Congenital Complete Heart Block

Isolated congenital complete heart block has a heterogenous aetiology. In approximately one-third of the patients in the present material the mothers had symptoms or signs of connective tissue disease. The mortality is highest in the neonatal period, much lower during childhood and adolescence and increases slowly later in life. There are elderly patients who are completely asymptomatic but patients are also encountered with signs and symptoms of more or less severe myocardial damage. Stokes-Adams attacks may occur at any age. About 10% of patients below 15 years of age in this material and about 25% of those above 15 years (mean age 30 years) were paced. In the neonatal period the predominant indication for pacing was heart failure. It is difficult to make a prognosis in the individual patient. Apart from already known risk factors, a fixed or decreasing low ventricular rate neonatally and a prolonged QT time seem to be bad prognostic signs.