An expanded phenotype of maternal SSA/SSB antibody-associated fetal cardiac disease

Abstract

Objectives: Conventional manifestations of fetal Sjogren's antibodies (SSA/SSB) associated cardiac disease include atrioventricular block (AVB), transient sinus bradycardia, endocardial fibroelastosis (EFE) and dilated cardiomyopathy. We describe other manifestations of cardiac disease.

Methods: We describe three fetuses with unique myocardial and conduction system disease.

Results: One had isolated EFE with subsequent mitral and tricuspid valve chordal avulsion, the second had sinoatrial and infrahissian conduction system disease, and in both, neonatal progression to life threatening disease occurred. The third had sinus node dysfunction and atrial flutter.

Conclusion: These findings expand the clinical phenotype of maternal SSA/SSB antibody associated fetal cardiac disease.

Figures

Figure 1

Case 1: Echocardiograms. (A) Four chamber view of the fetal heart at 26 weeks showing patchy and diffuse patchy echogenicity in the endocardium (arrows): two spots in the LA, along the chordae of the mitral and tricuspid valves, in the RV endocardium, and at the apex of the LV. (B) Rhythm strip obtained during magnetocardiography showing different T-wave morphologies consistent with T-wave alternans. (C) Echocardiogram at 1 day of age, showing normal dimensions and almost complete resolution of the patchy endocardial echogenicity. (D) At 3 months of age, the anterior leaflet of the mitral valve is flail, there is severe mitral insufficiency and left atrial enlargement.

Figure 2

Case 2: Postnatal electrocardiograms. Low left atrial rhythm, left axis deviation and left bundle branch block with 1:1 AV conduction and normal PR interval at 1 day of age. QRS duration is 100 ms. At 19 days of age, after IVIg. Occasional sinus beats are noted (arrows), the QTc has increased (489) ms and TWA is noted in the rhythm strip. At 2 months of age, the QRS duration had increased to 118 ms, and the left atrial rhythm returned. By 4 months of age, the QRS duration has increased to 150 ms, the rhythm is junctional, and the rate has fallen to 74 bpm. Beat-to-beat changes in QRS morphology are seen in lead V4.