Preserved Left Ventricular Function Research Articles

Right ventricular function of patients with multiple myeloma treated with carfilzomib

Abstract Carfilzomib is an irreversible proteasome inhibitor used for treatment of multiple myeloma which improves progression-free survival and reduces the risk of death. Several adverse effects are described and especially cardiovascular events. Pathophysiological mechanisms are not fully understood but oxidative stress may damage cardiomyocytes (1). Most common cardiovascular side effects are arterial hypertension, left heart failure, arrhythmia and thromboembolic events. Therefore, transthoracic echocardiogram (TTE) is recommended prior to the initiation of treatment and throughout the follow-up. Carfilzomib has also been shown to be associated with the development of pulmonary hypertension, however the effect on right ventricular function remains unknown (2). We aimed to evaluate the evolution of right ventricular (RV) function and RV-pulmonary artery coupling under carfilzomib. This retrospective study included thirty-six patients diagnosed with multiple myeloma with a median age of 65.5 [58.8 – 71.5] years. The main comorbidity was systemic hypertension (n=18, 50%) and twenty-eight (77.8%) patients received at least one line of chemotherapy before carfilzomib. Every patient underwent TTE before and during treatment with carfilzomib. Our results showed that the baseline TTE revealed preserved left ventricular (LV) function with a median LV ejection fraction of 60 [58.8 – 63.5] % without LV dilatation. Concerning RV function, tricuspid annular plane systolic excursion (TAPSE) was 24.5 [21.1 – 27.3] mm, and lateral tricuspid annulus peak systolic velocity (S’ wave) was 13.0 [11.7 – 15.0] cm/s. Systolic peak tricuspid regurgitation velocity (peak TRV) was measured at 2.4 [2.2 – 2.6] m/s. RV-pulmonary artery coupling, estimated by TAPSE/sPAP ratio was 0.94 [0.79 – 1.11] mm/mmHg. During the follow up, TAPSE significantly decreased under carfilzomib treatment at 21.5 [19.9 – 25.3] (p=0.03). TAPSE decreased in more than 57.1% of patients with a mean of 5.4 ± 4.1 mm. However, peak TRV was not different during follow-up (2.4 [2.3 – 2.5]] m/s, p=0.97) nor TAPSE/sPAP ratio (0.88 [0.80 – 1.10] mm/mmHg, p=0.17). These data are the first to suggest that carfilzomib may impair right ventricular (RV) function even in the absence of evident pulmonary hypertension. Therefore, close monitoring of RV function in patients treated with carfilzomib is crucial due to the risk of RV side effects. Transthoracic echocardiography (TTE), particularly the follow-up of tricuspid annular plane systolic excursion (TAPSE), seems to be a valuable screening tool for monitoring these patients before they undergo more specific evaluations.

The Interplay between Hyperaldosteronism and Heart Failure with Preserved Ejection Fraction: Pathophysiological Insights and Therapeutic Implications

Heart failure with preserved ejection fraction (HFpEF) is a complex syndrome characterized by preserved left ventricular systolic function, diastolic dysfunction, and systemic inflammation, often coexisting with metabolic and hemodynamic disorders. Primary hyperaldosteronism (PHA), a condition marked by excessive aldosterone secretion, is increasingly recognized as a pivotal contributor to the pathophysiological mechanisms underpinning HFpEF. This review explores the intricate relationship between hyperaldosteronism and HFpEF, emphasizing the role of aldosterone in myocardial remodeling, endothelial dysfunction, and systemic inflammation. Additionally, it highlights emerging evidence on aldosterone antagonists as potential therapeutic agents in mitigating HFpEF-related morbidity. By integrating clinical findings with molecular insights, we aim to elucidate how hyperaldosteronism exacerbates HFpEF phenotypes and propose strategies for targeted management. This synthesis underscores the need for tailored interventions in patients with concomitant hyperaldosteronism and HFpEF.

Predictive Value of Right Ventricular Sensing During Cardiac Device Implantation to Diagnose CardiacAmyloidosis.

Cardiac amyloidosis (CA) is an underdiagnosed disease, often presenting with electrical conduction abnormalities or ventricular arrhythmias, prompting implantation of cardiac implantable electrical devices (CIEDs). This study sought to evaluate the performance of right ventricular sensing (RVSens) during implantation of CIEDs in patients presenting with increased left ventricular (LV) wall thicknesses (ILVWT) and preserved LV function to predict the diagnosis of CA. All consecutive patients with ILVWT (maximal LV wall thickness≥12mm), preserved LV function and no significant aortic stenosis who underwent implantation of CIEDs at 2 French referral centers for rare and inheritable cardiac diseases were retrospectively evaluated. Clinical history, imaging, biological data, histologic data, and pacing lead parameters at the time of implantation were collected. Diagnostic categorization of ILVWT (CA, sarcomeric hypertrophic cardiomyopathy, or hypertensive heart disease) was determined according to guidelines. We included 218 patients with ILVWT, of which 150 had CA and 68 had sarcomeric hypertrophic cardiomyopathy or hypertensive heart disease. Ten patients (4.6%) had their device implanted before ILVWT diagnosis, 131 (60.1%) had their device implanted during the same hospitalization, and 77 (35.3%) in a subsequent hospitalization. An RVSens≤11mV was independently associated with CA diagnosis with a sensitivity of 84% and a specificity of 79.4% (AUC: 0.878). In patients with ILVWT, a relatively low RVSens (≤11mV) during device implantation was independently associated with CA diagnosis, prompting further evaluation to rule out CA if the diagnosis is uncertain.

