Congenital atrioventricular heart block: From diagnosis to treatment

Congenital atrioventricular block (CAVB), classified as such when diagnosed in utero, at birth or during the first month of life, is a rare condition with an estimated incidence between 1/15 000 and 1/22 000 live births. It is now accepted that the pathophysiology of this condition is predominantly associated with an immunologically mediated response to the conduction system, which occurs due to transplacental passage of maternal autoantibodies from mothers diagnosed, in most cases, with systemic lupus erythematosus or Sjögren syndrome. Fetal echocardiography continues to be the diagnostic gold standard, however there are other techniques with good results and advantages. Regarding therapeutics, both pharmacological measures and cardiac stimulation techniques have been developed to increase the safety of procedures, decrease associated mortality and morbidity, and provide a better quality of life for patients, although there are disagreements in deciding the best therapeutic plan. This review aims to summarize and elucidate the best diagnostic approach as well as the best therapeutic strategies. A search was performed in the PubMed and Science Direct databases of articles published and accepted for publication. The following search terms were used: “Congenital atrioventricular block”, “Neonatal lupus”, “Pacemaker”, “Pathophysiology”, “Electrophysiology”, and “Prenatal diagnosis”. Articles in Portuguese and English were selected. No time constraints were used. Repeated articles were excluded from the two databases.