Portal de Periódicos da CAPES

WITHDRAWN: Detection and successful treatment of emergent anti-SSA mediated fetal atrioventricular block

2016; Elsevier BV; Linguagem: Inglês

10.1016/j.ajog.2016.07.006

1097-6868

Bettina F. Cuneo, Steven Ambrose, Wayne Tworetzky,

Renal Diseases and Glomerulopathies

Fetal complete atrioventricular block occurs between 18 and 26 weeks in 2–4% of anti-SSA-positive pregnancies, but disease burden is considerable: 18% die in utero or before age 2 years, and survivors ultimately require permanent cardiac pacing.1Izmirly P.M. Saxena A. Kim M.Y. et al.Maternal and fetal factors associated with mortality and morbidity.Circulation. 2011; 124: 1927-1935Crossref PubMed Scopus (195) Google Scholar Reports suggest as the fetal rhythm transitions from normal to complete atrioventricular block, antiinflammatory treatment can improve atrioventricular conduction and even restore sinus rhythm.2Askanase A.D. Friedman D.M. Copel J. Spectrum and progression of conduction abnormalities in infants born to mothers with anti-SSA/Ro/SSB-La antibodies.Lupus. 2002; 11: 145-151Crossref PubMed Scopus (166) Google Scholar, 3Eliasson H. Sonesson S.E. Sharland G. et al.Isolated atrioventricular block in the fetus: a retrospective, multinational, multicenter study of 175 patients.Circulation. 2011; 24: 1919-1926Crossref Scopus (186) Google Scholar Little is known about this transition period of emergent complete atrioventricular block, and to date, surveillance methods have unsuccessfully detected emergent complete atrioventricular block in time for effective in utero treatment.4Friedman D.M. Kim M.Y. Copel J.A. et al.Utility of cardiac monitoring in fetuses at risk for congenital heart block: the PR Interval and Dexamethasone Evaluation (PRIDE) prospective study.Circulation. 2008; 29: 485-493Crossref Scopus (238) Google Scholar The objectives of the study were as follows: (1) to describe the transition times from normal fetal rhythm to emergent complete atrioventricular block and from emergent complete atrioventricular block to complete atrioventricular block; and (2) to describe long- and short-term results of in utero treatment of emergent complete atrioventricular block. This was an observational case series of 4 anti-SSA-positive pregnancies (3 with a previous complete atrioventricular block fetus) that developed fetal emergent complete atrioventricular block at 19–20 weeks of gestation. One was not undergoing surveillance, 3 participated in weekly surveillance echocardiograms4Friedman D.M. Kim M.Y. Copel J.A. et al.Utility of cardiac monitoring in fetuses at risk for congenital heart block: the PR Interval and Dexamethasone Evaluation (PRIDE) prospective study.Circulation. 2008; 29: 485-493Crossref Scopus (238) Google Scholar from 16–17 to 26 weeks and 2 of 3 assessed fetal rhythm 2 times per day using a commercially available Doppler device. Irregular fetal rhythm, or rate <100 bpm prompted immediate diagnostic echo. Mothers were given intravenous immune globulin and/or oral dexamethasone for surveillance or diagnostic echo findings of fetal emergent complete atrioventricular block. Subjects 1 and 2 (Figure, A and B): Emergent complete atrioventricular block was detected in time for treatment to restore 1:1 atrioventricular conduction. Subject 1 detected an irregular fetal heart rate during home Doppler monitoring 8 hours after hearing a regular rhythm. Subject 2’s 20-week surveillance fetal echo showed atrioventricular intervals of 120–230 ms. Both received dexamethasone and intravenous immune globulin and were in sinus rhythm within 3–7 days. Dexamethasone (tapered from 4 mg/d over 1 month) was continued until 32 weeks (subject 2) or birth (subject 1). Both neonates had 1:1 conduction and prolonged PR intervals, which have persisted for 4 and 2 years, respectively. Neither is paced. Subjects 3 and 4 (Figure, C): Emergent complete atrioventricular block was either not recognized in time for successful treatment or in utero treatment was successful but discontinued before 28 weeks. Subject 3 detected an irregular fetal heart rate during home Doppler monitoring at 19 4/7 weeks, 12 hours after hearing a normal rhythm, but the subject did not seek medical attention. Twelve hours later (24 hours after last normal rhythm), the fetus was in complete atrioventricular block and did not respond to intravenous immune globulin and dexamethasone. Subject 4 was diagnosed at 19 weeks with emergent complete atrioventricular block and responded to dexamethasone with restored 1:1 conduction, but dexamethasone was discontinued (24 weeks) and emergent complete atrioventricular block recurred at 26 weeks and was not treated. Both subjects were in complete atrioventricular block at birth, and both are paced and doing well at 4 years of age. The following conclusions were reached: (1) the transition from normal rhythm to emergent complete atrioventricular block and from emergent complete atrioventricular block to complete atrioventricular block occurs in <24 hours; (2) treatment of emergent complete atrioventricular block can restore 1:1 conduction; (3) treatment beyond 24 weeks may be necessary to maintain 1:1 conduction, at least until birth. These findings suggest that the daily surveillance of fetal rhythm is necessary to detect emergent complete atrioventricular block and institute successful treatment.