ANMCO Statement: semaglutide in cardio-nephro-metabolic disorders

Semaglutide, a glucagon-like peptide-1 receptor agonist, has emerged as a pivotal therapeutic agent in the management of the cardio-renal-metabolic continuum. Initially developed for glycemic control in type 2 diabetes mellitus, its benefits extend far beyond glucose regulation. Clinical trials have demonstrated semaglutide's potential to reduce major adverse cardiovascular events, particularly in overweight/obese patients with high cardiovascular risk, as well as improving functional capacity in patients suffering from heart failure with preserved left ventricular function. Additionally, it has shown promise in improving renal outcomes, such as slowing the progression of albuminuria and reducing the risk of chronic kidney disease in diabetic populations. These effects are likely due to its multifaceted mechanisms, including anti-inflammatory properties, weight reduction, blood pressure lowering, and direct renal protection. This review synthesizes current evidence on semaglutide's role in the interrelated domains of cardiovascular, renal, and metabolic health.

Low vitamin D levels are associated with impaired diastolic function in patients with acute coronary syndrome.

Vitamin D deficiency is a common clinical picture associated with poor cardiovascular prognosis. It is also associated with impaired diastolic dysfunction in stable coronary artery disease. We investigated the relationship between vitamin D levels and diastolic dysfunction in patients with acute coronary syndrome (ACS). One hundred seventeen patients with the ACS were enrolled in this cross-sectional study. The diastolic function of the patients was evaluated using echocardiography before discharge. We analyzed the relation between serum levels of 25(OH)D and echocardiographic measures of diastolic dysfunction. IVRT and E/e' were found to be significantly greater in patients with low vitamin D levels (115.48±13.64 vs. 106.88±14.80 ms, P<0.05; 10.19±2.80 vs. 8.1±3.2, P<0.05). When regression tests were performed, it was shown that vitamin D level was a predictor (OR=0.935, 95% CI: 0.886-0.987; P=0.015) and independent risk factor (OR=0.942, 95% CI: 0.888-0.998; P=0.042) for the development of diastolic dysfunction. We found that low vitamin D levels are associated with impaired diastolic function in patients with ACS with preserved left ventricular systolic function.

Qualitative and quantitative assessment of atrial functional mitral regurgitation: analysis from the REVEAL-AFMR registry.

The prevalence, characteristics, and prognosis of atrial functional mitral regurgitation (AFMR) based on severity remain unclear. No studies have systematically evaluated quantitative thresholds, such as effective regurgitant orifice area (EROA) or regurgitant volume, in relation to outcomes in AFMR. This multicentre study aimed to clarify the clinical implications of both qualitative and quantitative assessments of AFMR severity. In this first multicentre study across 26 centres, patients with at least moderate AFMR-defined by preserved left ventricular (LV) function, enlarged left atrium (LA), and absence of primary mitral valve changes-were retrospectively analysed. AFMR severity was evaluated using a comprehensive approach, including EROA, regurgitant volume, and regurgitant fraction. Among the 1007 patients, 728 (72.3%) had moderate, 146 (14.5%) moderate-to-severe, and 133 (13.2%) severe AFMR. Age, sex, natriuretic peptide levels, and LV ejection fraction were similar across all groups. Patients with severe AFMR had longer atrial fibrillation history, worse heart failure symptoms, larger LV and LA, and more severe tricuspid regurgitation. AFMR severity was independently associated with a higher risk of death, heart failure hospitalization, and mitral valve intervention (hazard ratio 1.51, P = 0.001 for moderate-to-severe, 2.80, P < 0.001 for severe). Quantitative thresholds showed a significantly higher event risk with EROA ≥ 0.30, regurgitant volume ≥ 60 mL, and regurgitant fraction ≥ 50%. Severe AFMR was common and linked to greater atrial fibrillation burden, cardiac structural issues, and an increased risk of adverse clinical events. Quantitative thresholds offer valuable guidance for clinical decision-making and treatment planning.

Surgery for a giant atherosclerotic left main trifurcation saccular coronary artery aneurysm.

