# Maternal Primary Sjögren's Syndrome Complicated by Irreversible Fetal Third‐Degree Congenital Heart Block: A Case Report From Nepal

## TL;DR

A case report from Nepal highlights irreversible fetal heart block in a pregnant woman with Sjögren's syndrome and the importance of early monitoring.

## Contribution

Highlights the importance of preconception counseling and fetal echocardiography in managing high-risk pregnancies.

## Key findings

- Third-degree fetal heart block was irreversible despite corticosteroid treatment.
- Hydroxychloroquine and serial fetal echocardiography are recommended for early detection and counseling.
- Preconception counseling is essential for managing pregnancies at high risk of congenital heart block.

## Abstract

In pregnancies of anti‐Ro/SSA– and anti‐La/SSB–positive women with primary Sjögren's syndrome, third‐degree fetal congenital heart block is usually irreversible despite corticosteroids. Preconception counseling, hydroxychloroquine prophylaxis, and serial fetal echocardiography from 16 weeks are essential to enable timely counseling, including discussion of termination in poor prognosis cases.