scholarly journals Radial global strain by cardiac magnetic resonance imaging predicts arrhythmic outcomes in hypertrophic cardiomyopathy

2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
P Martinez Vives ◽  
A Cecconi ◽  
A Vera ◽  
T Nogales-Romo ◽  
B Lopez-Melgar ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Score ◽  
Cardiac Death ◽  
Strain Analysis ◽  
Short Axis ◽  
Analysis Software ◽  
Tissue Tracking

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Hypertrophic cardiomyopathy (HCM) is a relatively prevalent condition associated with arrhythmic events and sudden cardiac death. Several tools are currently available to identify which HCM patients are at risk of developing these events. Purpose We aimed to evaluate the association of Tissue Tracking strain analysis by cardiac magnetic resonance (CMR) and the development of arrhythmic events in patients with HCM. Methods We prospectively analyzed 136 consecutive patients with HCM diagnosis (established according to current clinical practice guidelines) from January 2006 to October 2017. Every routine 24 hours ECG-monitoring test was registered and looked for sustained or non-sustained ventricular tachycardia (any VT). CMR studies were performed following our predefined CMR protocol for HCM with 1.5T magnets. Cine images were obtained with standard, retrospectively gated, steady-state free-precession (SSFP) sequences in 2, 3 and 4 chambers views and in 10–15 contiguous short-axis slices covering the ventricles from the base to the apex, with breath holding. The strain evaluation was performed by a commercially available Tissue Tracking analysis software, manually defining the endocardial border in short axis, 4, 3 and 2 chambers views and, after verifying adequate identification of the different structures, running the strain analysis (Figure 1, displaying myocardium identification by the strain analysis software). Results Mean follow-up was 49 ± 45 months. Mean age was 61 ± 15 years old (p for the comparation between the group with arrhythmic and the group without arrhythmic events 0.212) and 31% of patients were women (p 0.420). Mean ejection fraction was 69 ± 9.21% (p 0.223) and mean HCM-SCD (hypertrophic cardiomyopathy sudden cardiac death) risk score was 2.20 ± 1.34 (p <0.001). Median percentage of total myocardium showing late gadolinium enhancement (LGE) was 0.61 (interquartile range 2.9; p 0.170). Mean global radial strain (GRS) was 26.23 ± 8.78% (p <0.001). 21 VT episodes were recorded during follow-up. GRS showed an area under de ROC curve of 0.75 predicting VT during follow-up, selecting the value of 27% as the best sensitivity/specificity cutoff point. Statistically significant differences were not found when analyzing global circumferential strain (GCS) and global longitudinal strain (GLS) as VT predictors after adjusting for possible confusion factors (GRS, GCS and GLS distributions depicted in Figure 2). A binary GRS ≥27%/<27% variable was included in a logistic regression model adjusted by age, percent of total myocardium mass showing LGE and HCM-SCD risk score. Significantly more arrhythmic events were found to occur in patients with a GRS <27% (OR 7.33; 95% confidence interval 1.07 – 50.41; p 0.043) after adjusting by age, percent of total myocardium mass showing LGE, and HCM-SCD risk score Conclusions A GRS value of <27% on CMR appears to be a good predictor of worse arrhythmic prognosis in patients with HCM.

scholarly journals Cardiac magnetic resonance strain as a predictor of clinical events in hypertrophic cardiomyopathy

2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
P Martinez Vives ◽  
A Cecconi ◽  
A Vera ◽  
T Nogales-Romo ◽  
B Lopez-Melgar ◽  
...  
Keyword(s):  
Heart Failure ◽  
Atrial Fibrillation ◽  
Hypertrophic Cardiomyopathy ◽  
Hospital Admissions ◽  
Strain Analysis ◽  
Short Axis ◽  
Analysis Software ◽  
Clinical Events ◽  
Tissue Tracking