Aneurysms of the left main coronary artery are rare clinical entities with an incidence of 0.1%. We herein report a case of left main trifurcation aneurysm in a 57-year-old woman whose coronary angiography demonstrated a giant saccular aneurysm of trifurcation of the left main coronary artery, measuring 32 × 21mm with tight post-aneurysmal stenosis in the intermediate artery. The patient underwent coronary artery bypass grafting following the resection of CAA and reconstruction with saphenous vein roof-plasty, which ensured the preservation of the native coronary vasculature. At the 6-month follow-up, she remained asymptomatic and preserved preoperative left ventricular function.

Prevalence and prognostic significance of restriction versus systolic dysfunction in patients with transthyretin and light chain cardiac amyloidosis

Abstract The tenet of cardiac amyloidosis (CA) as a paradigm of heart failure with restrictive ventricular physiology and preserved systolic function has come under scrutiny. Thus, we aimed to evaluate the prevalence and clinical significance of left ventricular (LV) systolic dysfunction versus restriction in a large real-world cohort with CA, assessed at the time of diagnosis. Methods This is a multicenter retrospective analysis including 540 transthyretin (ATTR)-CA and 280 light chain (AL)-CA. Patients were divided into 3 phenotypes: 1) preserved LV function: LV ejection fraction (EF) ≥50% associated with grade 0/I diastolic dysfunction; 2) restriction: LV-EF≥50% associated with grade II/III diastolic dysfunction; 3) systolic dysfunction: LV-EF&amp;lt;50% irrespective of diastolic function. We analysed progression from preserved LV function towards the other two phenotypes and their survival. Results In ATTR-CA, prevalence of preserved LV function was 27%, restriction was 44% and systolic dysfunction was 28%. Among patients with preserved LV function, annual conversion rate to restriction was 24% and to systolic dysfunction was 17%. At 3 years, survival free from all-cause mortality was 88%, 76% and 63% respectively (figure A). In AL-CA, prevalence of preserved LV function was 30%, restriction was 49% and systolic dysfunction was 20%. Among patients with preserved LV function, annual conversion rate to restriction was 28% and to systolic dysfunction was 8%. At 3 years, survival free from all-cause mortality was 56%, 39% and 29% respectively (figure B). Conclusions Restriction was the presenting phenotype in less than half of patients, while systolic dysfunction in approximately one quarter. The annual rate of progression form preserved LV function towards restriction or systolic dysfunction was high. In ATTR systolic dysfunction was associated with worse outcome, compared to restriction; in AL both restriction and systolic dysfunction were associated with equally ominous outcome.

The prognostic impact of inducible ischemia in severe ischaemic cardiomyopathy: a randomised comparison of medical therapy and revascularisation

Abstract Background The presence of inducible myocardial ischaemia is used to select patients with stable coronary artery disease for revascularisation, based on observational data in patients with preserved left ventricular function. It is unclear if identification of a substrate for ischaemia is prognostic in patients with severe ischaemic left ventricular systolic dysfunction (LVSD). Purpose To determine if the extent of inducible ischaemia by stress perfusion CMR relates to clinical outcomes and/or the response to revascularisation in patients with ischaemic LVSD. Methods Patients enrolled in the REVIVED-BCIS2 randomised trial were eligible for inclusion if they had undergone stress perfusion CMR prior to randomisation (1). Visual assessment of myocardial perfusion and late gadolinium enhancement (LGE) was performed by an independent core laboratory, by consensus of two expert readers who were blinded to trial-group assignment and clinical or outcome data. For this analysis, segmental ischaemia was classified in a binary manner and the overall volume of ischaemia is expressed in relation to total LV mass, defined as [number of ischaemic segments]/16 * 100. The primary composite outcome was all-cause death or aborted sudden death. Secondary outcomes included left ventricular ejection fraction change at 6 and 12-months, cardiovascular death and sustained ventricular arrhythmias. Data are presented as median [IQR] or mean ± standard deviation. Results Of 700 participants enrolled, stress perfusion CMR was acquired in 200, 181 of whom had scans of sufficient quality for core laboratory analysis (91 randomised to percutaneous coronary intervention and 90 to optimal medical therapy; age 69±8 years, 86% male, 40% diabetic, baseline jeopardy score 10 [8-12]). On CMR, LVEF was 28±8%, scar burden was 18% [9-26] and ischaemic burden was 31% [13-56]. There was no association between ischaemic burden and the primary outcome (HR 1.0, p=0.997, CI 0.92-1.09) or any of the secondary outcomes (Figure 1). There was no relationship between ischaemia and randomised treatment group (Figure 2). Conclusion The REVIVED-BCIS2 trial incudes the largest cohort to date of stress perfusion CMR in severe LVSD, randomised to relief of ischaemia or medical therapy alone. In this study, ischaemic burden was not associated with clinical outcomes or the outcome following revascularisation.Figure 1 – HRs for 10% ischaemic burdenFigure 2 - KM plots for primary outcome

