Diagnosis, Management, and Long-Term Results of Patients with Congenital Complete Atrioventricular Block

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Abstract
Sixty-five patients with congenital complete atrioventricular block were studied. The median age at time of diagnosis was 7 mo. with 26 infants being diagnosed prior to 1 mo. 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 3 of 18 (17%) and below the bundle of His in 2 of 18 (11%). Permanent pacemakers were implanted in 17 (26%). Ten patients (15%) died. The highest risk was in patients with anatomic heart disease and patients diagnosed early in life.