Fetal Congenital Complete Heart Block Due to Maternal Sjögren Syndrome

Dhaval Akhani, Shilpa Kalane, Sumeet Pitkar, Usha Pratap, Aparna Kulkarni


Congenital complete heart block (CCHB) in the absence of structural heart disease is associated with maternal autoimmune disorders such as Sjögren syndrome or systemic lupus erythematosus, where maternal antibodies to Ro/SSA and La/SSB cross the placenta and damage the atrioventricular node of the fetus.

We present the case of a female, late preterm neonate (35 + 4 weeks of gestation) diagnosed of isolated CCHB at 26th week of gestation by fetal echocardiography. The mother was diagnosed of Sjögren syndrome and was administered dexamethasone and salbutamol for treating CCHB in the fetus. Fetal heart rate (HR) was around 70 to 80 beats/min throughout pregnancy. After 6 hours of birth, the neonate’s HR dropped below 55 beats/min and hence isoprenaline infusion was started. The neonate responded well to isoprenaline therapy and the HR improved to 80 to 90 beats/min. The neonate was discharged on oral orciprenaline on day 10. At 8 months of age, the infant was neurologically normal and thrived well with a baseline HR of 80 to 100 beats/min.

We conclude that (1) early diagnosis and prompt management of CCHB is important for better prognosis and prevention of associated complications; (2) neonates with CCHB should be managed at a tertiary care center; and (3) sympathomimetics have a promising role in the management of CCHB.

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