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Hypertrophic cardiomyopathy (HCM) is a relatively prevalent condition associated with cardiovascular death and heart failure. Several tools are currently available to identify which HCM patients are at risk of developing these events. Purpose We aimed to evaluate the association of new Tissue Tracking strain analysis by cardiac magnetic resonance (CMR) and the development of clinical events in patients with HCM. Methods We prospectively analyzed 136 consecutive patients with HCM diagnosis (established according to current clinical practice guidelines) from January 2006 to October 2017. Heart failure hospital admissions and death on follow-up were included in a combined clinical outcome. CMR studies were performed following our predefined CMR protocol for HCM with 1.5T magnets. Cine images were obtained with standard, retrospectively gated, steady-state free-precession (SSFP) sequences in 2, 3 and 4 chambers views and in 10–15 contiguous short-axis slices covering the ventricles from the base to the apex, with breath holding.  The strain evaluation was performed by a commercially available Tissue Tracking analysis software, manually defining the endocardial border in short axis, 4, 3 and 2 chambers views and, after verifying adequate identification of the different structures, running the strain analysis (Figure 1, displaying myocardium identification by the strain analysis software). Results Mean follow-up was 49 ± 45 months. Mean age was 61 ± 15.33 years old (p 0.024) and 31% of patients were women (p 0.01). 20% had atrial fibrillation (p 0.154). Mean ejection fraction was 69 ± 9.21% (p 0.762) and mean HCM-SCD (hypertrophic cardiomyopathy sudden cardiac death) risk score was 2.20 ± 1.35 (p 0.885). Mean global radial systolic strain rate (GRSsr) was -1,33 ± 0.59 s-1 (p 0.083). During follow-up, 12 heart failure hospital admissions and 14 death from any cause were registered. GRSsr showed an area under de ROC curve of 0.63 (95% confidence interval -CI- 0.51 – 0.75) predicting clinical events. The value of 1.40 s−1 was selected as the best sensitivity/specificity cutoff point. Three variables (sex, age, and previous history of atrial fibrillation) were chosen (through the allsets method) and included as adjusting variables together with <1,40 s−1/≥1.40 s−1 in a multivariate Cox’s regression model (p 0.002; AIC 99.7; Harrell C index 0.82). Patients with GRSsr <1.40 s−1 showed more clinical evens on follow-up vs those with GRSsr ≥1.40 s−1 (adjusted HR 6.57; 95% CI 2.01 – 21.49; p 0.002; Figure 2, displaying Kaplan-Meier survival estimates). Conclusions A GRSsr <1.40 s−1 emerges as a novel independent predictor of worse clinical outcomes in patients with HCM and preserved ejection fraction.

Supplementary role of left ventricular global longitudinal strain for predicting sudden cardiac death in hypertrophic cardiomyopathy

2021 ◽  
Author(s):  
Hyun-Jung Lee ◽  
Hyung-Kwan Kim ◽  
Sang Chol Lee ◽  
Jihoon Kim ◽  
Jun-Bean Park ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Score ◽  
Primary Endpoint ◽  
Cardiac Death ◽  
Longitudinal Strain ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
C Statistic

Abstract Aims We investigated the prognostic role of left ventricular global longitudinal strain (LV-GLS) and its incremental value to established risk models for predicting sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM). Methods and results LV-GLS was measured with vendor-independent software at a core laboratory in a cohort of 835 patients with HCM (aged 56.3 ± 12.2 years) followed-up for a median of 6.4 years. The primary endpoint was SCD events, including appropriate defibrillator therapy, within 5 years after the initial evaluation. The secondary endpoint was a composite of SCD events, heart failure admission, heart transplantation, and all-cause mortality. Twenty (2.4%) and 85 (10.2%) patients experienced the primary and secondary endpoints, respectively. Lower absolute LV-GLS quartiles, especially those worse than the median (−15.0%), were associated with progressively higher SCD event rates (P = 0.004). LV-GLS was associated with an increased risk for the primary endpoint, independent of the LV ejection fraction, apical aneurysm, and 2014 European Society of Cardiology (ESC) risk score [adjusted hazard ratio (aHR) 1.14, 95% confidence interval (CI) 1.02–1.28] or 2011 American College of Cardiology/American Heart Association (ACC/AHA) risk factors (aHR 1.18, 95% CI 1.05–1.32). LV-GLS was also associated with a higher risk for the composite secondary endpoint (aHR 1.06, 95% CI 1.01–1.12). The addition of LV-GLS enhanced the performance of the ESC risk score (C-statistic 0.756 vs. 0.842, P = 0.007) and the 2011 ACC/AHA risk factor strategy (C-statistic 0.743 vs. 0.814, P = 0.007) for predicting SCD. Conclusion LV-GLS is an important prognosticator in patients with HCM and provides additional information to established risk stratification strategies for predicting SCD.

scholarly journals 813 The added value of transthoracic three dimensional echocardiography in apical hypertrophic cardiomyopathy

