Utility of 4-Dimensional echocardiography and left ventricular systolic strain measured by 2-dimensional speckle-tracking for Danon Disease- a case series

Background Danon disease is an X-linked multisystemic disorder characterised by skeletal myopathy, cardiomyopathy and intellectual disability. Summary of cases Herein we describe two patients affected by Danon disease from the same family, a father (patient 1) and his daughter (patient 2). In patient 1 a short PR interval with pre-excitation was evident. In patient 2, over a 24-hour period 2369 atrial premature beats and rare isolated ventricular ectopics were detected. Both patients exhibited left ventricular hypertrophy with non-compaction myocardium, and the left ventricular ejection fraction was impaired in patient 1 and normal in patient 2. In patient 2, the total left ventricular strain value was reduced, and layer-specific strain revealed that subepicardial strain impaired more than in other layers. Late gadolinium enhancement was detected both in left and right ventricles in patient 2, and cardiac fibrosis was more apparent in the subepicardium of left ventricular free wall. Four-dimensional echocardiography revealed that left atrial reservoir strain and left ventricular total longitudinal strain were induced. Discussion Novel four-dimensional echocardiography and left ventricular systolic strain may play important role in diagnosis and myocardial functional evaluation in Danon disease.

Cardiac Involvement in Human Immunodeficiency Virus Infected Patients: An Observational Cardiac Magnetic Resonance Study

Objectives: To investigate the subclinical imaging changes in terms of myocardial inflammation and fibrosis and to explore the risk factors associated with myocardial fibrosis by cardiac magnetic resonance (CMR) approach in a Chinese HIV/AIDS cohort.Methods: We evaluated myocardial function (cine), myocardial inflammation (T1, T2), and myocardial fibrosis (through extracellular volume fraction [ECV] and late gadolinium enhancement [LGE]) by a multiparametric CMR scan protocol in a total of 68 participants, including 47 HIV-infected individuals, who were divided into two groups: asymptomatic HIV (HIV+) (n = 30), and acquired immunodeficiency syndrome (AIDS) (n = 17), and 21 healthy controls.Results: HIV-infected patients had lower left (55.3 ± 6.5 vs. 63.0 ± 7.9%, P < 0.001) and right ventricular systolic function (35.9 ± 15.7 vs. 50.8 ± 9.3%, P < 0.001). Radial systolic strain (30.7 ± 9.3 vs. 39.3 ± 9.4%, P = 0.001), circumferential systolic strain (−17.5 ± 2.6 vs. −19.4 ± 2.7%, P = 0.008), and longitudinal systolic strain (−9.4 ± 5.7 vs. −12.8 ± 3.1%, P = 0.012) were also decreased in HIV. Native T1 relaxation time (1,337.2 ± 70.2 vs. 1,249.5 ± 47.0 ms, P < 0.001), ECV value (33.5 ± 6.2 vs. 28.5 ± 2.9 ms, P = 0.026), and T2 relaxation time (45.2 ± 3.5 vs. 42.0 ± 2.6 ms, P = 0.001) were higher in HIV-infected patients compared with controls. Myocardial fibrosis, predominantly in the mid-inferior wall, was detected in 24.4% of the HIV-infected patients. HIV+ had a significantly lower value of ECV [29.1 (26.1, 31.8) vs. 35.2 (31.8, 41.9) %, P < 0.001] and frequency of LGE [3/25 (8%) vs. 7/16 (43.8%), P = 0.014)] compared with AIDS. AIDS was associated with myocardial fibrosis.Conclusions: HIV-infected patients were associated with changes in myocardial function and higher rates of subclinical myocardial inflammation and fibrosis, which were more abnormal with greater severity of the disease. AIDS was associated with myocardial fibrosis, where the observations supported earlier initiation of antiretroviral therapy in the Chinese HIV/AIDS cohort.

Heart function in juvenile idiopathic arthritis patients: A biventricular two-dimensional speckle-tracking echocardiography study

ABSTRACT Objectives We evaluated cardiac function in juvenile idiopathic arthritis (JIA) patients by 2D speckle-tracking echocardiography (2DSTE) and to assess possible associations with clinical, laboratorial, and treatment data. Methods A group of 42 JIA patients and 42 healthy controls were evaluated using both conventional echocardiography and 2DSTE. JIA patients underwent clinical and laboratory assessment. Results Conventional echocardiography data demonstrated normal left ventricular (LV) ejection fraction in both groups (71 vs. 71%; p = .69). 2DSTE analysis demonstrated that JIA patients presented significantly lower LV global systolic longitudinal strain (LVGLS) (−18.76 vs. −22%; p < .0001), LV systolic strain rate (LVSSR) (1.06 vs. 1.32 s−1; p < .0001), LV diastolic strain rate (LVDSR) (1.58 vs. 1.8 s−1; p < .0137), right ventricular global systolic strain (RVGLS) (−24.1% vs. −27.7%; p = .0002), and right ventricular systolic strain rate (RVSSR) (1.4 vs. 1.8 s−1; p = .0035). JIA patients under biological agents presented higher LVGLS (p = .02) and RVLS (p = .01). We also detected an association between LVGLS and C-reactive protein [CRP; −20% in normal CRP (10/42) vs. −18% in elevated CRP patients (32/42), p = .03]. Conclusions JIA patients present different echocardiographic status from healthy patients. Moreover, our data suggest that JIA patients under biological agents present association with better cardiac function as shown by strain analysis.

