonatal complications and to study the addition value of Uteroplacental-Doppler flow (UDF) parameters to predict obstetric complications. METHODS: A prospective cohort study examined outcomes in women with heart disease (HD), the majority of whom had corrective surgery and delivered between January 2007 and March 2012. RESULTS: One hundred and seventy-four patients with 179 pregnancies were included in the study. Obstetric complications, including premature labor, arose in 87 patients (48.6%). Neonatal complications were observed in 11 cases (7%). On multivariate analysis, maternal heart disease was predictive of adverse perinatal events (46 cases, 25.7%) and mode of delivery (Thierry's spatula) of third- or fourth-degree perineal tears (six cases, 3.2%). mWHO classification predicted obstetric complications (p = 0.0001) better than the CARPREG study. Impaired UDF (uterine artery pulsatility index-20 weeks and umbilical artery pulsatility index-32 weeks in HD versus healthy women: 20w 1.12 versus 1.34, p = 0.005; 32w 0.87 versus 1.09, p = 0.008) was associated with adverse obstetric and offspring outcome in the group of HD pregnant women. CONCLUSIONS: Nearly 50% of pregnancies were associated with an adverse obstetric outcome, particularly IUGR. mWHO was better at predicting obstetric and neonatal complications that CARPREG in all categories. Furthermore, compromised UDF combined with mWHO improved the prediction of obstetric and offspring complications in this population.

Physical examination-indicated cerclage in singleton and twin pregnancies: maternal-fetal outcomes.

OBJECTIVES: To study maternal and perinatal outcomes after physical examination-indicated cerclage in both singleton and twin pregnancies and evaluate the possible risk factors associated. STUDY DESIGN: Retrospective review of all women undergoing physical examination-indicated cerclage at the Hospital Vall d'Hebro, Barcelona from January 2009 to December 2012 after being diagnosed with cervical incompetence and risk of premature birth. RESULTS: During the study period, 60 cases of women diagnosed with cervical incompetence who were carrying live and morphologically-normal fetuses (53 singleton and 7 twin pregnancies), and who had an imminent risk of premature birth were evaluated. Mean gestational age until birth was 35 weeks in singleton and 32 weeks in twin pregnancies. Four cases (7.5%) of immature births and one case (2.0%) of neonatal death were recorded in singleton pregnancies. No cases of immature births or neonatal deaths were recorded in twin pregnancies. Diagnostic amniocentesis was performed IN all cases to rule out possible chorioamnionitis. CONCLUSIONS: Physical examination-indicated cerclage for cervical incompetence in women at risk for immature or preterm birth demonstrates good perinatal prognosis without increasing maternal morbidity in either singleton or twin pregnancies. The increase in gestation time in our study may also have been due to the fact that patients with subclinical chorioamnionitis were excluded by diagnostic amniocentesis.

Utilización de heparinas de bajo peso molecular para la profilaxis y el tratamiento de la enfermedad tromboembólica en pacientes embarazadas / Use of low molecular weight heparin for thromboprophylaxis and treatment of venous thromboembolism in pregnant women

Estudio observacional que valoró la seguridad de heparina de bajo peso molecular (HBPM) administrada para la profilaxis o el tratamiento de complicaciones tromboembólicas durante el embarazo. Diseño: Se revisaron retrospectivamente historias clínicas y se recogió el resultado del embarazo y los acontecimientos adversos. El riesgo tromboembólico (ETEV) se analizó mediante la clasificación del Royal College of Obstetrics and Gynaecologist. Resultados: Se incluyeron 127 pacientes (131 fetos) con edad media de 32,3 ± 4,3 años. La HBPM se indicó por ETEV aguda en 11 (8,6%) pacientes y por profilaxis en 116 (91,4%). En el grupo de profilaxis hubo 38 (30,0%), 49 (38,6%) y 29 (22,8%) pacientes con riesgo de ETEV alto, moderado y bajo respectivamente. Los nacidos vivos fueron 127 (97%) —19 (15,1%) pretérmino—. Una paciente con déficit de antitrombina desarrolló ETEV. Hubo 25 casos de sangrado (18 [72%] hematomas subcutáneos). Conclusión: La HBPM es bien tolerada y segura para la profilaxis y el tratamiento de las complicaciones tromboembólicas del embarazo (AU)

This observational study evaluated the safety of low molecular weight heparin (LMWH) for the prophylaxis and treatment of thromboembolic complications in pregnancy. Study Design: The medical records of pregnant women were identified and reviewed retrospectively. Information was extracted on LMWH use, pregnancy outcome, and adverse events. The Guidelines of the Royal College of Obstetrics and Gynaecologists were used to evaluate the thromboembolic risk. Results: Data were collected on 127 pregnancies (131 fetuses); the mean age was 32.3 ± 4.3. LMWH was prescribed for acute venous thromboembolism in 11 patients (8.6%) and for prophylaxis in 116 (91.4%). For the prophylaxis group, there were 38 (30.0%), 49 (38.6%) and 29 (22.8%) patients with high, moderate and low venous thromboembolism risk, respectively. There were 127 (97%) live births (19 preterm [15.1%]). In the prophylaxis group, one venous thromboembolism occurred in a patient with antithrombin deficiency. Safety outcomes included 25 cases of bleeding (18 [72%] were injection site hematomas). Conclusion: We found that LMWH was well tolerated and safe for the prophylaxis and treatment of thromboembolic complications during pregnancy (AU)

Inherited antithrombin deficiency and pregnancy: maternal and fetal outcomes.

OBJECTIVE: To describe the outcome of pregnancy in women with inherited antithrombin (AT) deficiency. STUDY DESIGN: A descriptive retrospective study was performed. Medical records were reviewed in order to collect data about maternal thrombotic complications and pregnancy outcomes. All women with known inherited AT deficiency and at least one pregnancy looked after at the Vall d'Hebron University Hospital were included. Relatives with known AT deficiency but no pregnancies looked after in our institution were excluded. Eighteen pregnancies were registered among nine AT-deficient women during 1991-2005. This cohort included women without antithrombotic treatment because AT deficiency was not known at the time of their pregnancies. RESULTS: In 12 pregnancies (66.7%) anticoagulant therapy with low-molecular weight heparin was given, while not in the other six (33.3%) because AT deficiency was not known at this time. Three episodes of venous thromboembolism were recorded (16.7%). Among all pregnancies 10 suffered an adverse outcome (55.6%), including miscarriage (11.1%), stillbirth (11.1%), intrauterine growth restriction (33.3%), placental abruption (6.7%), preeclampsia (6.7%) and intrapartum fetal distress (23.1%). No relation between AT activity and pregnancy complications was found. A lower incidence of pregnancy complications was observed among women with antithrombotic treatment. CONCLUSIONS: Inherited antithrombin deficiency is associated with a high risk of venous thromboembolism during pregnancy and the puerperium. We also observed a high incidence of poor pregnancy outcome among AT-deficient women.

Paciente con betatalasemia mayor y 2 embarazos a término / Beta-thalassemia major and pregnancy to term

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