Perinatology.  2016 Sep;27(3):185-189. 10.14734/PN.2016.27.3.185.

Fetal Congenital Complete Atrioventricular Block in a Mother with Isolated Serum Anti-SSA/Ro and Anti-SSB/La Antibodies

• Mok CW
• Park JY
• Kim K
• Choi SJ
• Oh SY
• Roh CR
• Kim JH

• KMID: 2355579
• DOI: http://doi.org/10.14734/PN.2016.27.3.185

Abstract

Congenital atrioventricular block (AVB) is rare but carries a significant fetal or neonatal mortality and morbidity. In about half of the cases, it is associated with a congenital structural heart anomaly and often results from the transplacental transfer of maternal anti-SSA/Ro and/or anti-SSB/La autoantibodies in women with autoimmune diseases. We report a case of an isolated congenital AVB with cardiomegaly and pericardial effusion in a woman who had both serum anti-SSA/Ro and anti-SSB/La antibodies, but had no clinical or laboratory evidence of autoimmune diseases. The mother was treated with oral dexamethasone for about 10 days antenatally until the fetal pericardial effusion regressed. AVB persisted without any fetal compromise throughout the remaining gestational period until elective cesarean delivery at term. A permanent pacemaker was implanted into the baby at 2 weeks after birth due to hypotension and aggravating heart failure. The baby did not develop any complications and was discharged from the hospital.

Keyword

Congenital Atrioventricular Block; Anti-SSA/Ro; Anti-SSB/La; Dexamethasone

MeSH Terms

Antibodies*
Atrioventricular Block*
Autoantibodies
Autoimmune Diseases
Cardiomegaly
Dexamethasone
Female
Heart
Heart Failure
Humans
Hypotension
Infant
Infant Mortality
Mothers*
Parturition
Pericardial Effusion
Antibodies
Autoantibodies
Dexamethasone

Figure

• 

  Fig. 1 (A) Fetal echocardiogram at 23+0 weeks of gestation showing 3:1 atrioventricular block with atrial rate of 150 bpm and ventricular rate of 56 bpm. (B) Pericardial effusion (arrowhead) at 23+0 weeks of gestation. (C) Regression of pericardial effusion (arrowhead) at 25+5 weeks of gestation.

• 

  Fig. 1 (A) Neonatal electrocardiogram after birth showing atrioventricular dissociation. (B) Neonatal echocardiogram after birth showing left ventricular ejection fraction of 59%. (C) Neonatal echocardiogram at 3 days after birth when a temporary pacemaker insertion was performed (left ventricular ejection fraction of 45.36%). (D) Electrocardiogram after insertion of permanent pacemaker showing a regular heart rate of 130 beats per minute.

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