Diagnosis, management, and long-term results of patients with congenital complete atrioventricular block.

Sixty-five patients with congenital complete atrioventricular block have been studied. The median age at time of diagnosis was 7 months with 26 infants being diagnosed prior to 1 month of age. Anatomic heart disease was present in 25/65 (39%) infants, with ventricular inversion/L-transposition of great arteries complex occurring in 20/25 infants (80%). The site of block was above the bundle of His in 11 of 18 (61%) patients studied, in the bundle of His in three of 18 (17%), and below the bundle of His in two of 18 (11%). Permanent pacemakers were implanted in 17 (26%). Ten patients (15%) have died. The highest risk was in patients with anatomic heart disease and patients diagnosed early in life.