Layer‐specific longitudinal strain predicts left ventricular maximum wall thickness in patients with hypertrophic cardiomyopathy

2021 ◽  
Author(s):  
Toshimitsu Tsugu ◽  
Yuji Nagatomo ◽  
Raluca Dulgheru ◽  
Patrizio Lancellotti
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Wall Thickness ◽  
Longitudinal Strain ◽  
Left Ventricular ◽  
Maximum Wall Thickness

P1772Diagnostic accuracy of a novel electrocardiographic criterion for the diagnosis of left ventricular hypertrophy in hypertrophic cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
P M Azevedo ◽  
C Guerreiro ◽  
R Ladeiras-Lopes ◽  
R Faria ◽  
N Ferreira ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Left Ventricular Hypertrophy ◽  
Wall Thickness ◽  
Ventricular Hypertrophy ◽  
High Specificity ◽  
Left Ventricular ◽  
S Wave ◽  
Diagnostic Modality ◽  
Maximum Wall Thickness ◽  
Higher Sensitivity

Abstract Introduction The 12-lead electrocardiogram (ECG) is a fundamental initial diagnostic modality for the early evaluation of a patient suspected of having hypertrophic cardiomyopathy (HCM). ECG criteria for the diagnosis of left ventricular hypertrophy (LVH) typically have low sensitivity and high specificity. Recently, a novel ECG criterion (Peguero-Lo Presti, PLP) with higher sensitivity (62%) and similar specificity (90%) was developed in a cohort of hypertensive patients, but its accuracy in patients with HCM has not been tested. We hypothesized that Peguero-Lo Presti criterion would improve upon the sensitivity of other criteria, while maintaining high specificity, for the diagnosis of LVH in patients for with HCM. Methods We retrospectively analyzed 215 consecutive patients who underwent cardiac magnetic resonance (CMR) between 2010 and 2018 for suspected HCM. All patients aged 18 years or older, who had CMR-confirmed HCM and an ECG without confounders (complete left or right bundle brunch block or paced ventricular rhythm) were included for analysis (n=88). Left ventricular mass (LVM) index and maximum wall thickness were derived from CMR analysis. The PLP criteria was defined as the sum of the deepest S wave (SD) in any lead and the S wave amplitude of lead V4 (SV4). Cornell voltage (CL) and Sokolow-Lyon (SL) were used for comparison. We randomly selected 88 gender-matched patients who performed an ECG and CMR for other clinical reasons and who had no structural heart disease or LVH for use as controls. The DeLong and McNemar's test were used to compare ROC area under the curve (AUC) and sensitivity and specificity, respectively, between the three criteria. Results 88 patients with HCM (63% male, mean age 56.7±15 years) were analyzed. The mean maximum wall thickness was 19.9±4.4mm and mean indexed LVM was 89.7±27g/m2. 34 patients (38.6%) had increased indexed LVM and 77 (87.5%) had at least one segment with late gadolinium enhancement (LGE). Discrimination by AUC was highest for PLP (0.85 [95% CI 0.8–0.9]), compared to CL (0.79, p=0.03) and SL (0.73, p=0.02). Using literature cut-offs, the sensitivity of PLP (60% [95% CI 50–70%]) was significantly higher compared to CL (40% [95% CI 30–50%, p<0,001) and SL (41%, [95% CI 31–51%], p=0.01), whilst maintaining high specificity (PLP 96%; CL 98%; SL 94%). After adjusting for LVM, the amount of LGE had a positive correlation with PLP amplitude (Spearman's rho=0.6, coef=2.4, p=0.01), but not Cornell or Sokolow. The sensitivity of PLP was significantly higher than CL and Sokolow in patients with LGE (61% vs 44% vs 43%, p<0.05). Conclusion The Peguero-Lo Presti criteria demonstrated higher sensitivity and similar specificity when compared to the Cornell and Sokolow-Lyon criteria for the diagnosis of LVH in a cohort of patients with hypertrophic cardiomyopathy. Therefore, they could become the standard ECG diagnostic criteria in patients suspected of having LVH and HCM.

