Congenital complete heart block in patients without anatomic cardiac defects

Forty-three patients with congenital complete heart block and an otherwise structurally normal heart were reviewed in an effort to better define the profile of an anatomically homogenous group and to identify factors that may predict the need for pacing. Fourteen patients (32%) developed "symptoms" during follow-up, including two with out-of-hospital cardiac arrest. Heart rate on electrocardiogram or Holter monitor did not clearly distinguish this subgroup. The presence of alternate "risk factors," such as atrial enlargement seen on electrocardiogram, cardiomegaly seen on x-ray film, or prolonged QT interval were independent predictors of symptoms and poor outcome (p less than 0.05). Ventricular ectopy determined on Holter monitoring was also common among the symptomatic group, although this finding was inconsistent. Prophylactic pacing is indicated in select patients with congenital complete heart block and otherwise normal anatomy. Surveillance for rick factors beyond rate criteria alone may refine this selection process.