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September 17, 1973

Hereditary Progressive Atrioventricular Conduction DefectA New Syndrome?

Author Affiliations

From the Department of Preventive Medicine and Public Health (Dr. Lynch, Ms. Krush, and Mr. Carter), the Department of Medicine (Drs. Mohiuddin, Sketch, and Runco), and the Cardiac Center (Dr. Runco), Creighton University School of Medicine.

JAMA. 1973;225(12):1465-1470. doi:10.1001/jama.1973.03220400011003

We studied a syndrome involving a progressive atrioventricular conduction defect in three generations of a large family. Clinical findings included a slow heart rate, episodes of lightheadedness, syncope, and absence of predisposing acquired or hereditary cardiac disorders. Electrocardiography showed a prolonged atrioventricular conduction with onset usually in the 30s, loss of R waves in the right precordial leads, and arrhythmia that occurs only as a late manifestation. Progression from first-degree to complete heart block was usually slow, but occasionally a fulminant course led to sudden death within two or three years. Specific differences from other forms of conduction defects suggest that this is a new hereditary syndrome.