Lenegre's disease in youth

The case of a 22-year-old white male without known heart disease who presented with activity related lightheadedness at age 19 and dizziness and fatigue at age 21 is described. Standard electrocardiograms (ECG's) revealed intermittent complete trifascicular block. Rapid progression of symptoms over the succeeding eight months resulted in increasing incapacity. Holter monitoring demonstrated that symptoms were related to development of second and higher degrees of A-V block. Normal A-H interval and markedly prolonged H-Q interval on His bundle electrograms indicated that block was infranodal and localized to bundle branch system. Conduction problems aside, clinical and laboratory evaluation including echocardiograms and cardiac catheterization, were unremarkable. Progression of bilateral bundle branch disease in a young patient without other demonstrable heart lesions and a negative family background conforms with criteria for Lenegre's disease. To our knowledge, this represents the youngest reported patient with this entity. Possible electrophysiologic basis of block and of exercise induced improvement in A-V conduction also are considered.