High sensitivity troponin I in hypertrophic cardiomyopathy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
J Osmanska ◽  
A Connelly ◽  
S Nordin ◽  
A Vega ◽  
J Simpson ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Troponin I ◽  
Troponin T ◽  
High Sensitivity ◽  
Left Ventricular ◽  
Imaging Data ◽  
Pathogenic Variants ◽  
Lv Mass ◽  
Esc Guidelines ◽  
High Concentrations

Abstract Background ESC guidelines recommend measurement of troponin T in patients with hypertrophic cardiomyopathy (HCM) because high concentrations are associated with cardiovascular events, heart failure and death. The cardiac Troponin I subunit is not expressed in skeletal muscle making it a cardio-specific isoform. The use of troponin biomarkers in management of patients HCM is limited because concentrations only weakly correlate with clinical parameters. Most studies are small, and few have examined their relation with genotype and mortality. Purpose To assess the relationship between high-sensitive troponin I (hsTnI) and characteristics of adults with HCM. Methods Patients included were adults with an established diagnosis of HCM referred to a single centre for genetic testing. Demographic, clinical and imaging data were recorded at baseline. Echocardiography and cardiac magnetic resonance (CMR) were performed according to EACVI standards. Quantification of late gadolinium enhancement (LGE) was performed using the 5 SD quantitative threshold. Genotype was evaluated using a 16 gene panel in an accredited UK laboratory. Pathogenic and likely pathogenic variants were considered as a positive genotype. Serum hsTnI was measured by a two-site electrochemiluminescence immunoassay on a Roche E170 analyser. Normal values for the assay 0–34 ng/L for males and 0–16 ng/L for females. Results 313 patients (n=200, 64% male) median age 57 (IQR 47–68) years were included. hsTnI concentration was abnormal in 69 (22%) patients. An abnormal hsTnI was more common in females (n=36, 32%) compared to males (n=33, 17%, c2 9.9, p<0.05). A pathogenic variant in a sarcomere gene was identified in 95 (30%) individuals. An abnormal hsTnI concentration was associated with higher left ventricular (LV) wall thickness (20mm v 18mm, p<0.05) and LV outflow tract (LVOT) gradient (34 v 22 mmHg) on echocardiography (n=313). Of the patients (n=204) who had a CMR, an abnormal hsTnI concentration was associated with higher LV mass (183 v 156g, p<0.05) and greater % LGE (30 v 16%, p<0.01, n=129). There was no difference in hsTnI between those with a positive or negative genotype. During follow-up, 18 patients died. Of the 9 patients that died with a normal hsTnI, two died suddenly. Conclusions In HCM, patients with abnormal hsTnI concentration have higher LV mass and LVOT gradient and more fibrosis. Whilst mortality is higher in those with abnormal hsTnI, sudden cardiac death may occur with a normal hsTnI. It may not be appropriate to extrapolate hsTnI sex-specific thresholds used in the diagnosis of myocardial infarction to HCM. Funding Acknowledgement Type of funding source: None

scholarly journals Reversed Septal Curvature Is Associated with Elevated Troponin Level in Hypertrophic Cardiomyopathy

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Renata Rajtar-Salwa ◽  
Tomasz Tokarek ◽  
Paweł Petkow Dimitrow
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Troponin I ◽  
High Sensitivity ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Septal Thickness ◽  
Left Ventricular Outflow ◽  
Echocardiographic Parameters ◽  
Ischemic Events ◽  
Septal Curvature