Echocardiographic Evaluation of Changes in Cardiac Hemodynamics, Loading Conditions, and Atrial Function after Transcatheter Closure of Atrial Septal Defect

Background The changes in loading conditions, atrial function, and the different echocardiographic parameters before and after transcatheter atrial septal defect (ASD) closure are still under study. So we felt the need to evaluate the echocardiographic changes that occur and detect the timing after closure at which the right-sided heart hemodynamics, and measurements are back to normal. Objectives To evaluate the changes in cardiac hemodynamics, loading conditions, and atrial function after percutaneous closure of ASD using echocardiography. Patients and Methods The study included 30 patients referred to percutaneous closure of ASD in Ain Shams University hospital we performed echocardiography before, 1 week, and 3 months after closure. Results The study showed that RV dimensions and volumes decreased significantly 1 week, and 3 months after ASD closure (p < 0.001). RA dimensions and volumes decreased significantly 1 week, and 3 months after ASD closure (p < 0.001). RA peak systolic strain, and strain rate increased significantly 1 week, and 3 months after ASD closure (p < 0.001). LA dimensions and volumes increased significantly 1 week, and 3 months after ASD closure (P < 0.001). LA peak systolic strain showed no significant difference before, 1week, and 3 months after ASD closure (P = 0.063), and strain rate showed no significant difference before, 1week, and 3 months after ASD closure (P = 0.207). Conclusion In our study, we have concluded that RV dimensions and volumes decreased significantly 1 week, and 3 months after ASD closure. RA dimensions and volumes decreased significantly 1 week, and 3 months after ASD closure. RA peak systolic strain, and strain rate increased significantly 1 week, and 3 months after ASD closure, as a result of improvement of the RA wall velocity, due to relief of the volume overload after closure of the shunt. LA peak systolic strain, and strain rate showed no significant difference before, 1week, and 3 months after ASD closure. Abbreviations list ASD (atrial septal defect), RV (right ventricle), RA (right atrium), LA (left atrium).

Incremental value of early systolic lengthening and postsystolic shortening in detecting left anterior descending artery stenosis using nonstress speckle-tracking echocardiography

The diagnosis of coronary artery disease (CAD) with nonstress echocardiography remains challenging. Although the assessment of either early systolic lengthening (ESL) or postsystolic shortening (PSS) allows the sensitive detection of CAD, it is unclear whether the integrated analysis of ESL and PSS in addition to the peak systolic strain can improve the diagnostic accuracy. We investigated the incremental value of ESL and PSS in detecting left anterior descending artery (LAD) stenosis using nonstress speckle-tracking echocardiography. Fifty-nine patients with significant LAD stenosis but without visual wall motion abnormalities on echocardiography at rest (30 single-vessel stenosis, 29 multivessel stenosis) and 43 patients without significant stenosis of any vessel were enrolled. The peak systolic strain, the time to ESL (TESL), and the time to PSS (TPSS) were analyzed in all LAD segments, and the incremental values of the TESL and TPSS in detecting LAD stenosis and the diagnostic accuracy were evaluated. In the apical anterior segment, the peak systolic strain was significantly lower and TESL and TPSS were significantly longer in the single-vessel group than in the no stenosis group. In the single-vessel group, the addition of TESL and TPSS to the peak systolic strain significantly increased the model power in detecting stenosis, and the integrated analysis improved diagnostic accuracy compared with the peak systolic strain alone. In contrast, this incremental value was not demonstrated in the multivessel group. The integrated analysis of the peak systolic strain, ESL, and PSS may allow better screening of single-vessel LAD stenosis using nonstress speckle-tracking echocardiography.

