scholarly journals Myocardial Strain and Association With Clinical Outcomes in Danon Disease: A Model for Monitoring Progression of Genetic Cardiomyopathies

Author(s):  
Quan M. Bui ◽  
Kimberly N. Hong ◽  
Megan Kraushaar ◽  
Gary S. Ma ◽  
Michela Brambatti ◽  
...  

Background Myocardial strain can identify subclinical left ventricular dysfunction in various cardiac diseases, but its association with clinical outcomes in genetic cardiomyopathies remains unknown. Herein, we assessed myocardial strain in patients with Danon disease (DD), a rare X‐linked autophagic disorder that causes severe cardiac manifestations. Methods and Results Echocardiographic images were reviewed and used to calculate myocardial strain from a retrospective, international registry of patients with DD. Regression analyses were performed to evaluate for an association of global longitudinal strain (GLS) and ejection fraction with the composite outcome (death, ventricular assist device, heart transplantation, and implantable cardioverter defibrillator for secondary prevention). A total of 22 patients with DD (male 14 [63.6%], median age 16.5 years) had sufficient echocardiograms for analysis. Absolute GLS was reduced with a mean of 12.2% with an apical‐sparing pattern observed. Univariable regression for GLS and composite outcome showed an odds ratio of 1.32 (95% CI, 1.02–1.71) with P =0.03. For receiver operating characteristic analysis, the areas under the curve for GLS and ejection fraction were 0.810 ( P =0.02) and 0.605 ( P =0.44), respectively. An absolute GLS cutoff of 10.0% yielded a true positive rate of 85.7% and false positive rate of 13.3%. Conclusions In this cohort of patients with DD, GLS may be a useful assessment of myocardial function and may predict clinical outcomes. This study highlights the potential use of myocardial strain phenotyping to monitor disease progression and potentially to predict clinical outcomes in DD and other genetic cardiomyopathies.

2021 ◽  
Vol 20 (1) ◽  
Author(s):  
Ke Shi ◽  
Meng-Xi Yang ◽  
Shan Huang ◽  
Wei-Feng Yan ◽  
Wen-Lei Qian ◽  
...  

Abstract Background Heart failure with preserved ejection fraction (HFpEF) is a heterogeneous syndrome with sex-specific pathophysiology. Estrogen deficiency is believed to be responsible for the development of HFpEF in women. However, estrogen deficiency does not seem to be completely responsible for the differences in HFpEF prevalence between sexes. While diabetes mellitus (DM) frequently coexists with HFpEF in women and is associated with worse outcomes, the changes in myocardial contractility among women with HFpEF and the DM phenotype is yet unknown. Therefore, we aimed to investigate sex-related differences in left ventricular (LV) contractility dysfunction in HFpEF comorbid with DM. Methods A total of 224 patients who underwent cardiac cine MRI were included in this study. Sex-specific differences in LV structure and function in the context of DM were determined. LV systolic strains (global longitudinal strain [GLS], circumferential strain [GCS] and radial strain [GRS]) were measured using cine MRI. The determinants of impaired myocardial strain for women and men were assessed. Results The prevalence of DM did not differ between sexes (p > 0.05). Despite a similar LV ejection fraction, women with DM demonstrated a greater LV mass index than women without DM (p = 0.023). The prevalence of LV geometry patterns by sex did not differ in the non-DM subgroup, but there was a trend toward a more abnormal LV geometry in women with DM (p = 0.072). The magnitudes of systolic strains were similar between sexes in the non-DM group (p > 0.05). Nevertheless, in the DM subgroup, there was significant impairment in women in systolic strains compared with men (p < 0.05). In the multivariable analysis, DM was associated with impaired systolic strains in women (GLS [β = 0.26; p = 0.007], GCS [β = 0.31; p < 0.001], and GRS [β = −0.24; p = 0.016]), whereas obesity and coronary artery disease were associated with impaired systolic strains in men (p < 0.05). Conclusions Women with DM demonstrated greater LV contractile dysfunction, which indicates that women with HFpEF comorbid with DM have a high-risk phenotype of cardiac failure that may require more aggressive and personalized medical treatment.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A Alfuhied ◽  
M Alfarih ◽  
P Kumar M ◽  
G Captur ◽  
P Nihoyannopoulos

