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Nonimmune Hydrops Fetalis
Published in Vincenzo Berghella, Maternal-Fetal Evidence Based Guidelines, 2022
Chelsea DeBolt, Katherine Connolly, Mary E. Norton, Joanne Stone
Bradyarrhythmias are most commonly the result of congenital heart block, either from an autoimmune cause or structural abnormalities affecting cardiac conduction. Transplacental passage of maternal antibodies associated with autoimmune diseases is seen in 30–50% of these cases. They can be present in association with anti-Sjogren's- syndrome-related antigen A (anti-Ro) or the combination or anti-Ro/SSA and anti-La/SSB antibodies (see Chapter 27) [1]. Structural abnormalities such as endocardial cushion defects in the setting of heterotaxy syndrome can also interfere with cardiac conduction and lead to heart block. Complete fetal heart block can lead to hydrops when the fetal heart rate is below 60 beats per minute. There have been several case reports showing successful prevention of hydrops after maternal administration of beta-sympathomimetics, such as terbutaline, though data are very limited [11, 12]. Corticosteroids have also been studied as possible treatment for fetal heart block and shown not to be effective in reversing third-degree block or preventing progression from second- to third-degree block [13]. At this time, in utero treatment of fetal hydrops as a result of fetal bradyarrhythmia is not recommended [1].
Cardiovascular system
Published in Jagdish M. Gupta, John Beveridge, MCQs in Paediatrics, 2020
Jagdish M. Gupta, John Beveridge
The history suggests a diagnosis of congenital heart block. This is usually due to maternal autoantibodies, though it is occasionally a result of congenitally corrected transposition with ventricular inversion or other anomalies. The presence of systolic and diastolic murmurs, cardiomegaly and wide pulse pressure are due to increased stroke volume rather than structural cardiac anomalies. Although most children do not need pacemakers at this age, this infant needs prompt insertion of a pacemaker as the resting heart rate is only 45 and there has been a convulsion. Unless there are syncopes, most patients lead a normal active life.
Autoimmune conditions
Published in David M. Luesley, Mark D. Kilby, Obstetrics & Gynaecology, 2016
These are caused by anti-Ro or anti-La antibodies and manifest as cutaneous neonatal lupus, affecting 5 percent of babies of Ro-positive women, and congenital heart block, affecting 2 percent of babies of Ro-positive women. If the first child is affected, the risk to the second child is 10-fold higher, and once two children are affected the risk rises to about 50 percent. Neonatal cutaneous lupus develops in the first 2 weeks of life and is a geographical skin lesion of the face or scalp. It may be precipitated by exposure to ultraviolet light and usually regresses spontaneously without scarring within 6 months. Congenital heart block develops in utero at 18–30 weeks’ gestation. There is no treatment, and one in five affected babies dies in utero or as a neonate. About half of those surviving require pacemakers in early infancy and the rest by their teens.10 Ro-positive women should be offered fetal cardiology screening [C]. Retrospective data suggest hydroxychloroquine may reduce the risk of congenital heart block.
Pregnancy outcome in women with different rheumatic diseases: a retrospective analysis
Published in Scandinavian Journal of Rheumatology, 2021
H Eisfeld, AM Glimm, GR Burmester, S Ohrndorf, M Backhaus
Women are affected more often than men by rheumatic diseases, especially by systemic lupus erythematosus (SLE), the incidence of which peaks in childbearing age. Numerous studies have shown that pregnancy increases the risk of flare in women with SLE (e.g. 1, 2). In contrast, there is no evidence of a higher rate of flare in pregnant women with other connective tissue diseases (CTDs) such as primary Sjögren’s syndrome (pSS) and systemic sclerosis (SSc). All CTDs, especially with anti-phospholipid antibody syndromes, are associated with an increased risk of pregnancy complications such as miscarriage, preterm birth, and hypertensive disorders (3–6). Furthermore, congenital heart block can occur in newborn infants of mothers with anti-Ro/La antibodies (7).
Cardiac arrhythmias in pregnant women: need for mother and offspring protection
Published in Current Medical Research and Opinion, 2020
Theodora A. Manolis, Antonis A. Manolis, Evdoxia J. Apostolopoulos, Despoina Papatheou, Helen Melita, Antonis S. Manolis
A variety of arrhythmias may occur in the fetus, detected in ≥2% of unselected pregnancies during the routine obstetrical ultrasound141. The majority of fetal arrhythmias are benign and may resolve spontaneously, but some of them, such as SVT, VT, AF/AFlu, and AV block, when sustained, may result in hydrops fetalis, fetal heart failure and fetal death, thus requiring early diagnosis and intervention in the mother and/or fetus142. Fetal congenital heart block has been detailed elsewhere143; fetal SVT and VT are discussed below.
Clinical Features, Autoantibodies, and Outcome of Neonatal Lupus Erythematosus
Published in Fetal and Pediatric Pathology, 2022
NLE may involve multiple organ systems, with the most common features being cutaneous, cardiac, hepatic, and haematologic [1]. Among all related complications, congenital heart block (CHB) which covers a spectrum of severity ranging from a first-degree atrioventricular (AV) block to second- and third-degree AV block has received increased attention. Of these, complete congenital heart block (CCHB) manifested by third-degree heart block, which involves complete AV dissociation is the most characteristic and life-threatening abnormality of NLE.