Defining the reference range for right ventricular systolic strain by echocardiography in healthy subjects: A meta-analysis

Background Right ventricular (RV) systolic strain has recently demonstrated prognostic value in various cardiovascular diseases. Despite this, the reference range including the lower limit of normal (LLN) and factors associated with RV strain measurements are not well-established. This meta-analysis aimed to determine the mean and LLN of two- (2D) and three-dimensional (3D) right ventricular global (RVGLS), free wall (RVFWLS) and interventricular septal wall (IVSLS) longitudinal strains in healthy individuals and factors that affect strain measurements. Methods In this meta-analysis, Pubmed, Embase and Cochrane databases were searched until 31 July 2020 for eligible studies reporting RVGLS, RVFWLS and/or IVSLS in at least 30 healthy subjects. We pooled the means and LLNs of RV strains by two- (2D) and three- (3D) dimensional echocardiography, and performed meta-regression analyses. Results From 788 articles screened, 45 eligible studies totaling 4439 healthy subjects were eligible for analysis. Pooled means and LLNs with 95% confidence intervals for 2D- RV strains were RVGLS -23.4% (-24.2%, -22.6%) and -16.4% (-17.3%, -15.5%) in 27 studies; RVFWLS -26.9% (-28.0%, -25.9%) and -18.0% (-19.2%, -16.9%) in 32 studies; and IVSLS –20.4% (-22.0%, -18.9%) and -11.5% (-13.6%, -9.6%) in 10 studies, and similar results for 3D- RV strains. Right ventricular fractional area change and vendor software were associated with 2D-RVGLS and RVFWLS means and LLNs. Conclusion We reported the pooled means and LLNs of RV systolic strains in healthy subjects, to define thresholds for abnormal, borderline and normal strains. Important factors associated with RV systolic strains include right ventricular fractional area change and vendor software.

Left ventricular dyssynchrony in long-term childhood cancer survivors treated with anthracyclines: a retrospective cross-sectional study

The purpose of this study was to investigate left ventricular contraction patterns in asymptomatic Childhood cancer survivors (CCS) using two-dimensional speckle tracking echocardiography (2DSTE). Left ventricular longitudinal and circumferential myocardial parameters were assessed using 2DSTE, in asymptomatic CCS and age matched healthy controls. Time to peak (T2P) systolic strain was quantified. Dyssynchrony index (DI) was measured by calculating the standard deviation of T2P systolic strain of six segments in each view. Difference between T2P systolic longitudinal strain of septal and lateral wall was also assessed as a parameter for dyssynchrony. We included 115 CCS with a median age of 17.2 years (range 5.6–39.5) and a median follow up of 11.3 years (range 4.9–29.5) and 119 controls. Conventional echocardiographic parameters and global longitudinal strain were significantly decreased in CCS compared to controls (p < 0.01 and p = 0.02, respectively). Dyssynchrony index did not differ between CCS and controls. There was a clinically insignificant smaller absolute difference between T2P systolic longitudinal of septal and lateral wall in CCS compared to controls. We showed no difference in longitudinal or circumferential left ventricular dyssynchrony in CCS compared to controls using 2DSTE. Future research should focus on assessing dyssynchrony in more segments and a larger CCS population, using both 2D and 3DSTE.

Effect of Active Cancer on the Cardiac Phenotype: A Cardiac Magnetic Resonance Imaging‐Based Study of Myocardial Tissue Health and Deformation in Patients With Chemotherapy‐Naïve Cancer

Background The overlap between cancer and cardiovascular care continues to expand, with intersections emerging before, during, and following cancer therapies. To date, emphasis has been placed on how cancer therapeutics influence downstream cardiac health. However, whether active malignancy itself influences chamber volumes, function, or overall myocardial tissue health remains uncertain. We sought to perform a comprehensive cardiovascular magnetic resonance‐based evaluation of cardiac health in patients with chemotherapy‐naïve cancer with comparison with a healthy volunteer population. Methods and Results Three‐hundred and eighty‐one patients with active breast cancer or lymphoma before cardiotoxic chemotherapy exposure were recruited in addition to 102 healthy volunteers. Both cohorts underwent standardized cardiovascular magnetic resonance imaging with quantification of chamber volumes, ejection fraction, and native myocardial T1. Left ventricular mechanics were incrementally assessed using three‐dimensional myocardial deformation analysis, providing global longitudinal, circumferential, radial, and principal peak‐systolic strain amplitude and systolic strain rate. The mean age of patients with cancer was 53.8±13.4 years; 79% being women. Despite similar left ventricular ejection fraction, patients with cancer showed smaller chambers, increased strain amplitude, and systolic strain rate in both conventional and principal directions, and elevated native T1 versus sex‐matched healthy volunteers. Adjusting for age, sex, hypertension, and diabetes mellitus, the presence of cancer remained associated with these cardiovascular magnetic resonance parameters. Conclusions The presence of cancer is independently associated with alterations in cardiac chamber size, function, and objective markers of tissue health. Dedicated research is warranted to elucidate pathophysiologic mechanisms underlying these findings and to explore their relevance to the management of patients with cancer referred for cardiotoxic therapies.