Electrophysiologic and imaging evidence for an occult myopathic substrate in patients with idiopathic ventricular arrhythmias.

Background: Idiopathic VA are traditionally considered benign, although occasional patients develop an ectopy-mediated cardiomyopathy (EMC). It is unclear whether patients with idiopathic VA in the absence of left ventricular (LV) dysfunction harbor a subclinical cardiomyopathy. We aim to assess for cardiomyopathic substrate in patients with idiopathic ventricular arrhythmias (VA) using imaging and electrophysiologic markers of early fibrosis.

Methods: Cardiac magnetic resonance (CMR) imaging and ventricular electroanatomic mapping was performed in 3 groups: patients undergoing ablation for idiopathic VA without (Group 1, n = 17) and with LV dysfunction (Group 2 [presumed EMC], n = 12) plus a control group undergoing ablation of supraventricular tachycardia (Group 3, n = 16). Global LV strain, T1 mapping and extended electrogram (EGM) characteristics were compared.

Results: Global strain was impaired in patients with presumed EMC (Group 2, p < 0.001). Native T1 times did not differ between groups, however patients in both idiopathic VA groups (Groups 1 and 2) had shorter post-contrast T1 times at 8 min compared to SVT controls (Group 3, p = 0.04). Similarly, the duration of the bipolar EGM was subtly prolonged in both Group 1 and 2 compared to Group 3 (p = 0.002). There were no between group differences in unipolar or bipolar voltage, the no. of bipolar EGM deflections or the maximal unipolar EGM dV/dt.

Conclusion: Patients with idiopathic VAs and apparently structurally normal hearts may have subtle CMR and electrophysiologic changes similar in magnitude to that seen in frank presumed EMC, possibly suggestive of an occult cardiomyopathic process.