Elevated mitral valve pressure gradient following transcatheter edge-to-edge repair is independently associated with inferior outcomes in atrial, but not in ventricular secondary mitral regurgitation

Abstract Background In transcatheter mitral valve edge-to-edge repair (M-TEER), the goal of optimizing post-procedural residual mitral regurgitation inadvertently carries the risk of generating relevant mitral stenosis, resulting in an elevated mean mitral valve pressure gradient (MPG). In patients with primary mitral regurgitation, an elevated post-procedural MPG has been found to independently predict impaired outcomes. However, the effect of elevated post-procedural MPG on outcome after M-TEER in patients with secondary mitral regurgitation (SMR) has yet to be fully elucidated. This study investigated the impact of elevated MPG on outcome in the EuroSMR registry, a large, multi-center real-world cohort of patients with SMR. Methods This retrospective study included patients who underwent M-TEER for SMR between 2009 and 2021 in 12 centers. Patients were stratified into two groups based on SMR etiology: (1) ventricular SMR (vSMR), and (2) atrial SMR (aSMR). The etiology was considered as vSMR in patients with a left ventricular ejection fraction (LV-EF) &amp;lt;50% and aSMR in subjects with preserved left ventricular function (LV-EF ≥50%) and a markedly dilated left atrium (Left Atrial Volume Index [LAVi] ≥40ml/m2). According to the proposed M-VARC threshold, post-procedural MPG was assessed by discharge transthoracic echocardiography and was considered elevated if &amp;gt;5 mmHg. The primary endpoint was all-cause mortality after 5 years, with statistical analysis conducted using Kaplan-Meier and uni- and multivariable Cox Regression analysis. Results Among the 1703 patients in the study (vSMR: n=1420; aSMR: n=283), elevated post-procedural MPG was observed in 9.4% (n=133) of vSMR and 19.1% (n=54) of aSMR patients. Atrial fibrillation was significantly more prevalent in aSMR (78.4% vs. 57.1%, p&amp;lt;0.001). Patients with vSMR had a higher median surgical risk assessed by the EuroScore II (aSMR vs. vSMR: 7.0% [IQR 4.0, 13.1] vs. 4.9% [IQR 2.9, 8.5], p&amp;lt;0.001). Kaplan-Meier analyses for all-cause mortality after 5 years demonstrated significant differences in aSMR patients (low vs. elevated MPG: 48.9% vs. 69.8%, log-rank p&amp;lt;0.01), while there was no difference in vSMR patients (55.7% vs. 60.8%, log-rank p=0.66) (Figure 1). This observed effect remained significant even after accounting for common confounding factors: elevated MPG was an independent predictor for all-cause mortality in aSMR patients (HR=1.86; 95%-CI: 1.14-3.02, p=0.013) but not in vSMR patients (HR=1.00; 95%-CI: 0.74-1.35, p=0.99). Conclusion Our findings suggest that elevated post-procedural MPG is an independent predictor of adverse outcomes in patients with aSMR, but not in those with vSMR. This effect may partially be explained by a reduced left atrial function and higher rates of atrial fibrillation.Figure 1.Study flowchart

Clinical outcomes and impact of mitral valve surgery in atrial functional mitral regurgitation: the REVEAL-AFMR registry

Abstract Background The real-world impact and effective treatment strategies of atrial functional mitral regurgitation (AFMR) are poorly understood. The aim of this study was to investigate the prevalence, clinical characteristics, and the effectiveness of mitral valve surgery in AFMR. Methods The REal-world obserVational study for invEstigAting the prevaLence and therapeutic options for Atrial Functional Mitral Regurgitation (REVEAL-AFMR) was a retrospective cohort study conducted across 26 Japanese centers. All transthoracic echocardiography performed in 2019 were reviewed and patients with moderate or severe AFMR defined by preserved left ventricular function, dilated left atrium, and absence of degenerative valvular changes. The primary composite outcomes were heart failure hospitalization and all-cause mortality. Besides traditional multivariable regression analysis, propensity score-based time-zero matching was performed to account for immortal time bias. Results In 177,235 patients who underwent echocardiography, 8,867 had moderate or severe MR. Within this group, 1,007 were diagnosed with AFMR (mean age 78±10, 56% female, 80% atrial fibrillation). Of these, 113 underwent MV surgery, with 81% receiving concurrent tricuspid valve surgery. Patients who underwent surgery were younger but had more severe MR, larger left heart chambers, and a higher prevalence of heart failure than those who remained under medical therapy. During a median follow-up of 1050 days, 28% experienced the primary outcome, and 18% died. Despite a more severe disease status, only the surgical group showed a decrease in natriuretic peptide levels at follow-up and had significantly lower rate of the primary outcome (hazard ratio 0.43 [0.29-0.64], p&amp;lt;0.001 after adjustment for 25 covariates). Propensity score-based time-zero matching also indicated a lower risk of the primary outcome in the surgical group (hazard ratio 0.39 [0.20–0.78], p=0.007). Multiple sensitivity analyses yielded consistent results. Subgroup analysis suggested a significantly greater benefit of surgery for patients with severe MR than those with non-severe MR (p for interaction=0.044). Conclusions Our registry revealed that patients with AFMR are typically old with predominantly with atrial fibrillation, facing poor outcomes. MV surgery was associated with fewer adverse events, a finding that should be validated in future clinical trials.