ASSESSMENT OF SEGMENTAL MYOCARDIAL STRAIN OF THE LEFT VENTRICULAR MYOCARDIUM IN CHILDREN WITH HYPERTROPHIC CARDIOMYOPATHY

2021 ◽  
Vol 100 (5) ◽  
pp. 46-52
Author(s):  
N.Yu. Chernykh ◽  
◽  
А.А. Tarasova ◽  
O.S. Groznova ◽  
I.M. Shigabeev ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Longitudinal Strain ◽  
Contractile Function ◽  
Myocardial Strain ◽  
Left Ventricular ◽  
Normative Values ◽  
Left Ventricular Myocardium ◽  
Open Label ◽  
Timely Initiation ◽  
Compensatory Increase

Assessment of segmental myocardial strain is a promising and relevant direction in the diagnosis of early impairments of left ventricular (LV) contractility in children with hypertrophic cardiomyopathy (HCM). Objective of the study: to assess the indicators of segmental myocardial strain in children with HCM. Materials and methods of research: prospective, open-label, nonrandomized, controlled. 61 patients with asymmetric HCM aged 7 to 17 years (median 9 [7,6; 13,2]) underwent echocardiography according to the standard technique with the determination of segmental longitudinal, radial, circular LV myocardium in 2D speckle tracking mode. 45 (74%) children had a non-obstructive form (NF) of HCM, 16 (26%) children had an obstructive form (OF). Obstruction was detected at the level of the LV outflow tract with a pressure gradient of 30-50 mm Hg. Results: when assessing segmental myocardial strain, a decrease in longitudinal strain was found less than the normative values in the antero-septal, anterior, antero-lateral segments in children with NF HCM with a compensatory increase in strain values in the contralateral segments (inferior septal, inferior lateral and lower ). In similar segments in children with OF, there was a significant decrease in deformity, while in the contralateral segments a less pronounced compensatory increase in deformity was determined, as well as a decrease in the minimum values of strain indicators. Assessment of segmental radial and circular strain, a statistically significant predominance of indicators in all segments, except for anterolateral, in the group with NF compared to the OF HCM was determined. A decrease in strain in the antero-septal, anterior, antero-lateral segments was found, but less pronounced compared to the indicators of longitudinal strain, as well as a compensatory increase in strain in the antero-septal values in the contralateral segments (inferior septal, inferior lateral and inferior). It has been found that the assessment of global strain values widely used in clinical practice might not be optimal enough, since too many segmental strain values are discarded and averaged, which are unevenly distributed, and therefore the assessment of strain in each segment of the myocardium in children with an asymmetric form of HCM acquires important diagnostic value. Conclusion: changes in the indicators of segmental myocardial strain reflect violations of LV contractile function and are most pronounced in OF compared with NF HCM. Their study in children is important for the timely initiation of therapy and improving the prognosis of the disease.

scholarly journals Aetiology-discriminative multimodality imaging of hypertrophic cardiomyopathy: deformation patterns relate to synchrotron-based assessment of microstructural tissue remodelling

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
F Loncaric ◽  
A Garcia-Alvarez ◽  
P Garcia-Canadilla ◽  
L Sanchiz ◽  
H Dejea ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Wall Thickness ◽  
Multimodality Imaging ◽  
Global Longitudinal Strain ◽  
Microstructural Analysis ◽  
Left Ventricular ◽  
Doppler Imaging ◽  
Deformation Analysis ◽  
Septal Myectomy ◽  
Regional Deformation

