Current and future role of echocardiography in arrhythmogenic right ventricular dysplasia/cardiomyopathy

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited progressive cardiomyopathy, clinically characterized by ventricular arrhythmias and increased risk of sudden cardiac death. Echocardiography has a role in the diagnosis and prognosis of ARVD/C. However, in the current era of magnetic resonance imaging (MRI), the role of echocardiography in ARVD/C patients and family member screening is subject to debate. Relatively novel echocardiographic techniques, such as three-dimensional right ventricular (3D-RV) imaging and tissue deformation imaging, may improve the diagnostic and prognostic performance of echocardiography in these patients. 3D-RV imaging provides more insights on RV anatomy and global function compared to conventional echocardiography. Subtle RV regional wall motion abnormalities, and mechanical dyssynchrony, are accurately measured by tissue deformation imaging. Several studies suggest an incremental value of novel echocardiographic parameters in addition to conventional measurements. Moreover, new parameters indicating subtle RV dysfunction, and mechanical dyssynchrony, are of predictive value and could help in risk stratification of ARVD/C patients. New robust parameters, derived from 3D-RV echocardiography and RV tissue deformation imaging, in combination with established conventional parameters, suggest that there is a current and future role for echocardiography in ARVD/C supplementing MRI

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Background For parents at high risk for cardiovascular events, presence of cardiovascular disease or risk factors in their offspring may be an indicator of their genetic load or exposure to (unknown) risk factors and might be related to the development of new or recurrent vascular events. Methods In 4,267 patients with vascular disease, hypertension, diabetes, or hypercholesterolemia enrolled in the SMART cohort, the presence of cardiovascular risk factors (hypertension, diabetes, hypercholesterolemia, smoking, or overweight) and cardiovascular disease (coronary artery disease, cerebrovascular disease, peripheral artery disease, or abdominal aortic aneurysm) was assessed in their 10,564 children. The relation between presence of cardiovascular disease or cardiovascular risk factors in their offspring and new or recurrent vascular events was determined by Cox proportional hazard analyses. Results Of the patients, 506 (12%) had offspring with cardiovascular disease, hypertension, hypercholesterolemia, or diabetes. Smoking in offspring was present in 1,972 patients (46%), and overweight in 845 patients (20%). During a median follow-up of 7.0 years (interquartile range 3.7-10.4), the composite outcome of myocardial infarction (MI), stroke, or vascular mortality occurred in 251 patients. Patients with offspring with cardiovascular disease, hypertension, hypercholesterolemia, or diabetes had an increased risk of vascular mortality (hazard ratio [HR] 2.9, 95% CI 1.2-7.1), MI (HR 1.6, 95% CI 1.1-2.5), and the composite outcome (HR 1.5, 95% CI 1.1-2.2). Diabetes in offspring was related to an increased risk of the composite outcome (HR 2.7, 95% CI 1.5-5.0), MI (HR 3.3, 95% CI 1.7-6.6), and vascular mortality (HR 3.4, 95% CI 0.8-14.8). Smoking and overweight in offspring were not related to increased vascular risk in parents. Conclusions Presence of cardiovascular disease, hypertension, hypercholesterolemia, and diabetes in offspring, with diabetes mellitus being the most contributing cardiovascular risk factor, is related to an increased risk of developing new or subsequent vascular events in patients already at high vascular risk

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OBJECTIVE Our aim is to compare the effect of type 2 diabetes on recurrent major cardiovascular events (MCVE) for patients with symptomatic vascular disease at different locations. RESEARCH DESIGN AND METHODS A total of 6,841 patients from the single-center, prospective Second Manifestations of ARTerial disease (SMART) cohort study from Utrecht, the Netherlands, with clinically manifest vascular disease with (n = 1,155) and without (n = 5,686) type 2 diabetes were monitored between 1996 and 2013. The effect of type 2 diabetes on recurrent MCVE was analyzed with Cox proportional hazards models, stratified for disease location (cerebrovascular disease, peripheral artery disease, abdominal aortic aneurysm, coronary artery disease, or polyvascular disease, defined as >= 2 vascular locations). RESULTS Five-year risks for recurrent MCVE were 9% in cerebrovascular disease, 9% in peripheral artery disease, 20% in those with an abdominal aortic aneurysm, 7% in coronary artery disease, and 21% in polyvascular disease. Type 2 diabetes increased the risk of recurrent MCVE in coronary artery disease (hazard ratio [HR] 1.67; 95% CI 1.25-2.21) and seemed to increase the risk in cerebrovascular disease (HR 1.36; 95% CI 0.90-2.07), while being no risk factor in polyvascular disease (HR 1.12; 95% CI 0.83-1.50). Results for patients with peripheral artery disease (HR 1.42; 95% CI 0.79-2.56) or an abdominal aortic aneurysm (HR 0.93; 95% CI 0.23-3.68) were inconclusive. CONCLUSIONS Type 2 diabetes increased the risk of recurrent MCVE in patients with coronary artery disease, but there is no convincing evidence that it is a major risk factor for subsequent MCVE in all patients with symptomatic vascular disease