Electrocardiogram Features of Left Ventricular Excessive Trabeculation with Preserved Cardiac Function in Light of Cardiac Magnetic Resonance and Genetics.

Background and Objectives: Although left ventricular excessive trabeculation (LVET) can cause heart failure, arrhythmia and thromboembolism, limited literature is available on the ECG characteristics of primary LVET with preserved left ventricular function (EF). We aimed to compare the ECG characteristics and cardiac MR (CMR) parameters of LVET individuals with preserved left ventricular EF to a control (C) group, to identify sex-specific differences, and to compare the genetic subgroups of LVET with each other and with a C population. Methods: In our study, we selected 69 LVET individuals (EF > 50%) without any comorbidities and compared them to 69 sex- and age-matched control subjects (42% females in both groups, p = 1.000; mean age LVET-vs-C: 38 ± 14 vs. 38 ± 14 years p = 0.814). We analyzed the pattern and notable parameters of the 12-lead ECG recordings. We determined the volumetric and functional parameters, as well as the muscle mass values of the left and right ventricles (LV, RV) based on the CMR recordings. Based on the genotype, three subgroups were established: pathogenic, variant of uncertain significance and benign. Results: In the LVET group, we found normal but elevated volumetric and muscle mass values and a decreased LV_EF, wider QRS, prolonged QTc, higher RV Sokolow index values and lower T wave amplitude compared to the C. When comparing MR and ECG parameters between genetic subgroups, only the QTc showed a significant difference. Over one-third of the LVET population had arrhythmic episodes and a positive family history. Conclusions: The subclinical morphological and ECG changes and the clinical background of the LVET group indicate the need for follow-up of this population, even with preserved EF.

Ramipril After Transcatheter Aortic Valve Implantation in Patients Without Reduced Ejection Fraction: The RASTAVI Randomized Clinical Trial.

Patients with aortic stenosis may continue to have an increased risk of heart failure, arrhythmias, and death after successful transcatheter aortic valve implantation. Renin-angiotensin system inhibitors may be beneficial in this setting. We aimed to explore whether ramipril improves the outcomes of patients with aortic stenosis after transcatheter aortic valve implantation. PROBE (Prospective Randomized Open, Blinded Endpoint) was a multicenter trial comparing ramipril with standard care (control) following successful transcatheter aortic valve implantation in patients with left ventricular ejection fraction >40%. The primary end point was the composite of cardiac mortality, heart failure readmission, and stroke at 1-year follow-up. Secondary end points included left ventricular remodeling and fibrosis. A total of 186 patients with median age 83 years (range 79-86), 58.1% women, and EuroSCORE-II 3.75% (range 3.08-4.97) were randomized to receive either ramipril (n=94) or standard treatment (n=92). There were no significant baseline, procedural, or in-hospital differences. The primary end point occurred in 10.6% in the ramipril group versus 12% in the control group (P=0.776), with no differences in cardiac mortality (ramipril 1.1% versus control group 2.2%, P=0.619) but lower rate of heart failure readmissions in the ramipril group (3.2% versus 10.9%, P=0.040). Cardiac magnetic resonance analysis demonstrated better remodeling in the ramipril compared with the control group, with greater reduction in end-systolic and end-diastolic left ventricular volumes, but nonsignificant differences were found in the percentage of myocardial fibrosis. Ramipril administration after transcatheter aortic valve implantation in patients with preserved left ventricular function did not meet the primary end point but was associated with a reduction in heart failure re-admissions at 1-year follow-up. URL: https://www.clinicaltrials.gov; Unique Identifier: NCT03201185.

Myocardial ischemia in patients with mild-to-moderate aortic stenosis: Interaction with cardiac remodeling and adverse events

