CONGENITAL COMPLETE ATRIOVENTRICULAR-BLOCK

Medical history and recent knowledge on definition, diagnosis, etiology, natural history, and management of congenital complete atrioventricular block (CCAVB) are reviewed. An association between material connective tissue disease and most cases of CCAVB has been demonstrated, but further and more specific proof of a causal relationship is needed. Mortality and morbidity in isolated CCAVB is highest in fetal life and in the neonatal period, but is also considerable in infancy, childhood, and adult life. The presence of associated structural heart disease and/or significant endomyocardial fibrosis increases the mortality and morbidity. Treatment of CCAVB is possible and effective in most cases, and prevention might be possible in selected cases in the near future. The low complication rate of pacemaker treatment of today justifies a generous attitude toward treatment. The risk of sudden death in adult life without prodromal symptoms, the gradually decreasing ventricular rate, and a high incidence of 'acquired' mitral insufficiency are arguments in favor of prophylactic pacemaker treatment.