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – EU funding. Main funding source(s): Horizon 2020 European Commission Project H2020-MSCA-ITN-2016 (764738) and the Clinical Research in Cardiology grant from the Spanish Cardiac Society. Background The aetiology of left ventricular hypertrophy (LVH) is a relevant clinical challenge with consequences for patient management. Phenotypes resulting from hypertensive remodelling and sarcomere mutation often overlap. Synchrotron X-ray phase-contrast imaging (X-PCI) is a technique that can provide 3-dimensional detailed information on myocardial micro-structure non-destructively. The aim is to relate macrostructural/functional, non-invasive, imaging phenotypes of hypertrophic cardiomyopathy (HCM) to the underlying myocardial microstructure assessed with X-PCI. Methods Myocardial tissue samples were obtained from three patients (P1-3) with obstructive myocardial hypertrophy undergoing septal myectomy. Medical history and the 5-year HCM risk scores were evaluated. The patients were imaged with magnetic resonance imaging and echocardiography prior to procedure. Myocardial structure was assessed with wall thickness, late gadolinium enhancement (LGE), whereas function with speckle-tracking deformation (STE) and tissue Doppler imaging (TDI). Myectomy tissue was imaged with X-PCI in the TOMCAT beamline, using a multiscale propagation-based protocol combining a low-resolution (LR) and a high-resolution (HR) setup (5.8 and 0.7 um pixel size, respectively). Results The clinical and imaging data are shown in Fig 1. On initial assessment, wall thickness, LGE distribution, global longitudinal strain and septal TDI demonstrated a similar macrostructural and functional phenotype of P1 and P2, whereas P3 stood out with more severe hypertrophy, scarring and dysfunction. Additional regional deformation analysis with STE revealed reduced deformation in the basal and mid septum in P1, paired with a hypertensive pattern of post-systolic shortening (PSS) (yellow arrows). In comparison, in P2 and P3, deformation was more heterogeneous regionally, with regions of almost complete absence of deformation (orange arrows). Upon further exploration with TDI, areas with abnormal deformation were identified on the transition from basal to mid septum in both P2 and P3, whereas deformation was normal, but reduced in P1, and paired with PSS. LR X-PCI defined regions of interest to scan with HR (yellow frame), where HR revealed extensive interstitial fibrosis (orange arrow) with normal myocyte size and organisation in P1, compatible with severe hypertensive remodelling. However, in P2 and P3, patches of fibrosis (yellow arrow) paired with enlarged myocytes organized in visible disarray, considerably more prominent in P3, were both compatible with sarcomere-mutation HCM. Conclusion The results demonstrate multiscale phenotyping of HCM - relating micro- and macrostructural findings to function, and integrating multimodality data. In-depth regional deformation analysis, validated by synchrotron-based microstructural analysis, showed potential to identify distinct imaging phenotypes in HCM, distinguishing between overlapping presentations in different aetiologies. Abstract Figure 1

Abstract 11093: Layer-specific Quantification of Myocardial Deformation by Strain Echocardiography May Reveal the Mechanism of Preserved Left Ventricular Ejection Fraction in Patients With Hypertrophic Cardiomyopathy

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Jing Ping Sun ◽  
Xianda Ni ◽  
Tingyan Xu ◽  
Min Xu ◽  
Xing Sheng Yang ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Ejection Fraction ◽  
Wall Thickness ◽  
Radial Strain ◽  
Left Ventricular ◽  
Relative Wall Thickness ◽  
Left Ventricular Ejection ◽  
Compensatory Mechanisms ◽  
Ventricular Ejection Fraction ◽  
Bull's Eye