Abstract Introduction Optimal timing of intervention in aortic stenosis (AS) is key. Left ventricular deformation parameters are sensitive markers of myocardial wellbeing in AS, but data is sparse so strain does not currently feature in the clinical decision-making process governing timing of AS intervention. We explore the trajectory of myocardial strain in AS of varying severity to potentially identify the most sensitive periods for valve intervention. Methods 50 patients from across the spectrum of isolated AS were prospectively recruited: mild AS n = 10; moderate AS n = 10; and severe AS n = 30 of which n = 10 in each of 3 ejection fraction (EF) groups: EF &gt; 50%, EF = 40-49% and EF &lt; 35%. Longitudinal, circumferential, and radial strain were measured by speckle tracking echocardiography (STE) using TomTec software. Results Strain parameters and EF were not significantly different between mild and moderate AS groups but almost all parameters (except for global circumferential strain (GCS)) showed significant deterioration with progression to severe AS, even in spite of preserved EF (&gt;50%; Table1). In patients with severe AS, strain parameters showed no significant differences between hearts with EF &gt; 50% and EF 40-49%, but they continued to deteriorate significantly (p &lt; 0.05) as hearts transitioned from 40-49% to &lt;35% (Figure 1, a&b). Conclusion The heart can maintain its deformation and global systolic function throughout mild and moderate AS but strain deteriorates significantly in severe AS, irrespective of EF. Tracking the precise timings of strain deterioration in severe AS could help refine optimal timings of AS intervention. LVEF and strain in varying AS severity Left ventricle ejection fraction and strain values in varying severity of AS Mild (n = 10) Moderate (n = 10) Severe (n = 10) P-value Mean (SD) Mean (SD) Mean (SD) EF (%) 65.4 (6.1) 65.8 (9.0) 60.4 (3.6)*§ 0.15 GLS (%) -14.2 (2.1) -13.6 (2.3) -11.8 (2.9)* 0.09 GCS (%) -30.3 (9.1) -31.0 (11.4) -26.5 (8.2) 0.55 GRS (%) 55.9 (15.5) 52.6 (24.0) 35.9 (13.5)*§ 0.05 EF: ejection fraction; GLS: global longitudinal strain; GCS: global circumferential strain; GRS: global radial strain; SD: standard deviation. Severe (n = 10): Severe AS with preserved EF &gt;50%. P-value: ANOVA-single factor; comparison in-between groups was performed using unpaired t-test (parametric) and Mann Whitney test (non-parametric). *significant difference P &lt; 0.05 mild vs severe. §significant difference P &lt; 0.05 moderate vs severe. Abstract P307 Figure.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yumin Li ◽  
Jia Liu ◽  
Yukun Cao ◽  
Xiaoyu Han ◽  
Guozhu Shao ◽  
...  

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.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Jun Shitara ◽  
Ryo Naito ◽  
Takatoshi Kasai ◽  
Hirohisa Endo ◽  
Hideki Wada ◽  
...  

Abstract Background The aim of this study was to determine the difference in effects of beta-blockers on long-term clinical outcomes between ischemic heart disease (IHD) patients with mid-range ejection fraction (mrEF) and those with reduced ejection fraction (rEF). Methods Data were assessed of 3508 consecutive IHD patients who underwent percutaneous coronary intervention (PCI) between 1997 and 2011. Among them, 316 patients with mrEF (EF = 40–49%) and 201 patients with rEF (EF < 40%) were identified. They were assigned to groups according to users and non-users of beta-blockers and effects of beta-blockers were assessed between mrEF and rEF patients, separately. The primary outcome was a composite of all-cause death and non-fatal acute coronary syndrome. Results The median follow-up period was 5.5 years in mrEF patients and 4.3 years in rEF patients. Cumulative event-free survival was significantly lower in the group with beta-blockers than in the group without beta-blockers in rEF (p = 0.003), whereas no difference was observed in mrEF (p = 0.137) between those with and without beta-blockers. In the multivariate analysis, use of beta-blockers was associated with reduction in clinical outcomes in patients with rEF (hazard ratio (HR), 0.59; 95% confidence interval (CI), 0.36–0.97; p = 0.036), whereas no association was observed among those with mrEF (HR 0.74; 95% CI 0.49–1.10; p = 0.137). Conclusions Our observational study showed that use of beta-blockers was not associated with long-term clinical outcomes in IHD patients with mrEF, whereas a significant association was observed in those with rEF.


