QRS alternans due to localized intraventricular block during ventricular tachycardia in Uhl's anomaly: a case report

Uhl's anomaly is a rare congenital heart disease characterized by 'Paper-like thinning of the right ventricular (RV) wall'. Since most patients with Uhl's anomaly die in infancy or childhood, the adult cases of this disorder have been limitedly reported and there were no past report describing this anomaly with ventricular tachycardia (VT), in which catheter ablation was successfully performed.

We report the case of a 43-year-old man with a suggested Uhl's anomaly and VT. He underwent a catheter ablation of the recurrent VT. An electrophysiological study showed the VT (cycle length = 460 ms) with a right bundle branch block pattern and inferior axis represented QRS alternans. Concealed entrainment by pacing from the anterior right ventricle (RV) suggested the mechanism of this VT was re-entry and the QRS alternans was simultaneously reproduced during the pacing. Furthermore, the intracardiac electrograms exhibiting local conduction blocks were documented on the RV free wall near the apex. The electrograms seen in a 2:1 ratio were coincident with slurs in the initial QRS on the 12-lead electrocardiogram. Since the anterior RV wall was considered to be a part of the VT circuit, a linear ablation in that area was performed resulting in VT termination.

The local conduction blocks near the RV apex were related to the mechanism of the QRS alternans during the VT in this case. That might be based on the variations in the myocardium lying in the RV, which is the specific feature of Uhl's anomaly.