The aim of study was to compare patients with hypertrophic cardiomyopathy divided according to septal configuration assessed in a 4-chamber apical window. The study group consisted of 56 consecutive patients. Reversed septal curvature (RSC) and non-RSC were diagnosed in 17 (30.4%) and 39 (69.6%) patients, respectively. Both RSC and non-RSC groups were compared in terms of the level of high-sensitivity troponin I (hs-TnI), NT-proBNP (absolute value), NT-proBNP/ULN (value normalized for sex and age), and echocardiographic parameters, including left ventricular outflow tract gradient (LVOTG). A higher level of hs-TnI was observed in RSC patients as compared to the non-RSC group (102 (29.2-214.7) vs. 8.7 (5.3-18) (ng/l), p = 0.001 ). A trend toward increased NT-proBNP value was reported in RSC patients (1279 (367.3-1186) vs. 551.7 (273-969) (pg/ml), p = 0.056 ). However, no difference in the NT-proBNP/ULN level between both groups was observed. Provocable LVOTG was higher in RSC as compared to non-RSC patients (51 (9.5-105) vs. 13.6 (7.5-31) (mmHg), p = 0.04 ). Furthermore, more patients with RSC had prognostically unfavourable increased septal thickness to left LV diameter at the end diastole ratio. Patients with RSC were associated with an increased level of hs-TnI, and the only trend observed in this group was for the higher NT-proBNP levels. RSC seems to be an alerting factor for the risk of ischemic events. Not resting but only provocable LVOTG was higher in RSC as compared to non-RSC patients.

Elevated level of N-terminal pro B-type natriuretic peptide is associated with myocardial fibrosis in hypertrophic cardiomyopathy patients with preserved ejection fraction

2020 ◽  
Author(s):  
Heshui Shi ◽  
Yumin Li ◽  
Jia Liu ◽  
Yukun Cao ◽  
Xiaoyue Zhou ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Ejection Fraction ◽  
Natriuretic Peptide ◽  
Myocardial Fibrosis ◽  
Troponin I ◽  
High Sensitivity ◽  
Left Ventricular ◽  
Preserved Ejection Fraction ◽  
Roc Curve Analysis ◽  
Potential Biomarker

Abstract Background: Myocardial fibrosis assessed by late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) has been reported to be significantly correlated with cardiovascular outcomes in hypertrophic cardiomyopathy (HCM) patients. However, data regarding non-invasive markers for detecting myocardial fibrosis were inconsistent and, not systematically evaluated in HCM patients with preserved ejection fraction (EF).Methods: In this study, 86 HCM patients with preserved EF and 33 controls were enrolled. The left ventricular function, end-diastolic maximum wall thickness (MWT), global systolic strains and extent of LGE (% LGE) were assessed. The biochemical indices were also recorded before the CMR examination.Results: Serum high-sensitivity cardiac troponin I (hs-cTnI) and N-terminal pro b-type natriuretic peptide (Nt-proBNP) levels were elevated in LGE-positive patients compared with LGE-negative patients (p < 0.05 for all). The LGE-positive patients had lower global longitudinal (GLS) and circumferential (GCS) strains than the LGE-negative group and the healthy controls (p < 0.05 for all). The LGE% was independently associated with the Nt-proBNP levels (standardized β = 0.627, p < 0.001), beta-blocker treatment (standardized β = -0.372, p = 0.01), MWT (standardized β = 0.481, p = 0.001) and GCS (standardized β = 0.406, p = 0.013). In the receiver operating characteristic (ROC) curve analysis, the combined parameters of Nt-proBNP ≥ 108 pg/mL and MWT ≥ 17.3 mm had good diagnostic performance for LGE, with a specificity of 81.3% and sensitivity of 70.0%.Conclusions: This study suggests that Nt-proBNP may be a potential biomarker associated with LGE% and, combined with MWT, was useful in detecting myocardial fibrosis in HCM patients with preserved EF. Additionally, LV GCS may be a more sensitive indicator for reflecting the presence of myocardial fibrosis than GLS.

