Case of the Week: Top 5 2020 Competition-Ventricular Tachycardia and Endocardial Fibroelastosis In Congenital Pulmonic Stenosis

Ventricular Tachycardia and Endocardial Fibroelastosis In Congenital Pulmonic Stenosis

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Video Summary

A 24-year-old man with neonatal critical pulmonic stenosis presented with palpitations and presyncope. Monitoring revealed sustained monomorphic ventricular tachycardia. Echocardiography and cardiac MRI showed right ventricular dilation, dysfunction, pulmonic regurgitation, and basal septal dyskinesis. Dark-blood late gadolinium enhancement imaging using FIDL revealed diffuse subendocardial enhancement of the right ventricle extending into the right atrium, consistent with endocardial fibroelastosis (EFE), later confirmed at electrophysiology study. He underwent successful dual-chamber ICD implantation. The case highlights EFE as a potential consequence of congenital valvular disease and the value of dark-blood MRI for detecting subendocardial scarring.

Keywords

endocardial fibroelastosis

ventricular tachycardia

cardiac MRI

pulmonic stenosis

ICD implantation