Key result
Familial dysautonomia is linked to prolonged QTc and abnormal QT adaptation after exercise.
Why the study?
Abnormalities in corrected QT intervals related to autonomic dysfunction in familial dysautonomia patients were not fully characterized with positional changes and exercise.
Do changes in position and exercise reveal abnormalities in corrected QT intervals in patients with familial dysautonomia?
Population
Patients with familial dysautonomia
Comparison
Corrected QT intervals with changes in position and after exercise
Design
Observational study
Authors
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QTc response to exercise may mark autonomic dysfunction in familial dysautonomia; leaves open prospective validation before any clinical adoption.
Observational
Do changes in position and exercise reveal abnormalities in corrected QT intervals in patients with familial dysautonomia?
Prolonged corrected QT intervals that fail to shorten appropriately with exercise can serve as a noninvasive indicator of autonomic dysfunction in familial dysautonomia.
Glickstein et al. (1993) conducted an observational in Familial dysautonomia. Changes in position and exercise was evaluated on Abnormalities in corrected QT intervals. Patients with familial dysautonomia exhibited prolonged corrected QT intervals (> 440 msec) with a lack of appropriate shortening after exercise, indicating aberrant autonomic regulation.
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