P2729NT-proBNP and high-sensitivity cardiac troponin T to diagnose cardiac amyloidosis

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
V Castiglione ◽  
A Aimo ◽  
A Barison ◽  
D Genovesi ◽  
C Prontera ◽  
...  
Keyword(s):  
Cardiac Troponin ◽  
Cardiac Amyloidosis ◽  
Troponin T ◽  
High Sensitivity ◽  
Left Ventricular ◽  
Clinical Suspicion ◽  
Al Amyloidosis ◽  
Cardiac Troponin T ◽  
Lv Mass ◽  
The Difference

Abstract Background Cardiac amyloidosis (CA) is characterized by the accumulation of misfolded proteins into amyloid fibrils, leading to cardiomyocyte toxicity, extracellular volume expansion and ventricular pseudohypertrophy. As a consequence of such processes, natriuretic peptides and cardiac troponins are chronically elevated in CA and hold significant prognostic value. The diagnostic yield of these biomarkers for CA has never been explored so far. Methods Plasma levels of N-terminal fraction of pro-B-type natriuretic peptide (NT-proBNP) and high-sensitivity cardiac troponin T (hs-cTnT) were measured in 230 patients referred to a tertiary centre with the clinical suspicion of cardiac amyloidosis. The final diagnosis was established according to current protocols, which include clinical, electrocardiographic, biohumoral, instrumental (echocardiography, cardiac magnetic resonance, diphosphonate scintigraphy), and biopsy examinations. Results Patients were aged 79 (interquartile interval 73–83) years and were predominantly males (n=147, 64%). Mean left ventricular (LV) ejection fraction was 55% (48–62%), and mean LV mass indexed was 150 (120–178) g/m2. CA was confirmed in 86 patients (37%), who had either light chain (AL) amyloidosis (n=25, 29%) or transthyretin (ATTR) amyloidosis (n=61, 71%). Alternative diagnoses were hypertensive cardiopathy (n=69, 48%), valvular disease (n=27, 19%), hypertrophic cardiomyopathy (n=18, 13%), or left ventricular hypertrophy with unknown or multifactorial mechanisms. Patients with CA showed higher NT-proBNP (5507 ng/L [2348–10326] vs. 1332 [392–3752], p<0.001) and hs-cTnT (65 ng/L [48–114] vs. 35 [21–52], p<0.001) than those without CA. The area under the curve (AUC) values for NT-proBNP and hs-cTnT were 0.712 and 0.775 respectively (p=0.062 for the difference). The combination of the two biomarkers improved discrimination over NT-proBNP alone (p=0.011), but not over hs-cTnT (p=0.470) (Figure). A NT-proBNP level <600 ng/L or a hs-cTnT level <17 ng/L were optimal for ruling out amyloidosis, with a negative predictive value of 95% in both cases. Patients with AL amyloidosis had higher NT-proBNP and hs-cTnT than those with ATTR (10809 ng/L [6292–17483] vs. 3084 [1841–7624], p=0.014; 130 ng/L [64–211] vs. 61 [48–95], p=0.006). The difference was even more prominent when biomarker levels were normalized for LV mass (NT-proBNP/LV mass, 33.9 ng/L/g [20.4–53.8] vs. 10.0 [5.8–23.5], p=0.002; hs-cTnT/LV mass, 0.48 ng/L/g [0.25–0.71] vs. 0.19 [0.14–0.26], p=0.001). NT-proBNP and hs-cTnT could effectively discriminate patients with AL amyloidosis among subjects with clinical suspicion of CA (AUC values of 0.787 and 0.805 respectively) (Figure). Figure 1 Conclusions Plasma NT-proBNP and hs-cTnT have diagnostic value in patients with suspected CA. In the subgroup with CA, both biomarkers are higher in patients with AL amyloidosis even when normalizing for LV mass, possibly because of a greater cardiotoxic effect of light-chain fibrils.

scholarly journals Description of interference in the measurement of troponin T by a high-sensitivity method

2019 ◽  
Vol 29 (2) ◽  
pp. 413-419
Author(s):  
Miguel Aliste-Fernández ◽  
Gemma Sole-Enrech ◽  
Ruth Cano-Corres ◽  
Silvia Teodoro-Marin ◽  
Eugenio Berlanga-Escalera
Keyword(s):  
Troponin I ◽  
Troponin T ◽  
High Sensitivity ◽  
Signs And Symptoms ◽  
Interdisciplinary Cooperation ◽  
Size Exclusion ◽  
Exclusion Chromatography ◽  
High Concentrations ◽  
High Sensitivity Troponin T ◽  
Sensitivity Method