BackgroundThe association between aortic valve stenosis (AVS) and myocardial perfusion abnormalities has been incompletely characterized. We sought to assess the predictors of myocardial ischemia in patients with mild-to-moderate AVS, and its relationship with long-term prognosis. MethodsEighty-nine patients with mild-to-moderate AVS (peak velocity between 2.6 and 4.0 m/second and aortic valve area > .6 cm2/m2), preserved left ventricular (LV) function, and either normal coronary arteries (28 patients) or non-obstructive coronary artery disease (<50% stenosis; 61 patients) were individuated. Myocardial perfusion imaging was performed using a cadmium–zinc–telluride camera, and the summed difference score (SDS) was computed. The presence of either LV hypertrophy (LVH) (LV mass index [LVMI] > 115 g/m2 [males] or 95 g/m2 [females]) or concentric LV remodeling (relative wall thickness: >.42) was determined at two-dimensional echocardiography. ResultsForty (45%) and 49 (55%) patients had mild and moderate AVS, respectively. Fifty (56%), 17 (19%), and 22 (25%) patients had normal LV geometry, concentric LV remodeling, and LVH, respectively. An interaction between LV remodeling and inducible ischemia was revealed with progressively higher values of SDS in patients with normal LV geometry (3 ± 3), concentric remodeling (4 ± 2), and LVH (7 ± 2) (P < .001).Accordingly, a moderate correlation existed between LVMI and SDS values (R: .67; P < .001). After a median follow-up of 84 ± 47 months, 27 adverse events were recorded, including 19 AV replacements and 8 deaths. On multivariable analysis, the presence of LVH (hazard ratio [HR]: 6.46; 95% confidence interval [CI]: 2.09–20.00; P = .001) and a higher SDS (HR: 1.41; 95% CI: 1.15–1.75; P = .001) were the two independent predictors of AE. ConclusionsIn patients with mild-to-moderate AVS, myocardial ischemia correlates with the severity of adverse LV remodeling. Patients with LVH and ischemia are at increased risk of AE.

Liver stiffness as a prognostic parameter and tool for risk stratification in advanced cardiac transthyretin amyloidosis.

In light of increasing therapeutic options, risk stratification of advanced cardiac transthyretin amyloidosis (ATTR-CA) is gaining clinical importance to avoid ineffective treatments. Liver stiffness as a marker of hypervolemia and hepatic congestion might predict mortality in advanced ATTR-CA and allow to identify patients at highest risk. Proven ATTR-CA patients underwent repeated vibration-controlled transient elastography (VTCE) and standardized serial workup within the local amyloidosis cohort study AmyKoS. Spearman correlation analyses and Cox regressions were performed to evaluate the prognostic value. 41 patients with ATTR-CA were included with median age of 76.6 (55.1-89.1) years, of which 90.2% were male and > 92% wild-type ATTR-CA. In total, 85 VCTE examinations were performed. Median follow-up was 43.7 (2.4-75.6) months; 26.8% of the patients died. At the first clinical evaluation, median left ventricular (LV) absolute global longitudinal strain (GLS) was 11.4 (5.2-19.0) % and median liver stiffness was 6.3 (2.4-22.9) kPa, both significantly correlated with mortality. NT-proBNP possessed statistically significant predictive power in ATTR-CA with more preserved LV function (absolute GLS ≥ 10), whereas stiffness seemed to be more discriminative for ATTR-CA with absolute GLS < 10. The use of alternative congestion surrogates such as liver vein dilation and tricuspid regurgitation peak velocity (tr-vmax) showed congruent results. Liver stiffness shows prognostic value regarding all-cause mortality and allows risk stratification in advanced ATTR-CA, particularly in those with markedly impaired longitudinal LV function. These results are transferable to other congestion surrogates.

Clinical Outcomes of Mitral Valve Surgery in Atrial Functional Mitral Regurgitation in the REVEAL-AFMR Registry

The characteristics and treatment strategies of atrial functional mitral regurgitation (AFMR) are poorly understood. To investigate the prevalence, clinical characteristics, and outcomes of mitral valve (MV) surgery in AFMR. This retrospective cohort study, called the Real-World Observational Study for Investigating the Prevalence and Therapeutic Options for Atrial Functional Mitral Regurgitation (REVEAL-AFMR), was conducted across 26 Japanese centers (17 university hospitals, 1 national center, 3 public hospitals, and 5 private hospitals). All transthoracic echocardiography procedures performed from January 1 to December 31, 2019, were reviewed to enroll adult patients (aged ≥20 years) with moderate or severe AFMR, defined by preserved left ventricular function, a dilated left atrium, and an absence of degenerative valvular changes. Data were analyzed from May 8, 2023, to May 16, 2024. Mitral valve surgery, with or without tricuspid valve intervention. The primary composite outcome included heart failure hospitalization and all-cause mortality. In 177 235 patients who underwent echocardiography, 8867 had moderate or severe MR. Within this group, 1007 (11.4%) were diagnosed with AFMR (mean [SD] age, 77.8 [9.5] years; 55.7% female), of whom 807 (80.1%) had atrial fibrillation. Of these patients, 113 underwent MV surgery, with 92 (81.4%) receiving concurrent tricuspid valve surgery. Patients who underwent surgery were younger but had more severe MR (57.5% [n = 65] vs 9.4% [n = 84]; P < .001), a larger mean (SD) left atrial volume index (152.5 [97.8] mL/m2 vs 87.7 [53.1] mL/m2; P < .001), and a higher prevalence of heart failure (according to the New York Heart Association class III [marked limitation of physical activity] or class IV [symptoms of heart failure at rest], 26.5% [n = 30] vs 9.3% [n = 83]; P < .001) than those who remained under medical therapy. During a median follow-up of 1050 days (IQR, 741-1188 days), 286 patients (28.4%) experienced the primary outcome. Despite a more severe disease status, only the surgical group showed a decrease in natriuretic peptide levels at follow-up and had a significantly lower rate of the primary outcome (3-year event rates were 18.3% vs 33.3%; log-rank, P = .03). Statistical adjustments did not alter these findings. The findings of this cohort study suggest that in patients with AFMR, who were typically older and predominantly had atrial fibrillation, MV surgery was associated with lower rates of adverse clinical outcomes. Future studies are warranted to investigate a possible causal relationship to better regulate cardiovascular medicine.