Purpose: We aimed to evaluate compensatory mechanisms in hypertrophic cardiomyopathy (HCM) patients (pts) with preserved left-ventricular (LV) ejection fraction (EF). Methods: Speckle-tracking echocardiography (Vivid E9, GE) was performed in 50 HCM with preserved LV EF (38 m; 49± 14 y, all LV EF > 55%) and 50 age, gender matched controls (38 m; 49±12 y). The global and segmental longitudinal (LS), circumferential (CS) and radial strain (RS) strains of endocardia (End), mid-wall and epicardia layers were analyzed using a novel layer-specific TTE. The ratio of End to epicardia strain (End/Epi) was calculated. Results: The LV EF were similar in pts and controls (64±8 vs 64±7 %, p=0.95). The diastolic function was significantly impaired in HCM pts compared with controls (E/E’:18.4±8.4 vs 8.6 ±2.4, p<0.0001). The absolute value of LS and CS was reserved at apical End layers (-34±7 vs -35±6, p=0.44); the remaining segments and LV global LS and CS of three layers were significantly smaller (LS,-16±5 vs -22±3; CS -24±8 vs -33±7; p<0.0001), and LS and CS End/Epi (1.7±0.3 vs 1.3±0.1, 3.4±1.1 vs 1.7±0.2 respectively, P <0.0001) was significantly higher in HCM pts than in controls. The RS and LV twist were preserved in all LV segments (27±10 vs 24±12, p=0.19; 20±8 vs 18±5, p=0.33; respectively). In HCM pts, the LV LS value at basal and middle levels revealed significant negative correlations with LV relative wall thickness (r=–0.65, –0.59 and –0.60, –0.54, respectively , p< 0.0001); and mild negative correlations (r=-0.33,-0.29, p<0.0001). The LV CS value at all levels revealed mild correlations with relative wall thickness (r=-0.22, p<0.05) . The LS were significantly reduced at the hypertrophic segments (Figure). Conclusions: In HCM patients with preserved LVEF, LV GLS was impaired, but apical End LS and basal End CS, LV RS as well as LV twist were maintained as the compensation for reduction LV LS and CS. The Bull’s eye of LS may help us to localize the lesion segments and define the type of HCM.

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.

P903Is left ventricular longitudinal strain a good pronostic factor in friedreich ataxia?

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
C Heuze ◽  
L Legrand ◽  
A Diallo ◽  
M L Monin ◽  
C Ewenczyk ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Wall Thickness ◽  
Longitudinal Strain ◽  
Independent Predictor ◽  
Friedreich Ataxia ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Old Patients ◽  
Ventricular Ejection Fraction

Abstract Introduction Friedreich ataxia (FRDA) is a rare genetic sensory ataxia. The causal mutation is an expanded trinucleotide repeat (GAA) in the frataxin gene. Hypertrophic cardiomyopathy is associated with FRDA and is the major cause of early death before 40 years old. Patients with progressive decline of the left ventricular ejection fraction (LVEF) have the worse prognosis. Speckle tracking echography with 2D longitudinal myocardial strain (GLS) is recognized as a more effective technique than conventional LVEF in detecting subtle changes in LV function. Purpose Evaluate the prognostic value of global longitudinal strain (GLS) in patients with FRDA as compared to LVEF. Methods From 2003 to 2017 consecutive patients with genetically confirmed FRDA were included. Longitudinal strain analysis was retrospectively performed with Tomtec software. News were obtained until April 2018, no patient was lost during follow-up. Results The study included 156 patients (51% male) of 35±12 years (mean ± SD) with an age at disease onset of 17±11 years, age at wheelchair use of 26±10 years, and GAA repeat on the shorter allele of 590±241 pb. The following echocardiographic parameters were studied at baseline: LVEF 64±9%, GLS −19.8±5% (n=141), septal wall thickness (SWT) 11.4±2.5 mm, posterior wall thickness (PWT) 10.4±1.8 mm, LV end diastolic diameter (LVEDD) 44.4±6mm. Correlation between GLS and LVEF was 0.31 (p=0.0002). After a mean follow-up of 7.7±4.0 years, 17 (11%) patients died and the outcome (cardiac arrhythmia, heart failure, stroke or death) concerned 28 (18%) patients. In univariate analysis (Cox model), factors associated with mortality were: GLS (HR: 1.2; 95% CI 1.10–1.32, p=0.0001), LVEF (HR: 0.88; 95% CI 0.85–0.92, p<0.0001), GAA (HR: 1.28; 95% CI 1.11–1.47, p=0.0008), age at onset (HR: 0.84; 95% CI 0.76–0.94, p=0.002), LVMi (HR: 1.02; 95% CI 1.01–1.04, p=0.0078), SWT (HR: 1.18 95% CI 1.01–1.36, p=0.03) and LVEDD (HR: 1.09; 95% CI 1.00–1.19, p=0.04). In multivariate analysis LVEF was the only independent predictor of long-term mortality (HR: 0.93; 95% CI 0.88; 0.99, p=0.02). Similarly GLS was not an independent predictor of the composite outcome in multivariate analysis. Conclusion GLS is a predictor of morbimortality but is not superior to LVEF in FRDA patients. Further prospective studies are mandatory to assess the early predictive value of 2D GLS.