Introduction: Measurement of high-sensitivity troponin T (hs-TnT) has become an essential step in the diagnosis of acute myocardial infarction. This high-sensitivity method allows quantifying the concentration of troponin T in blood of healthy subjects with a lower inaccuracy compared to previous reagent generations. However, the presence of certain compounds in the sample may interfere with the result. We present a patient who had repeatedly high concentrations of hs-TnT in the serum sample that did not agreed with the signs and symptoms. In addition, ultrasensitive troponin I concentration was undetectable. Materials and methods: To investigate the presence of an interfering compound, different analysis were carried out. In order to discard macro complexes in the sample, the serum was precipitated with polyethylene glycol. In addition, the serum was incubated with Scantibodies Heterophilic Blocking Tube, which can block heterophilic antibodies. Finally, a size exclusion chromatography of the sample was performed by the manufacturer. What happened: The interfering substance was allocated into fractions with proteins of 150kDa, corresponding to high molecular weight proteins like immunoglobulin G (IgG). This compound was responsible for the falsely elevated hs-TnT results and it affected only the high-sensitivity methods. Main lesson: The detected interfering compound was probably an IgG. This type of interference must be kept in mind in front of discordant results, even if they are extremely rare. Therefore, interdisciplinary cooperation between clinicians, laboratory and manufacturer is essential.

Abstract 371: Prevalence and Spectrum of Thin Filament Mutations in 1025 Patients with Hypertrophic Cardiomyopathy

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Melissa L Will ◽  
Andrew P Landstrom ◽  
J. Martijn Bos ◽  
Bernard J Gersh ◽  
Steve R Ommen ◽  
...  
Keyword(s):  
Genetic Testing ◽  
Hypertrophic Cardiomyopathy ◽  
Cardiac Troponin ◽  
Thin Filament ◽  
Troponin I ◽  
Mutational Analysis ◽  
Troponin T ◽  
Cohort Analysis ◽  
Left Ventricular ◽  
Septal Curvature

Background Recently, genetic testing for sarcomeric hypertrophic cardiomyopathy (HCM) became a commercially available diagnostic test involving analysis of 8 HCM-associated myofilament genes, four of which encode thin filament proteins - TNNT2- encoded cardiac troponin T, TPM1 -encoded alpha tropomyosin, TNNI3- encoded cardiac troponin I, and ACTC- encoded cardiac actin. Compared to the two most common subtypes of sarcomeric HCM, much less is known about the prevalence and spectrum of thin filament HCM. Methods Comprehensive open reading frame/splice site mutational analysis of these 4 thin filament-encoding genes was performed, using polymerase chain reaction, denaturing high performance liquid chromatography, and direct DNA sequencing, on the largest assembled cohort of unrelated patients (N = 1025, 61% male, 49 ± 18 years at diagnosis, maximal left ventricular wall thickness, LVWT, 22.3 ± 7 mm) with clinically diagnosed HCM that were seen at the Mayo Clinic. Results Nineteen distinct missense mutations, involving conserved residues and absent in 600 reference alleles, were identified in 36 of 1025 patients (3.5%) including TNNT2 (16 patients), TNNI3 (12), TPM1 (7) and ACTC (1). Among these 36 patients with thin filament-HCM, their average age at diagnosis was 33.2 ± 16 years and the average LVWT was 21 ± 8 mm. Eighteen patients (50%) had evidence for familial HCM and 13% had a positive history for sudden cardiac death. Only 13 patients (36%) had a reverse septal curvature by echocardiography. Instead, 12 patients (34%) had either sigmoidal or neutral septal contours and 9 patients (25%) had apical variant-HCM. Conclusion Here, we present the largest cohort analysis of thin filament HCM to date and detail the prevalence and spectrum of HCM-associated mutations in these 4 genes. Compared to the strong association between reverse septal curvature and thick filament HCM, the septal morphology associated with thin filament HCM was quite varied. Contrary to published estimates of the frequency of troponin T-HCM (5 – 10%) and the other subtypes of thin filament HCM, the prevalence of thin filament HCM was very low in this cohort. If confirmed in other cohorts, a revised cascade for HCM genetic testing should be considered.

