Abstract 8808: Early Repolarization Syndrome: Valsalva Maneuver in Familial Screening
Introduction: Early repolarization (ER) has been recently linked with idiopathic ventricular fibrillation. In spite of a frequent familial past history of SCD, ER identification in relatives is limited by variability of the ER over the time and with the variation of autonomic tone and heart rate. The aim of the study was to test the ability of valsalva maneuver (VM) to identify larges families affected by early repolarization syndrome (ERS).
Methods: We have screened relatives of 2 families in which SCD occurred with ERS. Patients were considered to be affected in the presence of a positive deflection on the S wave in two consecutive leads and at least a 1 mm amplitude above baseline. A VM was performed in relatives to increase or reveal ER. Comparison between heart rate and J wave amplitude before and after VM was performed with a paired Student's t-test.
Results: In the first family, 4 SCD occured whose one with an ER pattern. We identified 25 other ER out of 82 available ECG (prevalence 34%). In the second family, 4 SCD occurred whose one with an ER pattern. We identified 17 other ER out of 29 available ECG (prevalence 58%). In those families, ER is transmitted with an autosomal dominant trait through respectively 5 and 4 generations. VM was performed for 80 relatives. It increased ER amplitude in 17/20 (85%) affected patients and revealed ER in 17/60 (28%) patients unaffected at rest. VM succeeded in unmasking pattern for 5/11 (45%) expected carrier of ER previously considered as unaffected. Heart rate at rest was 75 ± 13 bpm and decreased of 3,9 ± 7,9 bpm (p<0,001) during the test without any differences between groups.
Conclusion: Malignant forms of ER can be transmitted as an autosomal dominant trait in large families. VM could be helpful to unmask ER for familial screening of relatives. Further investigations are required to assess prognosis of VM.
- © 2011 by American Heart Association, Inc.