Optimal echocardiographic assessment of myocardial dysfunction for arrhythmic risk stratification in phospholamban mutation carriers

AIMS: Phospholamban (PLN) p.Arg14del mutation carriers are at risk of developing malignant ventricular arrhythmias (VAs) and/or heart failure. Currently, left ventricular ejection fraction (LVEF) plays an important role in risk assessment for VA in these individuals. We aimed to study the incremental prognostic value of left ventricular mechanical dispersion (LVMD) by echocardiographic deformation imaging for prediction of sustained VA in PLN p.Arg14del mutation carriers. METHODS AND RESULTS: We included 243 PLN p.Arg14del mutation carriers, which were classified into three groups according to the '45/45' rule: (i) normal left ventricular (LV) function, defined as preserved LVEF ≥45% with normal LVMD ≤45 ms (n = 139), (ii) mechanical LV dysfunction, defined as preserved LVEF ≥45% with abnormal LVMD >45 ms (n = 63), and (iii) overt LV dysfunction, defined as reduced LVEF <45% (n = 41). During a median follow-up of 3.3 (interquartile range 1.8-6.0) years, sustained VA occurred in 35 individuals. The negative predictive value of having normal LV function at baseline was 99% [95% confidence interval (CI): 92-100%] for developing sustained VA. The positive predictive value of mechanical LV dysfunction was 20% (95% CI: 15-27%). Mechanical LV dysfunction was an independent predictor of sustained VA in multivariable analysis [hazard ratio adjusted for VA history: 20.48 (95% CI: 2.57-162.84)]. CONCLUSION: LVMD has incremental prognostic value on top of LVEF in PLN p.Arg14del mutation carriers, particularly in those with preserved LVEF. The '45/45' rule is a practical approach to echocardiographic risk stratification in this challenging group of patients. This approach may also have added value in other diseases where LVEF deterioration is a relative late marker of myocardial dysfunction

Is quantitative analysis superior to visual analysis of planar thallium 201 myocardial exercise scintigraphy in the evaluation of coronary artery disease? - Analysis of a prospective clinical study

Quantitative analysis of myocardial exercise scintigraphy has been previously reported to be superior to visual image interpretation for detection of the presence and extent of coronary artery disease. Computer analysis of perfusion defects and washout rate of thallium 201 was performed on scintigrams from a group of 131 consecutive patients (prospective group), using criteria defined from a previous group of 72 patients (initial group), and compared with visual interpretation of scintigrams for detection and evaluation of coronary artery disease. The sensitivity of the quantitative technique with regard to overall detection of coronary artery disease was not significantly different from the visual method (69% and 74%, respectively), whereas the specificity was higher (86% and 68%). Quantitative analysis did not increase the sensitivity of thallium imaging over the visual method in the left anterior descending artery (46% vs 65%) and the right coronary artery (51% vs 72%) but did increase sensitivity in the left circumflex artery (75% vs 47%). Whereas in the initial group quantitative analysis resulted in a better identification of multivessel disease (sensitivity 81 % vs 57%), in the prospective group sensitivity decreased (54% vs 67%) without significant loss of specificity. The initial group had a 40% incidence of three-vessel disease and the prospective group, 22% (P < 0.05). One-vessel disease was higher in the prospective group (32% vs 11%, P < 0.05). Thus, assessing the quantitative technique in a larger prospective patient population, there was no improvement of detection of the presence and extent of coronary artery disease when compared with visual interpretation

Screen-detected abnormal ankle brachial index: A risk indicator for future cardiovascular morbidity and mortality in patients with manifest cardiovascular disease

