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(Chest. 1970;58:236-243.)
© 1970 American College of Chest Physicians

Clinical and Pathologic Studies in the Hereditary Syndrome of a Long QT Interval, Syncopal Spells and Sudden Death

John Phillips M.D.1 and Herbert Ichinose M.D.1

1 Departments of Medicine and Pathology, Tulane University School of Medicine, The Veterans Administration Hospital and the Charity Hospital of New Orleans

Clinical and pathologic investigations of a family whose members suffered from a heritable syndrome of syncopal spells, sudden death, and QT interval prolongation on the electrocardiogram are reported. All members had normal hearing. The mode of inheritance appeared to be autosomal dominance. The syndrome is very similar to one first reported in 1957 by Jervell and Lange-Nielsen except in the latter disorder, nerve-deafness is present and the mode of inheritance is autosomal recessive. In the syndromes reported, attacks of syncope and sudden death are usually due to ventricular fibrillation and this is frequently triggered by physical or acute emotional stress. Of importance and as illustrated by the propositus of this study, the electrocardiogram may be normal at rest only to show the characteristic bizarre QT prolongation after exercise. Pathologic study of the heart of one of the family members who died suddenly revealed diffuse and extensive fibrosis of the conducting system along with abnormal changes in the small arterial vessels in this area. The part these lesions play in the pathogenesis of the clinical syndrome is not entirely clear. The syndrome is probably more unrecognized than rare and may explain a number of instances of "atypical seizures." Any individual with such seizures should have an electrocardiogram, preferably after exercise, as an integral part of the diagnostic evaluation.




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