P3839Hypertension or hypertrophic cardiomyopathy? Using cardiovascular magnetic resonance imaging to unmask the great imitator

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
T Keteepe-Arachi ◽  
A Malhotra ◽  
J Basu ◽  
G Parry-Williams ◽  
B Ensam ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hypertrophic Cardiomyopathy ◽  
Magnetic Resonance ◽  
Wall Thickness ◽  
Lifestyle Modification ◽  
Left Ventricular ◽  
Grey Zone ◽  
Resonance Imaging ◽  
Hypertensive Patients ◽  
Significant Difference

Abstract Background Structural cardiac adaptations due to hypertension (HTN) present a diagnostic challenge when differentiating from hypertrophic cardiomyopathy (HCM), using traditional imaging techniques such as echocardiography (echo). Cardiac magnetic resonance imaging (CMR) offers reproducible anatomical, functional quantification and myocardial tissue characterisation which discriminates between hypertension and HCM. Purpose To identify hypertensive individuals with undiagnosed HCM using CMR imaging. Methods 100 consecutive hypertensive patients underwent CMR at a tertiary centre dedicated blood pressure clinic (55% male, mean age 51 years). In keeping with ESC guidelines, end diastolic wall thickness (EDWT) ≥15mm identified individuals within the “grey zone” between hypertension and with a potential HCM diagnosis. 19 individuals were referred on to the dedicated inherited cardiac conditions clinic for further evaluation. Four patients expressed a definitive LV phenotype and were diagnosed with HCM. CMR parameters were compared in three groups: Hypertensive (HTN), “grey zone” Hypertensive (GZH) and HCM. Results CMR demonstrated end diastolic wall thickness (EDWT) >11 mm in 50% of hypertensives. 73% of the referred patients were Afro-Caribbean (AC) and all 4 HCM patients were AC. All referrals demonstrated EDWTs ≥14mm, 9 (47%) demonstrated late gadolinium enhancement of which 3 (16%) had HCM. Three had asymmetrical septal hypertrophy – 2 were in the HCM cohort and one underwent endomyocardial biopsy confirming HTN. Left ventricular mass index (LVMI) was significantly higher in GZH compared to HTN (p<0.0001) and in HCM compared to HTN (p=0.0004). EDWT was significantly greater in GZH compared to HTN (p<0.0001) and in HCM compared to HTN (p=0.0002). There was no significant difference in these parameters between GZH and HCM. Table 1. P-values for CMR data in hypertensive (HTN), gray zone hypertensive (GZH) and hypertrophic cardiomyopathy (HCM) cohorts CMR Parameter HCM vs. HTN GZH vs. HTN GZH vs. HCM EDVI NS p=0.025 NS EDWT p=0.0002 p<0.0001 NS LVMI p=0.0004 p<0.0001 NS Conclusion This study reports a 4% prevalence of HCM among hypertensive patients - 20 x greater than in the general population - which would be left undiagnosed using echo alone. Screening hypertensive individuals with CMR is not routine but we advocate its use in these individuals especially in Afro Caribbeans and in those in the “grey zone”, to identify undiagnosed HCM, which has significant implications for lifestyle modification and family screening. Acknowledgement/Funding Cardiac Risk in the Young

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