The association of high grade atrioventricular heart block with systemic connective tissue diseases is very rare. To date, only sporadic case reports or reviews appeared in the literature. Three cases of such association observed by the author are described here. The patients were a 51 years old man with systemic sclerosis and two women, a 64 years old patient with visceral nodous seropositive rheumatoid arthritis, and second was a 74 years old patient with systemic lupus erythematosus, the oldest of the group of patients with this disease. In two of the three patients, Adams-Stokes attack was a cause of death. The author did not observe this high grade atrioventricular block in patients with systemic connective tissue diseases the last 32 years. First and second grade heart blocks were, however, o chi asionally seen and responded well to the treatment of the systemic disease. Thus, in patients with systemic connective tissue diseases and emergency symptoms, it is recommended to consider also this rare association. While, two decades ago, patients frequently died after the Adams-Stokes attack, the treatment of the high grade block is now successful due the permanent cardiostimulator. In the last years, there are almost no reports about the association of systemic connective tissue diseases with high grade heart block, presumably because of the efficient new treatment approached to systemic diseases including modern immunomodulation drugs.

Keywords: Adams-Stokes Syndrome /complications/; Aged; Connective Tissue Diseases /complications/; Female; Heart Block /complications/; Humans; Male; Middle Aged