Abstract 17090: Multifocal Ectopic Purkinje Tachycardia: A New Familial Syndrome.
Introduction: We report a new cardiac familial syndrome characterized by frequent polymorphic premature ventricular contractions (PVCs) originating from multiple ectopic foci from the left fascicular-Purkinje system. This syndrome that we called Multifocal Ectopic Purkinje Tachycardia (MEPT), affected 13 patients in a large family of 19 individuals over 3 generations. It was transmitted with an autosomal dominant pattern.
Methods: Invasive electrophysiological (EP) studies were realized in six patients. Intra-cardiac 3D navigation systems were used in three of them. Precise location of the PVC firing was based on the identification of pre-systolic Purkinje potentials, early endocardial activation mapping with a QS unipolar pattern of the electrogram and concordant pace mapping.
Results: Affected patients had frequent PVCs with various right bundle branch block (RBBB) patterns and right to left axis variations. Episodes of faintness and syncope related to polymorphic ventricular tachycardia (VT) episodes were reported in 3 females. Sudden death at rest was reported in one 4-month child and two adults (29 and 50-year-old males). A transient dilated cardiomyopathy (DCM) was diagnosed in 3 females and in one male. Defibrillators were implanted in three patients. Their left ventricular functions recovered once the number of PVCs dropped on Hydroxyquinidin. Invasive EP studies confirmed that multiple ectopic foci were fired from the left fascicular-Purkinje system. There were no criteria for reentry mechanisms. Radiofrequency applications did not succeed in suppressing PVCs as the whole left Purkinje tissue was involved.
Conclusions: This new cardiac familial syndrome is characterized by frequent polymorphic PVCs originating from multiple ectopic foci localized in the left fascicular-Purkinje system. This so called ”Multifocal Ectopic Purkinje tachycardia” syndrome may be associated with tachycardia induced DCM, and sudden cardiac death.
- © 2010 by American Heart Association, Inc.