Published online Jul 6, 2022. doi: 10.12998/wjcc.v10.i19.6602
Peer-review started: December 1, 2021
First decision: January 12, 2022
Revised: January 20, 2022
Accepted: May 13, 2022
Article in press: May 13, 2022
Published online: July 6, 2022
Processing time: 204 Days and 22.4 Hours
Congenital complete heart block (CCHB) with normal cardiac structure and negativity for anti-Ro/La antibody is rare. Additionally, CCHB is much less frequently diagnosed in adults, and its natural history in adults is less well known.
A 23-year-old woman was admitted to our hospital for frequent syncopal episodes. She had bradycardia at the age of 1 year but had never had impaired exercise capacity or a syncopal episode before admission. The possible diagnosis of acquired complete atrioventricular block was carefully ruled out, and then the diagnosis of CCHB was made. According to existing guidelines, permanent pacemaker implantation was recommended, but the patient declined. With regular follow-up for 28 years, the patient had an unusually good outcome without any invasive intervention or medicine. She had an uneventful pregnancy and led a normally active life without any symptoms of low cardiac output or syncopal recurrence.
This case implies that CCHB in adulthood may have good clinical outcomes and does not always require permanent pacemaker implantation.
Core Tip: Congenital complete heart block (CCHB) is a very rare disorder that is largely diagnosed at the fetal or infant stage. Therefore, it is infrequently diagnosed in adulthood, and the natural history of CCHB in adults is less well known. Despite the controversial literature, permanent pacing is widely recommended for the prevention of sudden death among patients with CCHB. This case illustrated an unexpectedly good course in an adult with CCHB at the onset of syncope who refused permanent pacing but led a normally active life. This suggests that CCHB in adulthood may have good outcomes and does not always require permanent pacing.