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Valeria Rella ◽  
Mara Gavazzoni ◽  
Michele Tomaselli ◽  
Giorgio Oliverio ◽  
Valentina Volpato ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Wall Thickness ◽  
Cardiac Death ◽  
Contrast Echocardiography ◽  
Three Dimensional ◽  
Added Value ◽  
Maximal Wall Thickness ◽  
Apical Hypertrophy

Abstract We present the case of a 73-year-old patient with a recent diagnosis of hypertrophic cardiomyopathy (HCM). He was asymptomatic and has no family history of sudden cardiac death (SCD), syncope or ventricular arrhythmias. An echocardiogram performed at the moment of diagnosis (2020), showed left ventricular (LV) asymmetric apical hypertrophy with maximal wall thickness of 21 mm. Cardiac magnetic resonance (CMR) confirmed LV apical hypertrophy with mid-ventricular obliteration, and late gadolinium enhancement in the apical segments, without wall motion abnormalities present at rest. According to 2014 ESC guidelines, his calculated risk score for sudden cardiac death was low (1.23% at 5 years). On 2021, a comprehensive transthoracic echocardiographic examination including advanced techniques (three-dimensional echo-3DE-, and two-dimensional speckle-tracking-2DSTE) was done as part of his routine follow-up in our cardiomyopathy outpatient clinic. The echo study showed an asymmetric pattern of LV hypertrophy with a maximal wall thickness of 21 mm at the level of the anterolateral apical segment, normal LV volumes (end-diastolic volume 55 mL/m2) and ejection fraction (69%) by 3DE. LV longitudinal strain analysis by 2DSTE showed impaired LV myocardial deformation mainly at the apical LV segments (GLS = −13.6%). There was evidence of dynamic intracavitary obstruction (maximal gradient 32 mmHg at rest and raised to 52 mmHg during Valsalva manoeuvre). 3DE views of the LV (both multi-slice display and 3D rendered image) allowed to avoid foreshortening of the LV apical views, and to appreciate the actual wall motion at the real LV apex. They revealed a LV apical aneurysm which was not detected in the conventional LV-focused apical 2D views (Figure 1A and B). Apical hypertrophic cardiomyopathy (ApHCM) is a variant of HCM that is characteristic of focal thickening of the LV apical myocardium and was reported to have a more benign course than other non-apical forms. However, the presence of LV aneurysm in ApHCM patients is associated with an increased risk for ventricular arrhythmias, sudden cardiac death and thromboembolism. Accordingly, the detection of apical LV aneurysms has significant impact on patient management. Guidelines recommend the use of contrast echocardiography or CMR when the apical region of the LV is suboptimally visualized by conventional 2D echocardiography. However, contrast echocardiography may still be affected by apical foreshortening resulting in suboptimal accuracy, as it is a 2D technique. On the other end, CMR may be contraindicated or not widely available for routine yearly follow-up for all HCM patients requiring regular imaging follow-up. Our clinical case emphasizes the added value of 3DE to increase the sensitivity of transthoracic echocardiography in detecting apical LV aneurysms in patients with apical HCM with important clinical implications for the management of the patient. 813 Figure 1(A) 2D 4chamber-view showing maximal wall thickness in the apical segments (21 mm) with apical obliteration. At a first evaluation, apical aneurism is not easily detected. (B) 4D rendering of the apex showing the apical aneurism.

scholarly journals Long-term risk of sudden cardiac death in hypertrophic cardiomyopathy - a cardiac magnetic resonance outcome study

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
A Seitz ◽  
S Greulich ◽  
D Herter ◽  
F Guenther ◽  
S Probst ◽  
...  
Keyword(s):  
Risk Factors ◽  
Cardiac Magnetic Resonance ◽  
Hypertrophic Cardiomyopathy ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Risk Models ◽  
Lv Mass ◽  
Lge Cmr

