CONGENITAL COMPLETE HEART-BLOCK

The congenital complete heart block is a rare disease, which occurs pr edominantly in children with otherwise normal hearts. The etiologic co ncept is focusing on two main points: 1) an isolated maldevelopment of the conduction axis and 2) the heart block as a symptom of transplace ntal maternal antibodies against cardiac tissue in children of mothers with an autoimmune disease. Cardiac insufficiency, syncope and sudden death are the main features in the clinical course. The most importan t diagnostic tools are EGG, Holter-ECG and exercise tests. The only th erapeutic approach we have is implantation of a pacemaker. Therapy is indicated in a symptomatic child or in an asymptomatic one which fulfi lls certain criteria considered to be a marker for syncope or sudden d eath. While in children with otherwise normal hearts the prognosis is rather good, patients with structural heart defects tend to have a hig h mortality, especially in the newborn period.