Dataset Information

Diagnosis and Management of Fetal Autoimmune Atrioventricular Block.

ABSTRACT: Autoimmune congenital atrioventricular block (CAVB) has been extensively studied in recent decades. The American Heart Association published guidelines for monitoring pregnant women with anti-Ro/Sjögren's syndrome antigen A (SSA) or anti-La/Sjögren's syndrome antigen B (SSB) autoantibodies, which are considered to increase the risk of CAVB. Information about the natural history of the disease in utero has contributed to the detection of fetuses with CAVB in the treatable stage. Hydroxychloroquine (HCQ) may be used to prevent CAVB. The lack of large randomized control trials is a major drawback to fully confirm the benefits of fluorinated steroids such as dexamethasone. Although, when combined with a ?-sympathomimetic agent, the outcome of administering a fluorinated steroid in complete CAVB is still controversial. Novel treatments targeting the immunological process might prevent the recurrence of CAVB in pregnant women with previously affected children.

SUBMITTER: Hansahiranwadee W

PROVIDER: S-EPMC7434531 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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Diagnosis and Management of Fetal Autoimmune Atrioventricular Block.

Hansahiranwadee Wirada W

International journal of women's health 20200812

Autoimmune congenital atrioventricular block (CAVB) has been extensively studied in recent decades. The American Heart Association published guidelines for monitoring pregnant women with anti-Ro/Sjögren's syndrome antigen A (SSA) or anti-La/Sjögren's syndrome antigen B (SSB) autoantibodies, which are considered to increase the risk of CAVB. Information about the natural history of the disease <i>in utero</i> has contributed to the detection of fetuses with CAVB in the treatable stage. Hydroxychl ...[more]

PMID: 32884363

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Project description:ImportancePacemaker implantations as a treatment for atrioventricular (AV) block are increasing worldwide. Prevention strategies for AV block are lacking because modifiable risk factors have not yet been identified.ObjectiveTo identify risk factors for AV block in community-dwelling individuals.Design, setting, and participantsIn this population-based cohort study, data from the Mini-Finland Health Survey, conducted from January 1, 1978, to December 31, 1980, were used to examine demographics, comorbidities, habits, and laboratory and electrocardiographic (ECG) measurements as potential risk factors for incident AV block. Data were ascertained during follow-up from January 1, 1987, through December 31, 2011, using a nationwide registry. A total of 6146 community-dwelling individuals were included in the analysis performed from January 15 through April 3, 2018.Main outcomes and measuresIncidence of AV block (hospitalization for second- or third-degree AV block).ResultsAmong the 6146 participants (3449 [56.1%] women; mean [SD] age, 49.2 [12.9] years), 529 (8.6%) had ECG evidence of conduction disease and 58 (0.9%) experienced a hospitalization with AV block. Older age (hazard ratio [HR] per 5-year increment, 1.34; 95% CI, 1.16-1.54; P < .001), male sex (HR, 2.04; 95% CI, 1.19-3.45; P = .01), a history of myocardial infarction (HR, 3.54; 95% CI, 1.33-9.42; P = .01), and a history of congestive heart failure (HR, 3.33; 95% CI, 1.10-10.09; P = .03) were each independently associated with AV block. Two modifiable risk factors were also independently associated with AV block. Every 10-mm Hg increase in systolic blood pressure was associated with a 22% higher risk (HR, 1.22; 95% CI, 1.10-1.34; P = .005), and every 20-mg/dL increase in fasting glucose level was associated with a 22% higher risk (HR, 1.22; 95% CI, 1.08-1.35; P = .001). Both risk factors remained statistically significant (HR for systolic blood pressure, 1.26 [95% CI, 1.06-1.49; P = .007]; HR for glucose level, 1.22 [95% CI, 1.04-1.43; P = .01]) after adjustment for major adverse coronary events during the follow-up period. In population-attributable risk assessment, an estimated 47% (95% CI, 8%-67%) of AV blocks may have been avoided if all participants exhibited ideal blood pressure and 11% (95% CI, 2%-21%) may have been avoided if all had a normal fasting glucose level.Conclusions and relevanceIn this analysis of data from a population-based cohort study, suboptimal blood pressure and fasting glucose level were associated with AV block. These results suggest that a large proportion of AV blocks are assocated with these risk factors, even after adjusting for other major adverse coronary events.

Project description:BackgroundAmong all fetal heart block patients, > 50% cases are associated with maternal autoimmune diseases, and such patients should receive treatment. However, nearly half of fetal heart block cases involve a mother with negative results following autoimmune antibody screening. A few studies have reported long QT syndrome (LQTS) can also present as a severe fetal bradycardia, which does not respond to fetal treatment. Herein, we reported a rare case of an infant who presented with high-degree autoimmune-mediated fetal atrioventricular block (AVB) with LQTS induced by a novel KCNH2 variant. This case led us to review our prenatal therapeutic strategy.Case presentationA 1-year-old boy presented to our heart center having experienced syncope 5 times in the past year. He had previously presented with fetal bradycardia during the fetal stage from 27 + 3 gestational weeks. The fetal echocardiography demonstrated AVB (2:1 transmission). As the maternal autoimmune antibody results were positive, his mother had received dexamethasone treatment during pregnancy; subsequently, the fetal AVB had changed from 2:1 to 4:3 transmission with elevated ventricular beating rates. However, this patient was identified to have complete AVB after birth. The initial electrocardiogram and Holter measurements at hospital administration showed complete AVB, pleomorphic ventricular tachycardia, a prolonged QT interval (QT = 602 ms, corrected QT = 538 ms), and wide and deep inverted T-waves. Meanwhile, torsades de pointes could be observed in several transit ventricular tachycardias based on Holter monitoring review. Genetic testing revealed KCNH2 c.2483G > A variant-induced LQTS. An implantable cardioverter defibrillator device and permanent pacemaker were both considered as therapeutic alternations; his parents ultimately accepted the implantation of a permanent pacemaker.ConclusionsFor fetuses with autoimmune-mediated AVB, intrauterine treatment should still be pursued immediately. However, once the treatment outcomes are deemed unacceptable or unexpected, other genetic variant-related channelopathies should be highly suspected. If the fetus lacks a positive family history, fetal genetic testing should be recommended to improve the prognosis of such patients by introducing integrative therapeutic strategies between the prenatal and postnatal phases.

Dataset Information

Diagnosis and Management of Fetal Autoimmune Atrioventricular Block.

Publications

Diagnosis and Management of Fetal Autoimmune Atrioventricular Block.

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