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): Robert Bosch Stiftung; Deutsche Forschungsgemeinschaft Background Sudden cardiac death (SCD) is an appalling complication of hypertrophic cardiomyopathy (HCM). There is an ongoing discussion about the optimal SCD risk stratification strategy in HCM since established SCD risk models have suboptimal discriminative power. Objective To evaluate the prognostic value of late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) for SCD risk stratification compared to the ESC SCD risk score and traditional SCD risk factors in an >10-year follow-up study. Methods 220 consecutive patients with HCM and LGE-CMR were enrolled. Follow-up data was available in 203 patients (median age 58 years, 61% male) after a median follow-up period of 10.4 years. Results LGE was present in 70% of patients with a median LGE amount of 1.6%, the median ESC 5-year SCD risk score was 1.84. In the overall cohort, SCD rates were 2.3% at 5 years, 4.8% at 10 years, and 15.7% at 15 years, independent from established risk models. A LGE amount of >5% (LV mass) portends the highest risk for SCD with SCD prevalences of 5.5% at 5 years, 13.0% at 10 years and 33.3% at 15 years. Conversely, patients with no or ≤5% LGE amount (of LV mass) have favorable prognosis. Conclusions LGE-CMR in HCM patients allows effective 10-year SCD risk stratification beyond established risk factors. LGE amount might be added to established risk models to improve its discriminatory power. Specifically, patients with >5% amount of LGE should be carefully monitored and might be adequate candidates for primary prevention ICD during the clinical long-term course. Abstract Figure.

scholarly journals Sudden cardiac death in childhood hypertrophic cardiomyopathy is best predicted by a combination of ECG Risk‐score and HCMRisk‐Kids score

2021 ◽  
Author(s):  
Ingegerd Östman‐Smith ◽  
Gunnar Sjöberg ◽  
Jenny Alenius Dahlqvist ◽  
Per Larsson ◽  
Eva Fernlund
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Score ◽  
Cardiac Death

The diagnostic value of cardiac magnetic resonance in athletes with suspected structural myocardial diseases

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
H Vago ◽  
L Szabo ◽  
D Balla ◽  
Z.S Dohy ◽  
C.S Czimbalmos ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Sudden Cardiac Death ◽  
Magnetic Resonance ◽  
Cardiac Death ◽  
Left Ventricular ◽  
Funding Source ◽  
Research Development ◽  
Myocardial Disease ◽  
Myocardial Diseases

Abstract Introduction Sudden cardiac death (SCD) is the leading cause of death in athletes occurring usually during intensive training. Cardiac magnetic resonance (CMR) is a reliable technique to assess ventricular volumes and function. Furthermore, it provides tissue-specific information and has a crucial role in detecting structural myocardial diseases. Aim We aimed to investigate the prevalence of myocardial structural heart diseases and the etiology of sudden cardiac death in highly trained athletes and their outcome during follow-up. Method We examined athletes (training ≥6 hours/week) who underwent CMR due to suspected structural myocardial disease at Semmelweis University Heart and Vascular Center between 2009 and 2019. Cine movie images and late gadolinium enhanced (LGE) images were performed. Athletes with structural myocardial alterations were followed for the endpoint of all-cause-mortality. Results CMR was performed on a total of 338 athletes (280 male, 24±11 age). The indications for CMR were as follows: aborted sudden cardiac death/sustained ventricular tachycardia (SVT) (4%), ECG alterations (36%), echocardiographic alterations (32%), positive family history of SCD or cardiomyopathies (CMP) (3%), and patients' complaints, e.g. palpitation, syncope, dyspnoea, chest complaints (25%). CMR confirmed structural myocardial disease in 82 athletes with the following distribution: 20 hypertrophic (HCM), 10 arrhythmogenic (AC), 8 dilated (DCM), and 7 non-compact (NCCMP) CMP. The CMR images of three patients indicated Fabry disease. We found post-myocardial infarction scars in 7 cases, and atypical non-ischemic scars in 28 athletes. Besides pathological conditions, we identified minor alterations in 58 patients (51 male, 25±12 age) such as: increased trabeculation, nonspecific LGE in left ventricular insertion point and myocardial crypts. Among athletes examined after aborted sudden cardiac death or SVT we found structural heart disease in 11 males and one female: AC (n=7), HCM (n=1), NCCMP (n=1) and atypical non-ischemic scars (n=3, in two patients the localisation was lateral subepicardial) were diagnosed. During the median follow up of five years one patient died in whom CMR showed lateral scar formation and only mildly reduced left ventricular ejection fraction (50%). Conclusions The most common structural alteration was non-ischaemic scar, the most common CMP was HCM, and the leading cause of sudden cardiac death or SVT in our competitive athletes was AC and lateral subepicardial scar formation. LGE pattern in various cardiomyopathies Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): Project no. NVKP_16-1-2016-0017 has been implemented with the support provided from the National Research, Development and Innovation Fund of Hungary, financed under the NVKP_16 funding scheme. This project was supported by a grant from the National Research, Development and Innovation Office (NKFIH) of Hungary (K 120277).

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