scholarly journals Fibrosis and wall thickness affect ventricular repolarization dynamics in hypertrophic cardiomyopathy

2018 ◽  
Vol 23 (6) ◽  
pp. e12582 ◽  
Author(s):  
Mikko Jalanko ◽  
Heikki Väänänen ◽  
Mika Tarkiainen ◽  
Petri Sipola ◽  
Pertti Jääskeläinen ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Wall Thickness ◽  
Ventricular Repolarization ◽  
Repolarization Dynamics

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.

scholarly journals P1416 Clinical importance of consecutive transthoracic echocardiography in the patients with hypertrophic cardiomyopathy

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
K Kim ◽  
H R Kim ◽  
M G Kang ◽  
H Y Park ◽  
J S Koh ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Wall Thickness ◽  
Transthoracic Echocardiography ◽  
Clinical Importance ◽  
P Value ◽  
Limited Data ◽  
Clinical Predictors ◽  
Total N ◽  
Clinical Benefits

Abstract Funding Acknowledgements nothing OnBehalf nothing Background prediction of outcomes Hypertrophic cardiomyopathy (HCM) have been robustly analyzed with echocardiography. However, there is limited data of serial follow-up (FU) transthoracic echocardiography (TTE) to predict outcomes in patients with HCM. Objectives This study aim is to discover clinical predictors associated with consecutive TTE follow-up in patients with HCM. Methods From 2010 to 2016, 162 patients with HCM were enrolled retrospectively. Concentric LVH and others systolic disease related to wall thickness were excluded. Index TTE (baseline) was measured when firstly admitted in our hospital. FU TTE was analyzed at the end of follow-up, defined as the last recorded value in patients who did not develop events or the last recorded value before events developed. Results The average of FU TTE and clinical FU period was 3.7 ± 2.0 years. Clinical outcomes were defined as stroke, syncope, heart failure, arrhythmia and death. Interestingly, only baseline TR V max was a predictor for clinical outcome whereas the others echo parameters were not associated with events (Table 1). KM curve showed the TR Vmax ≥2.5m/s was also significant (log rank = 0.008, Fig 1.) Conclusions Our study showed short-term FU TTE did not bring clinician with clinical benefits in the aspect of prediction for events. Only baseline TR V max was good correlation with cardiovascular outcomes and even in the survival analysis. Serial TTE and changed values Total N = 162 index TTE (baseline) FU TTE Change of FU per year event no event p-value event no event p-value event no event p-value IVDd, mm 14 ± 4 15 ± 5 0.500 15 ± 5 14 ± 5 0.758 0.23 ± 0.51 -0.07 ± 1.27 0.200 LVIDd, mm 47 ± 5 48 ± 6 0.256 47 ± 7 48 ± 6 0.560 -0.22 ± 2.79 0.10 ± 2.27 0.444 LVEF, % 62 ± 5 61 ± 7 0.379 61 ± 6 61 ± 10 0.927 -0.43 ± 3.10 -0.04 ± 4.94 0.620 LAVI 43 ± 9 43 ± 8 0.879 57 ± 27 58 ± 23 0.849 0.53 ± 14.5 3.11 ± 7.2 0.134 EA ratio 0.9 ± 0.6 0.9 ± 0.6 0.782 1.0 ± 0.8 0.9 ± 0.6 0.595 -0.02 ± 0.76 0.003 ± 0.027 0.594 DT,ms 196 ± 58 201 ± 62 0.603 203 ± 91 217 ± 89 0.370 17 ± 57 5 ± 40 0.154 septal e` 4.4 ± 2.1 4.2 ± 1.6 0.585 4.4 ± 1.6 4.6 ± 1.7 0.438 0.24 ± 0.91 0.05 ± 0.65 0.190 E of e` 17 ± 11 17 ± 23 0.993 15 ± 9 15 ± 6 0.726 -0.48 ± 4.42 -1.66 ± 22.78 0.728 TR velocity 2.6 ± 0.5 2.4 ± 0.4 0.012 2.7 ± 0.6 2.6 ± 0.4 0.604 0.05 ± 0.30 0.04 ± 0.18 0.905 Max wall thickness 17 ± 3 18 ± 3 0.137 17 ± 4 17 ± 3 0.888 -0.01 ± 2.19 -0.18 ± 1.14 0.522 Abstract P1416 Figure. TR Vmax and CV outcomes in the KM curve

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

Increased Right Ventricular Wall Thickness in Hypertrophic Cardiomyopathy

1988 ◽  
Vol 2 (3) ◽  
pp. 134-139 ◽  
Author(s):  
J. Candell-Riera ◽  
A. Alvarez-Auñón ◽  
F. Balda-Caravedo ◽  
H. Garcia-del-Castillo ◽  
G. Permanyer-Miralda ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Right Ventricular ◽  
Wall Thickness ◽  
Ventricular Wall ◽  
Right Ventricular Wall ◽  
Ventricular Wall Thickness

P1248Characteristics and clinical significance of right ventricular involvement in patients with hypertrophic cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
J W Seo ◽  
K U Choi ◽  
J Y Choi ◽  
P J Lkhagvasuren ◽  
C Y Shim ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Ejection Fraction ◽  
Right Ventricular ◽  
Clinical Significance ◽  
Wall Thickness ◽  
Systolic Pressure ◽  
Cardiovascular Death ◽  
Cardiovascular Outcomes ◽  
Volume Index ◽  
Lv Ejection Fraction

Abstract Background A few studies have demonstrated bi-ventricular hypertrophy in hypertrophic cardiomyopathy (HCM). However, clinical significance of HCM with right ventricular (RV) involvement has not been fully established. Therefore, we aimed to investigate the structural characteristics and clinical significance of RV hypertrophy in patients with HCM. Methods In a single center, large HCM registry, 256 patients with HCM who underwent both cardiac magnetic resonance (CMR) and transthoracic echocardiography within 6 months were retrospectively analyzed. RV involvement was defined as increased RV wall thickness >7 mm on CMR in any segments of RV free wall and apex. Patients who had evidence of significant RV pressure overload (RV systolic pressure >50mmHg) or had undergone septal myectomy were excluded. Cardiovascular outcomes were defined as the composite of cardiovascular death and hospitalization for cardiovascular disease. Results Among 256 patients, 41 (16%) showed RV involvement. During follow-up period (median 1099 days), 32 cardiovascular outcomes (3 cardiovascular death and 29 cardiovascular hospitalization) were occurred. Patients with RV involvement showed a significantly higher left ventricular (LV) thickness (23.8±5.8 vs. 21.2±5.0 mm, p=0.004), more advanced diastolic dysfunction, and larger left atrial volume index (LAVI, 48.5±20.3 vs. 40.2±14.9 mm/m2, p=0.016) compared to those without RV involvement. In multivariate cox regression analysis, presence of RV involvement (HR: 4.21, 95% CI: 1.99–8.90, p<0.001) and LV ejection fraction <50% (HR: 4.29, 95% CI: 1.37–13.43, p=0.012) were independently associated with cardiovascular outcomes. The Kapan-Meier curve showed that there was a significant decrease in probability of cardiovascular outcomes-free survival in patients with RV involvement (p=0.007) after using 1:1 propensity score matching (n=82) to adjust for age, sex, LV ejection fraction, LV maximal wall thickness, LAVI, and RV systolic pressure than patients without RV involvement (Figure 1). Figure 1 Conclusion RV involvement in patients with HCM were not rare (16%). Patients with RV involvement showed more advanced LV structure and dysfunction, suggesting an indicator of severe HCM. RV involvement in HCM has clinical significance related to cardiovascular outcomes.

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