Abstract 14042: Weight Loss Favorably Impacts Left Ventricular Mass and Wall Thickness by CMR and CT in Patients With Hypertrophic Cardiomyopathy: A Retrospective Case Series

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Maria DeFonte ◽  
Jonathan Goldstein ◽  
Daniele Massera ◽  
Alexandra Stepanovic ◽  
Alexander Gee ◽  
...  
Keyword(s):  
Weight Loss ◽  
Hypertrophic Cardiomyopathy ◽  
Wall Thickness ◽  
Phenotypic Expression ◽  
Case Series ◽  
Left Ventricular ◽  
Retrospective Case ◽  
Pathogenic Variants ◽  
Lv Mass ◽  
The Impact

Introduction: Hypertrophic Cardiomyopathy (HCM) is a relatively common inherited heart disease with variable phenotypic expression. While pathogenic variants in sarcomeric genes are considered responsible for the development of left ventricular (LV) hypertrophy, environmental modulation of phenotype may explain the known heterogeneity. Obesity is common in HCM patients and is associated with increased LV mass in the general population. Hypothesis: We hypothesized that weight loss in obese patients with HCM would be associated with a decrease in LV mass and maximal wall thickness. Methods: Patients with HCM who achieved therapeutic weight loss and underwent cardiac MRI (CMR) or computed tomography (CT) before and afterwards were included. Standard LV measurements including wall thickness in an 18-segment model were performed blindly with respect to name, time and BMI for un-biased comparison. Results: We included 6 patients (2 female, age 55 ± 6.5 years, baseline BMI = 36.7 ± 5.2 kg/m 2 ) who achieved 16.3 ± 10.8 kg weight loss after 35 ± 12 months with diet and exercise (n=4) or bariatric surgery (n=2). After weight loss, we observed a mean proportional decrease in total LV mass of 25 ± 16% (p=0.004), and a 19 ± 7% decrease in indexed LV mass (p=0.007). Furthermore, there was a numerical decrease in mean wall thickness in 14 out of 18 LV segments measured; 9 segments had more than a 10% decrease. The most noticeable changes were at the basal inferolateral wall (25 ± 13% decrease) and basal inferoseptum (19 ± 18%) decrease (Table). Conclusions: In this series of patients with HCM, weight loss favorably affected LV mass and wall thickness. Further research is needed to explore the impact of weight loss on HCM phenotypic expression and symptoms.

scholarly journals Effects of candesartan on left ventricular hypertrophy and fibrosis in patients with hypertrophic cardiomyopathy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
H Maqsood ◽  
H.A Shakeel ◽  
H.F Shoukat ◽  
M.D Khan ◽  
S.A.Y Shah ◽  
...  
Keyword(s):  
Late Gadolinium Enhancement ◽  
Hypertrophic Cardiomyopathy ◽  
Interstitial Fibrosis ◽  
Angiotensin Receptor Blockers ◽  
Left Ventricular ◽  
Funding Source ◽  
Gadolinium Enhancement ◽  
Percent Change ◽  
Lv Mass ◽  
Significant Difference

