Autoimmune congenital heart block: complex and unusual situations

Brito-Zerón, Pilar; Izmirly, Peter; Ramos‐Casals, Manuel; Buyon, Jill P.; Khamashta, M. A.

doi:10.1177/0961203315624024

Cited by 42 publications

(28 citation statements)

References 121 publications

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“…While the overwhelming majority of ACHB cases involve anti-Ro positive mothers, isolated anti-La associated cases have also been reported, representing less than 1% of total cases [70–72]. Therefore recommendations for screening and therapy apply to mothers positive for either autoantibody.…”

Section: Pregnancy Considerations In Autoimmune Connective Tissue Dismentioning

confidence: 99%

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Pregnancy and autoimmune connective tissue diseases

Marder

Littlejohn

Somers

2016

Best Practice & Research Clinical Rheumatology

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The autoimmune connective tissue diseases predominantly affect women and often occur during the reproductive years. Thus, specialized issues in pregnancy planning and management are commonly encountered in this patient population. This chapter provides a current overview of pregnancy as a risk factor for onset of autoimmune disease, considerations related to the course of pregnancy in several autoimmune connective tissue diseases, and disease management and medication issues before and during pregnancy and the postpartum period. A major theme that has emerged across these inflammatory diseases is that active maternal disease during pregnancy is associated with adverse pregnancy outcomes, and that maternal and fetal health can be optimized when conception is planned during times of inactive disease and through maintaining treatment regimens compatible with pregnancy.

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Section: Pregnancy Considerations In Autoimmune Connective Tissue Dismentioning

confidence: 99%

“…The estimated recurrence of heart block in subsequent pregnancies for a mother with a previous child born with ACHB is around 18% [74]. Among children born with ACHB, a 20% overall mortality rate, with 79% 3-year survival, has been observed, with pacemaker requirement in 64% [72]. …”

Section: Pregnancy Considerations In Autoimmune Connective Tissue Dismentioning

confidence: 99%

Pregnancy and autoimmune connective tissue diseases

Marder

Littlejohn

Somers

2016

Best Practice & Research Clinical Rheumatology

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“…CHB is a more serious form of NLS, affecting 2% of newborns of anti-Ro-positive women with known connective tissue diseases65,66 – it remains a very rare condition in the general population, affecting 1:20,000 pregnancies 67. If an anti-Ro/La-positive woman has had a baby with CHB, then the risk to subsequent pregnancies increases to 18%.…”

Section: Antenatal/postnatal Challenge: Neonatal Lupusmentioning

confidence: 99%

“…The actual incidence may be higher as incomplete forms of CHB have been described, including first-degree heart block that can progress during childhood. Most children with CHB will need a permanent pacemaker (47%–64% in first year67,68 and 75% by 10 years),68 and up to 20% may die in the perinatal period 66,68. Neonatal CHB may also be the first presentation of otherwise asymptomatic connective tissue disease in up to 50% of women: all mothers of infants with CHB are tested for anti-Ro/La antibodies 69…”

Section: Antenatal/postnatal Challenge: Neonatal Lupusmentioning

confidence: 99%

Management of systemic lupus erythematosus during pregnancy: challenges and solutions

Knight¹,

Nelson‐Piercy²

2017

OARRR

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Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease predominantly affecting women, particularly those of childbearing age. SLE provides challenges in the prepregnancy, antenatal, intrapartum, and postpartum periods for these women, and for the medical, obstetric, and midwifery teams who provide their care. As with many medical conditions in pregnancy, the best maternal and fetal–neonatal outcomes are obtained with a planned pregnancy and a cohesive multidisciplinary approach. Effective prepregnancy risk assessment and counseling includes exploration of factors for poor pregnancy outcome, discussion of risks, and appropriate planning for pregnancy, with consideration of discussion of relative contraindications to pregnancy. In pregnancy, early referral for hospital-coordinated care, involvement of obstetricians and rheumatologists (and other specialists as required), an individual management plan, regular reviews, and early recognition of flares and complications are all important. Women are at risk of lupus flares, worsening renal impairment, onset of or worsening hypertension, preeclampsia, and/or venous thromboembolism, and miscarriage, intrauterine growth restriction, preterm delivery, and/or neonatal lupus syndrome (congenital heart block or neonatal lupus erythematosus). A cesarean section may be required in certain obstetric contexts (such as urgent preterm delivery for maternal and/or fetal well-being), but vaginal birth should be the aim for the majority of women. Postnatally, an ongoing individual management plan remains important, with neonatal management where necessary and rheumatology followup. This article explores the challenges at each stage of pregnancy, discusses the effect of SLE on pregnancy and vice versa, and reviews antirheumatic medications with the latest guidance about their use and safety in pregnancy. Such information is required to effectively and safely manage each stage of pregnancy in women with SLE.

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“…Autoantibody-mediated heart block in NL is typically associated with a structurally normal heart; however, structural abnormalities such as valvular lesions are occasionally reported. Additional cardiac life-threatening abnormalities that may be associated with NL include congestive heart failure due to dilated cardiomyopathy and endocardial fibroelastosis [94].…”

Section: Lupus Anti-ro/ssa and Anti-la/ssb Antibodies And Neonatal Lmentioning

confidence: 99%