Congenital heart block associated with undiagnosed maternal Primary Sjögren's Syndrome – a case report and discussion

Abstract

Congenital heart block (CHB) has been linked with Sjögren's Syndrome. This paper reports a case of previously undiagnosed maternal Primary Sjögren's Syndrome (1 degrees SS) that was only discovered following the birth of the patient's first child with CHB. The possible pathophysiological mechanisms underlying CHB associated with 1 degrees SS are discussed.

Congenital heart block associated with Sjögren syndrome: case report

“…Foetal heart expresses M1R while maternal heart expresses mainly M2R. The majority of cases of CHB are subclinical at birth; with a case reported of normal ECG at birth and subsequent development of asymptomatic heart block at 2 years [ 9 ].…”

Intrathoracic Implantation of a Dual-Chamber Pacemaker in a Preterm Infant with Congenital AV Block

“…Complete atrioventricular (AV) block can be congenital or acquired and is characterized by non‐propagation of the atrial impulse to the ventricles . Congenital complete AV block can be concomitant with congenital heart diseases or maternal connective tissue disorders like systemic lupus erythematosus and Sjögren's syndrome . Early pacemaker implantation is considered in cases when congenital complete AV block has lead to hydrops fetalis, low ventricular rate, and ventricular dysfunction …”

“…1 Congenital complete AV block can be concomitant with congenital heart diseases or maternal connective tissue disorders like systemic lupus erythematosus and Sjö gren's syndrome. 2 Early pacemaker implantation is considered in cases when congenital complete AV block has lead to hydrops fetalis, low ventricular rate, and ventricular dysfunction. 3 Permanent pacemaker generators are implanted subcutaneously or submuscularly on the chest or the abdominal wall.…”