Ventricular arrhythmias in patients with bicuspid aortic valves

Introduction: Bicuspid aortic valves (BAV) are the most common congenital heart defects and the extent of ventricular arrhythmias (VA) in patients with BAV is unclear. The objective of this study is to describe VAs and late gadolinium enhancement cardiac magnetic resonance imaging (LGE-CMR) in patients with BAV. Methods: A total of 19 patients with BAV (18 males, age: 58 +/- 13 years) were referred for VA ablation procedures. Ten patients had BAVs at the time of ablation, nine patients had prior aortic valve replacement for a BAV. All but one patient had LGE-CMR and all patients underwent programmed ventricular stimulation at the time of the ablation. Results: Frequent PVCs were the targeted VAs in 17/19 patients and VT in 2/19 patients. Monomorphic ventricular tachycardia (VT) was inducible in 6 patients. A total of 15 VTs were inducible (2.5 +/- 1.0 VTs per patient with a mean cycle length of 322 +/- 83 msec). LGE was present in 13 patients. Patients with inducible VT had larger borderzone and core scar compared to non-inducible patients (7.8 +/- 2.1 cm(3) vs. 2.5 +/- 3.1 cm(3) and 5.1 +/- 2.6 cm(3) vs. 1.9 +/- 3.0 cm(3), p-value < .05 for both). PVCs and VTs were mapped to the periaortic valve area in 12 patients and 4 patients, respectively. The PVC burden was reduced from 27 +/- 13 to 3 +/- 6 (p < .001) and the ejection fraction improved from 49 +/- 13% to 55 +/- 9% (p = .005). Conclusions: VAs in patients with BAV often originate from the perivalvular area and patients often have LGE and inducible VT. LGE may be due to ventricular remodeling secondary to the presence of BAV and harbors the arrhythmogenic substrate for VT.