Association of Maternal Systemic Lupus Erythematosus with Congenital Complete Heart Block

Background— The goal of this statement is to review available literature and to put forth a scientific statement on the current practice of fetal cardiac medicine, including the diagnosis and management of fetal cardiovascular disease. Methods and Results— A writing group appointed by the American Heart Association reviewed the available literature pertaining to topics relevant to fetal cardiac medicine, including the diagnosis of congenital heart disease and arrhythmias, assessment of cardiac function and the cardiovascular system, and available treatment options. The American College of Cardiology/American Heart Association classification of recommendations and level of evidence for practice guidelines were applied to the current practice of fetal cardiac medicine. Recommendations relating to the specifics of fetal diagnosis, including the timing of referral for study, indications for referral, and experience suggested for performance and interpretation of studies, are presented. The components of a fetal echocardiogram are described in detail, including descriptions of the assessment of cardiac anatomy, cardiac function, and rhythm. Complementary modalities for fetal cardiac assessment are reviewed, including the use of advanced ultrasound techniques, fetal magnetic resonance imaging, and fetal magnetocardiography and electrocardiography for rhythm assessment. Models for parental counseling and a discussion of parental stress and depression assessments are reviewed. Available fetal therapies, including medical management for arrhythmias or heart failure and closed or open intervention for diseases affecting the cardiovascular system such as twin–twin transfusion syndrome, lung masses, and vascular tumors, are highlighted. Catheter-based intervention strategies to prevent the progression of disease in utero are also discussed. Recommendations for delivery planning strategies for fetuses with congenital heart disease including models based on classification of disease severity and delivery room treatment will be highlighted. Outcome assessment is reviewed to show the benefit of prenatal diagnosis and management as they affect outcome for babies with congenital heart disease. Conclusions— Fetal cardiac medicine has evolved considerably over the past 2 decades, predominantly in response to advances in imaging technology and innovations in therapies. The diagnosis of cardiac disease in the fetus is mostly made with ultrasound; however, new technologies, including 3- and 4-dimensional echocardiography, magnetic resonance imaging, and fetal electrocardiography and magnetocardiography, are available. Medical and interventional treatments for select diseases and strategies for delivery room care enable stabilization of high-risk fetuses and contribute to improved outcomes. This statement highlights what is currently known and recommended on the basis of evidence and experience in the rapidly advancing and highly specialized field of fetal cardiac care.

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“…93 Fetuses can be affected in the presence of maternal serologic evidence of disease and no overt clinical symptoms.…”

Section: Autoimmune Disease and Autoantibody Positivitymentioning

confidence: 99%

Diagnosis and Treatment of Fetal Cardiac Disease

Donofrio¹,

Moon-Grady²,

Hornberger³

et al. 2014

Circulation

983

543

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“…There are several other conditions in which maternal antibodies are known to be pathogenic, including hemolytic disease of the newborn, due to the transfer of antibodies against the rhesus antigen on fetal red blood cells (30,31); neonatal idiopathic thrombocytopenia purpura (32); and heart block that results from transfer of antibodies against Ro or La antigens (33,34). Interestingly, in the congenital heart block, the mother may have systemic lupus erythematosus but is often asymptomatic at the time.…”

Section: Discussionmentioning

confidence: 99%

Plasma from human mothers of fetuses with severe arthrogryposis multiplex congenita causes deformities in mice

Jacobson¹,

Polizzi²,

Morriss‐Kay³

et al. 1999

J. Clin. Invest.

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“…Although a high proportion of infants with congenital complete heart block are born to women with systemic lupus erythematosus, [77][78][79] no published reports show an association between maternal connective tissue disease and an increased risk of structural congenital cardiovascular malformations.…”

Section: Systemic Lupus Erythematosusmentioning

confidence: 99%

Noninherited Risk Factors and Congenital Cardiovascular Defects: Current Knowledge

Jenkins¹,

Correa²,

Feinstein³

et al. 2007

Circulation

698

336

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Abstract-Prevention of congenital cardiovascular defects has been hampered by a lack of information about modifiable risk factors for abnormalities in cardiac development. Over the past decade, there have been major breakthroughs in the understanding of inherited causes of congenital heart disease, including the identification of specific genetic abnormalities for some types of malformations. Although relatively less information has been available on noninherited modifiable factors that may have an adverse effect on the fetal heart, there is a growing body of epidemiological literature on this topic. This statement summarizes the currently available literature on potential fetal exposures that might alter risk for cardiovascular defects. Information is summarized for periconceptional multivitamin or folic acid intake, which may reduce the risk of cardiac disease in the fetus, and for additional types of potential exposures that may increase the risk, including maternal illnesses, maternal therapeutic and nontherapeutic drug exposures, environmental exposures, and paternal exposures. Information is highlighted regarding definitive risk factors such as maternal rubella; phenylketonuria; pregestational diabetes; exposure to thalidomide, vitamin A cogeners, or retinoids; and indomethacin tocolysis. Caveats regarding interpretation of possible exposure-outcome relationships from case-control studies are given because this type of study has provided most of the available information. Guidelines for prospective parents that could reduce the likelihood that their child will have a major cardiac malformation are given. Issues related to pregnancy monitoring are discussed. Knowledge gaps and future sources of new information on risk factors are described.

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Association of Maternal Systemic Lupus Erythematosus with Congenital Complete Heart Block

Cited by 261 publications

References 19 publications

Diagnosis and Treatment of Fetal Cardiac Disease

Diagnosis and Treatment of Fetal Cardiac Disease

Plasma from human mothers of fetuses with severe arthrogryposis multiplex congenita causes deformities in mice

Noninherited Risk Factors and Congenital Cardiovascular Defects: Current Knowledge

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