Prognostic Value of Quantitative Contrast-Enhanced Cardiovascular Magnetic Resonance for the Evaluation of Sudden Death Risk in Patients With Hypertrophic Cardiomyopathy

Abstract

M ore than 50 years after its contemporary description, hypertrophic cardiomyopathy (HCM) remains the most common cause of sudden death in the young. [1][2][3][4][5][6] Although several clinical markers have proved to be useful guides for risk stratification, 3-5,7 current strategies do not identify all HCM patients at risk for sudden death. 3,5,8,9 Over the last Background-Hypertrophic cardiomyopathy (HCM) is the most common cause of sudden death in the young, although not all patients eligible for sudden death prevention with an implantable cardioverter-defibrillator are identified. Contrast-enhanced cardiovascular magnetic resonance with late gadolinium enhancement (LGE) has emerged as an in vivo marker of myocardial fibrosis, although its role in stratifying sudden death risk in subgroups of HCM patients remains incompletely understood. Methods and Results-We assessed the relation between LGE and cardiovascular outcomes in 1293 HCM patients referred for cardiovascular magnetic resonance and followed up for a median of 3.3 years. Sudden cardiac death (SCD) events (including appropriate defibrillator interventions) occurred in 37 patients (3%

Late gadolinium enhancement in Brugada syndrome: A marker for subtle underlying cardiomyopathy?

“…(16,17) In non-ischemic cardiomyopathies including dilated cardiomyopathy and hypertrophic cardiomyopathy, arrhythmogenesis has been associated with presence of late gadolinium enhancement (LGE) representing focal myocardial fibrosis on CMR. (18,19) Systematic assessment for myocardial fibrosis in BrS has not been performed and therefore we sought to characterise a cohort of patients using the CMR LGE technique.…”

Myocardial energy depletion and dynamic systolic dysfunction in hypertrophic cardiomyopathy

“…Volumetric cardiac MR delayed hyperenhancement (expressed as a percentage of the LV mass) has been associated with sudden cardiac death or aborted sudden death events 78 . The relationship with fibrosis would seem to validate the re-entry mechanism as a cause for monomorphic ventricular arrhythmias in HCM.…”

“…Even in patients with a level of fibrosis of 15% of LV mass have a rate of sudden cardiac death or aborted sudden death of barely more than 1% per year. 78 …”

Myocardial Scar Delineation Using Diffusion Tensor Magnetic Resonance Tractography

“…In ischemic cardiomyopathy, delineation of the extent of infarction and the area of viable myocardium plays an important role in patient management and the assessment of long‐term prognosis 1, 2, 3, 4. Myocardial tissue characterization is also of value in the diagnosis and assessment of a number of nonischemic cardiomyopathies 5, 6, 7. In addition, scar‐imaging techniques are increasingly used in the preprocedural assessment of the underlying arrhythmic substrate for patients undergoing catheter ablation for ventricular arrhythmias,8 for which intraprocedural scar definition is performed using endocardial or epicardial voltage mapping 8, 9…”

“…A variety of imaging modalities and approaches have been used to assess myocardial injury, infiltration, and viability, most notably late gadolinium enhancement (LGE),1, 2, 3, 5, 6, 7 and positron emission tomography 10, 11, 12. LGE has emerged as a leading technique for tissue characterization of the myocardium; however, several issues complicate its use.…”