Objectives The ankle brachial index (ABI) can be used to diagnose peripheral arterial disease (PAD). The clinical relevance of the ABI, especially in patients with known clinically manifest cardiovascular disease (CVD), is unknown. The authors set out to investigate the relationship between a screen-detected ABI and the risk for future cardiovascular morbidity and mortality in patients with clinically manifest CVD. Design, materials and methods Patients with clinically manifest CVD were selected from the UCC-SMART cohort (n = 8360) and divided into four groups: normal ABI (0.91–1.39), screen-detected low ABI ≤ 0.9, screen-detected high ABI ≥ 1.4, and patients with known PAD irrespective of their ABI. Adjusted Cox Proportional Hazard Ratios (HRs) for Major Adverse Cardiovascular Events (MACE), Major Adverse Limb Events (MALE), and all-cause mortality were calculated. In addition, stratified analyses for women and men and for the presence of diabetes were performed. Results During a median follow-up of 8.3 years (IQR 7.7) 1646 MACE, 601 MALE and 1958 all-cause mortalities were observed. Compared with normal ABI patients, patients with a screen-detected low ABI and patients with manifest PAD had a higher risk of MACE, MALE, and all-cause mortality with HRs of 1.9 (95% CI 1.6–2.2) for MACE, 7.6 (95% CI 5.7–10.1) for MALE, 1.7 (95% CI 1.5–2.0) for mortality and 1.3 (95% CI 1.2–1.5) for MACE, 13.8 (95% CI 11.1–17.1) for MALE, 1.7 (95% CI 1.5–1.9) for mortality, respectively. Screen-detected high ABI did not increase the risk of either MACE or MALE, however, was associated with lower risk of all-cause mortality with a HR of 0.6 (95% CI 0.5–0.9). Stratified analyses for women & men and for diabetes status were comparable for all three outcomes. Conclusions In patients with manifest CVD but without PAD, a screen-detected low ABI is a powerful risk indicator for cardiovascular events, limb events, and all-cause mortality

Both male and female obese ZSF1 rats develop cardiac dysfunction in obesity-induced heart failure with preserved ejection fraction

Heart failure with a preserved ejection fraction (HFpEF) is associated with multiple comorbidities, such as old age, hypertension, type 2 diabetes and obesity and is more prevalent in females. Although the male obese ZSF1 rat has been proposed as a suitable model to study the development of diastolic dysfunction and early HFpEF, studies in female animals have not been performed yet. Therefore, we aimed to characterize the cardiac phenotype in female obese ZSF1 rats and their lean counterparts. Additionally, we aimed to investigate whether differences exist in disease progression in obese male and female ZSF1 rats. Therefore, male and female ZSF1 rats, lean as well as obese (N = 6-9/subgroup), were used. Every two weeks, from 12 to 26 weeks of age, systolic blood pressure and echocardiographic measurements were performed, and venous blood was sampled. Female obese ZSF1 rats, as compared to female lean ZSF1 rats, developed diastolic dysfunction with cardiac hypertrophy and fibrosis in the presence of severe dyslipidemia, increased plasma growth differentiation factor 15 and mild hypertension, and preservation of systolic function. Although obese female ZSF1 rats did not develop hyperglycemia, their diastolic dysfunction was as severe as in the obese males. Taken together, the results from the present study suggest that the female obese ZSF1 rat is a relevant animal model for HFpEF with multiple comorbidities, suitable for investigating novel therapeutic interventions

Reduction of blood culture contamination rate by an educational intervention

Background: Although mechanical dyssynchrony parameters derived by speckle tracking echocardiography (STE) may predict response to cardiac resynchronization therapy (CRT), comparability of parameters derived with different STE vendors is unknown. Methods: In the MARC study, echocardiographic images of heart failure patients obtained before CRT implantation were prospectively analysed with vendor specific STE software (GE EchoPac and Philips QLAB) and vendor-independent software (TomTec 2DCPA). Response was defined as change in left ventricular (LV) end-systolic volume between examination before and six-months after CRT implantation. Basic longitudinal strain and mechanical dyssynchrony parameters (septal to lateral wall delay (SL-delay), septal systolic rebound stretch (SRSsept), and systolic stretch index (SSI)) were obtained from either separate septal and lateral walls, or total LV apical four chamber. Septal strain patterns were categorized in three types. The coefficient of variation and intra-class correlation coefficient (ICC) were analysed. Dyssynchrony parameters were associated with CRT response using univariate regression analysis and C-statistics. Results: Two-hundred eleven patients were analysed. GE-cohort (n = 123): age 68 years (interquartile range (IQR): 61-73), 67% male, QRS-duration 177ms (IQR: 160-192), LV ejection fraction: 26 +/- 7%. Philips-cohort (n = 88): age 67 years (IQR: 59-74), 60% male, QRS-duration: 179 ms (IQR: 166-193), LV ejection fraction: 27 +/- 8. LV derived peak strain was comparable in the GE-(GE: -7.3 +/- 3.1%, TomTec: -6.4 +/- 2.8%, ICC: 0.723) and Philips-cohort (Philips: -7.7 +/- 2.7%, TomTec: -7.7 +/- 3.3%, ICC: 0.749). SL-delay showed low ICC values (GE vs. TomTec: 0.078 and Philips vs. TomTec: 0.025). ICC's of SRSsept and SSI were higher but only weak (GE vs. TomTec: SRSsept: 0.470, SSI: 0.467) (Philips vs. QLAB: SRSsept: 0.419, SSI: 0.421). Comparability of septal strain patterns was low (Cohen's kappa, GE vs. TomTec: 0.221 and Philips vs. TomTec: 0.279). Septal strain patterns, SRSsept and SSI were associated with changes in LV end-systolic volume for all vendors. SRSsept and SSI had relative varying C-statistic values (range: 0.530-0.705) and different cut-off values between vendors. Conclusions: Although global longitudinal strain analysis showed fair comparability, assessment of dyssynchrony parameters was vendor specific and not applicable outside the context of the implemented platform. While the standardization taskforce took an important step for global peak strain, further standardization of STE is still warranted

