Relation of Late Gadolinium Enhancement and Extracellular Volume Fraction to Ventricular Arrhythmias in Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HC) represents a major cause of sudden cardiac death in young adults. Late gadolinium enhancement (LGE) and extracellular volume (ECV) by T1 mapping are cardiac magnetic resonance (CMR) techniques to quantify fibrosis in HC. The relationships of LGE and ECV with ventricular arrhythmia, left ventricular (LV) diastolic function, and risk factors for sudden cardiac death (SCD) in HC are unclear. We studied 103 HC patients (mean age 51 ± 14, 42% women) who underwent CMR from 2012 to 2014. Global LGE and mean ECV were evaluated in relation to history of nonsustained ventricular tachycardia (NSVT), diastolic function by echocardiography, and SCD risk factors. LGE was present in 71 (69%) subjects. Wide variation was demonstrated in LGE (0.5% to 45.9%) and mean ECV (17.6% to 47.4%). Prevalence of NSVT increased continuously with LGE and was greater in subjects with ECV above the study population mean (27%). Increased LGE was associated with LV diastolic dysfunction and LV wall thickness. In conclusion, while ECV appears to have a threshold (27%) above which it is associated with NSVT, LGE demonstrates a more robust relationship with NSVT and measures of diastolic dysfunction.