Abstract
The prognosis of patients with hypertrophic cardiomyopathy (HCM) varies greatly. Cardiac magnetic resonance (CMR) is the gold standard method for assessing left ventricular (LV) mass and volumes. Myocardial fibrosis can be noninvasively detected using CMR. Moreover, feature-tracking (FT) strain analysis provides information about LV deformation. We aimed to investigate the prognostic significance of standard CMR parameters, myocardial fibrosis, and LV strain parameters in HCM patients. We investigated 187 HCM patients who underwent CMR with late gadolinium enhancement and were followed up. LV mass (LVM) was evaluated with the exclusion and inclusion of the trabeculae and papillary muscles (TPM). Global LV strain parameters and mechanical dispersion (MD) were calculated. Myocardial fibrosis was quantified. The combined endpoint of our study was all-cause mortality, heart transplantation, malignant ventricular arrhythmias and appropriate implantable cardioverter defibrillator (ICD) therapy. The arrhythmia endpoint was malignant ventricular arrhythmias and appropriate ICD therapy. The LVM index (LVMi) was an independent CMR predictor of the combined endpoint independent of the quantification method (p < 0.01). The univariate predictors of the combined endpoint were LVMi, global longitudinal (GLS) and radial strain and longitudinal MD (MDL). The univariate predictors of arrhythmia events included LVMi and myocardial fibrosis. More pronounced LV hypertrophy was associated with impaired GLS and increased MDL. More extensive myocardial fibrosis correlated with impaired GLS (p < 0.001). LVMi was an independent CMR predictor of major events, and myocardial fibrosis predicted arrhythmia events in HCM patients. FT strain analysis provided additional information for risk stratification in HCM patients.
Highlights
Hypertrophic cardiomyopathy (HCM) is a primary myocardial disease that is characterized by substantial heterogeneity in phenotypic expression, clinical course, and overall prognosis
According to the current European and American guidelines on HCM, the sudden cardiac death (SCD) risk stratification is based on age, family history of SCD, maximal left ventricular (LV) wall thickness, left atrial diameter, LV outflow tract gradient, previous unexplained syncope and the occurrence of non-sustained ventricular tachycardia [5, 6], data in the literature suggest an additional prognostic role of the detection and quantification of myocardial fibrosis using cardiac magnetic resonance (CMR) examination [7, 8]
We investigated the prognostic factors of arrhythmia events, and we found that LV endsystolic volume index (ESVi), left ventricular ejection fraction (LVEF), LVMiconv, LVMiTB, TPM index (TPMi) and myocardial fibrosis were significant univariate predictors of arrhythmia events (Table 2)
Summary
Hypertrophic cardiomyopathy (HCM) is a primary myocardial disease that is characterized by substantial heterogeneity in phenotypic expression, clinical course, and overall prognosis. According to the current European and American guidelines on HCM, the SCD risk stratification is based on age, family history of SCD, maximal LV wall thickness, left atrial diameter, LV outflow tract gradient, previous unexplained syncope and the occurrence of non-sustained ventricular tachycardia [5, 6], data in the literature suggest an additional prognostic role of the detection and quantification of myocardial fibrosis using cardiac magnetic resonance (CMR) examination [7, 8]. LV hypertrophy is usually measured using maximal end-diastolic wall thickness, and the diagnosis of HCM and SCD risk stratification are based on this parameter. Threshold-based methods define the endocardial surface based on the different signal intensities of blood and myocardium; the TPM are measured as part of the ventricular mass [9]. There is no consensus on which evaluation method is more reliable in the case of HCM [10]
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