Global Left Ventricular Myocardial Work Efficiency in Patients With Severe Rheumatic Mitral Stenosis and Preserved Left Ventricular Ejection Fraction

BACKGROUND: Assessment of left ventricular (LV) function plays a pivotal role in the management of patients with valvular heart disease, including those caused by rheumatic heart disease. Noninvasive LV pressure-strain loop analysis is emerging as a new echocardiographic method to evaluate global LV systolic function, integrating longitudinal strain by speckle-tracking analysis and noninvasively measured blood pressure to estimate myocardial work. The aim of this study was to characterize global LV myocardial work efficiency in patients with severe rheumatic mitral stenosis (MS) with preserved ejection fraction (EF). METHODS: We retrospectively included adult patients with severe rheumatic MS with preserved EF (> 50%) and sinus rhythm. Healthy individuals without structural heart disease were included as a control group. Global LV myocardial work efficiency was estimated with a proprietary algorithm from speckle-tracking strain analyses, as well as noninvasive blood pressure measurements. RESULTS: A total of 45 individuals with isolated severe rheumatic MS with sinus rhythm and 45 healthy individuals were included. In healthy individuals without structural heart disease, the mean global LV myocardial work efficiency was 96% (standard deviation [SD], 2), Compared with healthy individuals, median global LV myocardial work efficiency was significantly worse in MS patients (89%; SD, 4; p < 0.001) although the LVEF was similar. CONCLUSIONS: Individuals with isolated severe rheumatic MS and preserved EF, had global LV myocardial work efficiencies lower than normal controls

Left Ventricle Myocardial Work Correlated with Functional Capacity in Severe Rheumatic Mitral Stenosis with Preserved Left Ventricular Ejection Fraction

Background and Aims: Functional capacity is reduced in mitral stenosis (MS) patients. Previous studies showed a correlation between left atrial strain and functional capacity in this population. However, currently, no left ventricle (LV) echocardiographic parameters were associated with functional capacity in patients with MS. Noninvasive LV pressure-strain loop analysis is a new echocardiographic method for evaluating LV function, integrating longitudinal strain from speckle-tracking analysis and noninvasively measured blood pressure to estimate myocardial work (MW) that overcomes the preload-dependent characteristics conventional parameters by integrating afterload. This study aimed to evaluate the association between MW and functional capacity measured using exercise tests in patients with severe MS and preserved LV ejection fraction (LVEF). Methods: Adult patients with symptomatic severe rheumatic MS (mitral valve area 50%) and sinus rhythm who underwent echocardiography and exercise stress test in our hospital from 2019 to 2021 were included. Exclusion criteria were suboptimal image quality for myocardial deformation analysis, significant mitral regurgitation or aortic valve lesions, coronary artery disease, intracardiac shunt, and atrial fibrillation. Standard echocardiographic parameters were measured, and all MW parameters were included. Exercise treadmill testing was performed using the modified Bruce protocol. Results: A total of 33 individuals with isolated severe rheumatic MS in sinus rhythm (age 39.8 ± 9.8 years) were included in the study. Patients with severe isolated MS showed significantly impaired LV-global longitudinal strain values compared to normal reference values. Furthermore, patients with severe MS showed significantly lower values of global work index, global constructive work, and efficiency compared to normal values and higher wasted work. Global work efficiency was significantly correlated to the duration of exercise (P = 0.025, Pearson’s r = 0.389). Conclusions: In stable patients with isolated severe mitral stenosis, MW efficiency significantly correlated with functional capacity measured objectively through exercise testing