Congenital Heart Block

Card Electrophysiol Clin. 2021 Dec;13(4):691-702. doi: 10.1016/j.ccep.2021.07.006.


Congenital complete heart block (CCHB) defines atrioventricular conduction abnormalities diagnosed in utero or within the first 27 days of life. Maternal autoimmune disease and congenital heart defects are most commonly responsible. Recent genetic discoveries have highlighted our understanding of the underlying mechanism. Hydroxychloroquine shows promise in preventing autoimmune CCHB. Patients may develop symptomatic bradycardia and cardiomyopathy. The presence of these and other specific findings warrants placement of a permanent pacemaker to relieve symptoms and prevent catastrophic events. The mechanisms, natural history, evaluation, and treatment of patients with or at risk for CCHB are reviewed.

Keywords: Atrioventricular block; Cardiomyopathy; Congenital heart block; Heart block; Pacemaker; Prevention; Structural heart disease; Treatment.

Publication types

  • Review

MeSH terms

  • Atrioventricular Block* / therapy
  • Bradycardia
  • Heart Block / congenital
  • Heart Defects, Congenital*
  • Humans
  • Pacemaker, Artificial*

Supplementary concepts

  • Congenital heart block