Abstract Introduction Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular (LV) hypertrophy in the absence of pressure overload. Manifestations of the disease include heart failure associated with diastolic dysfunction and atrial and ventricular tachyarrhythmias. Pathological features of HCM include myocyte hypertrophy, interstitial fibrosis, and myocyte disarray and are mediated by angiotensin II. Purpose This study aimed to evaluate the effects of candesartan on left ventricular (LV) hypertrophy and fibrosis in patients with hypertrophic cardiomyopathy (HCM). Methods In double-blind fashion, 30 patients (6 women, 24 men; age: 55±11 years) with HCM were randomly assigned to receive placebo (n=13) or candesartan 50 mg twice a day (n=17) for 1 year. To measure LV mass and extent of fibrosis, cardiac magnetic resonance imaging was performed at baseline and 1 year as assessed by late gadolinium enhancement. Results There was a trend toward a significant difference in the percent change in LV mass (median: +5% with placebo vs. −5% with candesartan; p=0.06). There was a significant difference in the percent change in the extent of late gadolinium enhancement, with the placebo group experiencing a larger increase (+30±27% with placebo vs. −22±44% with candesartan; p=0.03). Conclusion Our study concludes reduction of the progression of myocardial hypertrophy and fibrosis with candesartan in patients with hypertrophic cardiomyopathy. Our study population was limited so we warrant larger trials to confirm a place for angiotensin receptor blockers in the management of patients with hypertrophic cardiomyopathy. Figure 1 Funding Acknowledgement Type of funding source: Other. Main funding source(s): Self funding

scholarly journals Predictive values of multiple non-invasive markers for myocardial fibrosis in hypertrophic cardiomyopathy patients with preserved ejection fraction

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yumin Li ◽  
Jia Liu ◽  
Yukun Cao ◽  
Xiaoyu Han ◽  
Guozhu Shao ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Ejection Fraction ◽  
Myocardial Fibrosis ◽  
Troponin I ◽  
Myocardial Strain ◽  
Left Ventricular ◽  
Preserved Ejection Fraction ◽  
Predictive Values ◽  
Non Invasive ◽  
Potential Biomarker

AbstractMyocardial fibrosis assessed by late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) is associated with cardiovascular outcomes in hypertrophic cardiomyopathy (HCM) patients, but little is known about the utility of non-invasive markers for detecting LGE. This study aims to explore the association between cardiac-specific biomarkers, CMR myocardial strain, left ventricular (LV) hypertrophy and LGE in HCM patients with preserved ejection fraction (EF) and investigate the predictive values of these indexes for LGE. We recruited 33 healthy volunteers and 86 HCM patients with preserved EF to undergo contrast-enhanced CMR examinations. In total, 48 of 86 HCM patients had the presence of LGE. The LGE-positive patients had significant higher serum high-sensitivity cardiac troponin I (hs-cTnI) and N-terminal pro b-type natriuretic peptide (Nt-proBNP) levels and lower global longitudinal (GLS) and circumferential (GCS) strains than the LGE-negative group. The LGE% was independently associated with the Nt-proBNP levels, GCS, LV end-diastolic maximum wall thickness (MWT) and beta-blocker treatment. In the receiver operating characteristic curve analysis, the combined parameters of Nt-proBNP ≥ 108.00 pg/mL and MWT ≥ 17.30 mm had good diagnostic performance for LGE, with a specificity of 81.25% and sensitivity of 70.00%. These data indicate that serum Nt-proBNP is a potential biomarker associated with LGE% and, combined with MWT, were useful for identifying myocardial fibrosis in HCM patients with preserved EF. Additionally, LV GCS may be a more sensitive indicator for reflecting the presence of myocardial fibrosis than GLS.

scholarly journals Prognostic significance of cardiac magnetic resonance-based markers in patients with hypertrophic cardiomyopathy

2021 ◽  
Author(s):  
Zsofia Dohy ◽  
Liliana Szabo ◽  
Attila Toth ◽  
Csilla Czimbalmos ◽  
Rebeka Horvath ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Hypertrophic Cardiomyopathy ◽  
Magnetic Resonance ◽  
Myocardial Fibrosis ◽  
Ventricular Arrhythmias ◽  
Prognostic Significance ◽  
Strain Analysis ◽  
Left Ventricular ◽  
Icd Therapy ◽  
Lv Mass

AbstractThe 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.

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