Clinical implications of different methods to assess left atrial remodeling: A comparative study between echocardiography and cardiac magnetic resonance imaging for left atrial volume index quantification

BackgroundLeft atrial volume index (LAVI) serves as a crucial marker for assessing left atrial (LA) remodeling, particularly in patients with mitral valve regurgitation (MR). Recent guidelines recommend a LAVI exceeding 60 mL/m2 as Class IIa recommendation for mitral valve repair surgery in asymptomatic MR patients with preserved left ventricular function. Traditionally, echocardiography is the standard for assessing LAVI in MR patients. However, cardiac magnetic resonance imaging (CMR) is increasingly recognized for its more precise measurements of cardiac dimensions and volumes. But still, literature remains scarce on comparing the efficacy of both modalities in assessing LAVI measurements. MethodsThis retrospective study included 168 MR patients undergoing both echocardiography and CMR assessments within a six-month period. LAVI measurements were compared using Pearson correlation and Bland-Altman plots. Patients were stratified based on MR grades, and clinical implications were assessed. ResultsMean LAVI differed significantly between echocardiography and CMR (47.1 ± 20.8 mL/m2 versus 70 ± 20.3 mL/m2, p < 0.001, respectively). CMR consistently yielded higher LAVI measurements compared to echocardiography, with a mean difference of approximately 20 mL/m2. CMR measurements resulted in an increased incidence of patients meeting the class IIa LAVI criterion (LAVI >60 mL/m2) by 37%. Variations in LAVI did not differ across MR grades. ConclusionEchocardiography systematically underestimates LAVI compared to CMR in MR patients. While current guidelines rely on echocardiography, CMR's precision suggests the need for CMR-specific LAVI cutoff values to guide clinical management effectively. Establishing such values could refine patient stratification and timing of surgery, potentially improving clinical outcomes for MR patients.

Kawasaki disease with shock as the primary manifestation: How to distinguish from toxic shock syndrome?: A case report and literature review.

Kawasaki disease (KD) is a vasculitis syndrome of small to medium-sized arteries that has typical clinical characteristics such as fever, rash, cervical lymphadenopathy, conjunctivitis, and mucosal changes. Cardiac manifestations, including coronary artery aneurysms, myocarditis, myocardial infarction, and sudden cardiac death, are the most serious complications observed in KD. On rare occasions, it may accompanied with reduced organ perfusion due to systolic hypotension, a condition known as Kawasaki disease shock syndrome (KDSS). KDSS is a serious complication that can be presented to the emergency department as an initial feature when typical clinical symptoms of KD have not be detected. We report the case of a 12-year-old boy admitted with prolonged fever, bilateral non-purulent conjunctivitis, and signs of shock such as hypotension and tachycardia. Laboratory findings showed elevated inflammatory markers, hypoalbuminemia, and sterile pyuria. He was initially treated with intravenous cefotaxime and vancomycin considering the possible diagnosis of toxic shock syndrome, while the treatment was not effective. Subsequent chest computerized tomography and ultrasound identified pulmonary consolidation and polyserous effusion. Echocardiography revealed mild biatrial dilatation and mild valvular regurgitation with preserved left ventricular function. After a multidisciplinary consultation, a diagnosis of KDSS was made. To prevent coronary artery lesions and other severe complications, the patient immediately received immunoglobulin, corticoid, and acetylsalicylic acid. Soon afterwards, he showed significant improvement, with the temperature dropped to normal and hypotension corrected about 24 hours post-intravenous immunoglobulin therapy. Polyserous effusions also disappeared before discharge. Follow-up echocardiography revealed normal results. Clinicians should maintain a high index of suspicion for KD and consider pulmonary involvement and polyserous effusions as potential complications. For children with KD, any symptoms pointing to infection should be carefully considered. When there is no etiologic evidence, antibiotics should be used with caution. Our case also highlights the importance of considering KDSS as a differential diagnosis in children presenting with prolonged fever and shock. Early recognition, timely treatment, and close monitoring are key to preventing severe complications and ensuring favorable outcomes in patients with KDSS.

