Intracardiac ultrasound to detect aneurysm in arrhythmogenic right ventricular dysplasia/cardiomyopathy

Abstract

Arrhythmogenic right ventricular dysplasia/cardiomyopathy is a genetic disorder characterized by fibrofattty replacement of the right ventricular myocardium. In the revised 2010 Task Force Criteria, a major criteria for ARVD/C is the presence of RV aneurysm by 2D echo. Our report demonstrates that intracardiac ultrasound can detect RV aneurysms and also focal absence of trabeculations which brings additional value to the diagnosis of ARVD/C. A 26-year-old male patient suffering from multiple sustained episodes of ventricular tachycardia was implanted with an internal cardiac defibrillator after confirmation of the disease by cardiac magnetic resonance imaging. Intracardiac ultrasound was performed using a 6F, 9 MHz catheter and the iLAB intravascular system (Boston Scientific). Images of right ventricular inflow tract, outflow tract, apex and pulmonary artery were taken and saved on videotape. ICE revealed loss of trabecular structure at the right ventricular outflow tract with an antero-septal aneurysm at this level. Intravascular ultrasound provides useful information on the presence of aneurysms and of tissue characterization.

Figure 1:

Intracardiac ultrasound of the right ventricular outflow tract and right ventricular apex in a patient with arrhythmogenic right ventricular dysplasia/cardiomyopathy. (A) At the level of the RVOT an aneurysm is present (red arrow) that was also identified during contrast-enhanced computed tomography. Lack of trabeculations is a marker of ARVD/C. (B) At the level of the apex, trabeculations are present (yellow arrow); in this particular patient the apex was not affected by dysplasia.