Author(s):  
Akshar Jaglan ◽  
Sarah Roemer ◽  
Ana Cristina Perez Moreno ◽  
Bijoy K Khandheria

Abstract Aims Myocardial work (MW) is a novel parameter that can be used in a clinical setting to assess left ventricular (LV) pressures and deformation. We sought to distinguish patterns of global MW index in hypertensive vs. non-hypertensive patients and to look at differences between categories of hypertension. Methods and results Sixty-five hypertensive patients (mean age 65 ± 13 years; 30 male) and 15 controls (mean age 38 ± 12 years; 7 male) underwent transthoracic echocardiography at rest. Hypertensive patients were subdivided into Stage 1 (n = 32) and Stage 2 (n = 33) hypertension based on 2017 American College of Cardiology guidelines. Exclusion criteria were suboptimal image quality for myocardial deformation analysis, reduced ejection fraction, valvular heart disease, intracardiac shunt, and arrhythmia. Global work index (GWI), global constructive work (GCW), global wasted work (GWW), and global work efficiency were estimated from LV pressure–strain loops utilizing proprietary software from speckle-tracking echocardiography. LV systolic and diastolic pressures were estimated using non-invasive brachial artery cuff pressure. Global longitudinal strain and LV ejection fraction were preserved between the groups with no statistically significant difference, whereas there was a statically significant difference between the control and two hypertension groups in GWI (P = 0.01), GCW (P &lt; 0.001), and GWW (P &lt; 0.001). Conclusion Non-invasive MW analysis allows better understanding of LV response under conditions of increased afterload. MW is an advanced assessment of LV systolic function in hypertension patients, giving a closer look at the relationship between LV pressure and contractility in settings of increased load dependency than LV ejection fraction and global longitudinal strain.


Kardiologiia ◽  
2021 ◽  
Vol 61 (8) ◽  
pp. 68-75
Author(s):  
E. K. Serezhina ◽  
A. G. Obrezan

This systematic review is based on 19 studies from Elsevier, PubMed, Embase, and Scopus databases, which were found by the following keywords: LA strain (left atrial strain), STE (speckle tracking echocardiography), HF (heart failure), and HFpEF (heart failure with preserved ejection fraction). The review focuses on results and conclusions of studies on using the 2D echocardiographic evaluation of left atrial (LA) myocardial strain for early diagnosis of HFpEF in routine clinical practice. Analysis of the studies included into this review showed a significant decline of all LA functions in patients with HFpEF. Also, multiple studies have reported associations between decreased indexes of LA strain and old age, atrial fibrillation, left ventricular hypertrophy, left and right ventricular systolic dysfunction, and LV diastolic dysfunction. Thus, the review indicates significant possibilities of using indexes of LA strain in evaluation of early stages of both systolic and diastolic myocardial dysfunction. Notably, LA functional systolic and diastolic indexes are not sufficiently studied despite their growing significance for diagnosis and prognosis of patients with HFpEF. For this reason, in addition to existing models for risk stratification in this disease, including clinical characteristics and/or echocardiographic data, future studies should focus on these parameters. 


Author(s):  
Marcio Silva Miguel Lima ◽  
Hector R Villarraga ◽  
Maria Cristina Donadio Abduch ◽  
Marta Fernandes Lima ◽  
Cecilia Beatriz Bittencourt Viana Cruz ◽  
...  

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