Intraoperative Assessment of Noninvasive Left Ventricular Myocardial Work Indices in Patients Undergoing Aortic Valve Replacement

ObjectivesEvaluation of noninvasive left ventricular (LV) myocardial work (MW) enables insights into cardiac contractility and efficacy beyond conventional echocardiography. However, there is limited intraoperative data on patients undergoing surgical aortic valve replacement (AVR). The aim of this study was to describe the feasibility and the intraoperative course of this technique of ventricular function assessment in these patients and compare it to conventional two- and three-dimensional echocardiographic measurements and strain analysis. DesignProspective observational study SettingAt single university hospital Participants25 patients scheduled for isolated AVR with preoperative preserved left and right ventricular function, sinus rhythm, without significant other heart valve disease or pulmonary hypertension, and an uneventful intraoperative course. InterventionsTransesophageal echocardiography (TEE) was performed after induction of anesthesia (T1), after termination of cardiopulmonary bypass (T2), and after sternal closure (T3). Evaluation was performed in stable hemodynamics, in sinus rhythm or atrial pacing and vasopressor support with norepinephrine ≤ 0.1 µg/kg/min. Measurements and Main ResultsEchoPAC v206 software (GE Vingmed Ultrasound AS, Norway) was used for analysis of 2D- and 3D LV ejection fraction (EF), LV global longitudinal strain (GLS), LV global work index (GWI), LV global constructive work (GCW), LV global wasted work (GWW), and LV global work efficiency (GWE). Estimation of myocardial work was feasible in all patients. Although there was no significant difference in the values of 2D- and 3D-EF, GWI and GCW decreased significantly after AVR [T1 v T2, 1647 ± 380 mmHg% v 1021 ± 233 mmHg%, p<0.001; T1 v T2, 2095 ± 433 mmHg% v 1402 ± 242 mmHg%, p<0.001, respectively], while GWW remained unchanged [T1 v T2, 296 mmHg% (IQR 178-452) v 309 mmHg% (IQR 255-438), p=0.97]. This resulted in a decreased GWE directly after bypass [T1 v T2, 84 ± 6 % v 78 ± 5 %, p<0.001], but GWE already improved at the end of surgery [T2 v T3, 78 ± 5 % v 81 ± 5 %, p=0.003]. There was no significant change in the values of GWI, GCW, 2D- and 3D-LVEF before and after sternal closure [T2 v T3]. ConclusionLV MW analysis showed a reduction of LV work load after bypass in our group of patients, which was not detected by conventional echocardiographic measures. This evolving technique provides deeper insights into cardiac energetics and efficiency in the perioperative course of aortic valve replacement surgery.

Impact of Anatomical and Viability-Guided Completeness of Revascularization on Clinical Outcomes in Ischemic Cardiomyopathy

BackgroundComplete revascularization of coronary artery disease has been linked to improved outcomes in patients with preserved left ventricular (LV) function. ObjectivesThis study sought to identify the impact of complete revascularization in patients with severe LV dysfunction. MethodsPatients enrolled in the REVIVED-BCIS2 (Revascularization for Ischemic Ventricular Dysfunction) trial were eligible if baseline/procedural angiograms and viability studies were available for analysis by independent core laboratories. Anatomical and viability-guided completeness of revascularization were measured by the coronary and myocardial revascularization indices (RIcoro and RImyo), respectively, where RIcoro = (change in British Cardiovascular Intervention Society Jeopardy score [BCIS-JS]) / (baseline BCIS-JS) and RImyo= (number of revascularized viable segments) / (number of viable segments supplied by diseased vessels). The percutaneous coronary intervention (PCI) group was classified as having complete or incomplete revascularization by median RIcoro and RImyo. The primary outcome was death or hospitalization for heart failure. ResultsOf 700 randomized patients, 670 were included. The baseline BCIS-JS and SYNTAX (Synergy Between PCI With Taxus and Cardiac Surgery) scores were 8 (Q1-Q3: 6-10) and 22 (Q1-Q3: 15-29), respectively. In those patients assigned to PCI, median RIcoro and RImyo values were 67% and 85%, respectively. Compared with the group assigned to optimal medical therapy alone, there was no difference in the likelihood of the primary outcome in those patients receiving complete anatomical or viability-guided revascularization (HR: 0.90; 95% CI: 0.62-1.32; and HR: 0.95; 95% CI: 0.66-1.35, respectively). A sensitivity analysis by residual SYNTAX score showed no association with outcome. ConclusionsIn patients with severe LV dysfunction, neither complete anatomical nor viability-guided revascularization was associated with improved event-free survival compared with incomplete revascularization or treatment with medical therapy alone. (Revascularization for Ischemic Ventricular Dysfunction) [REVIVED-BCIS2]; NCT01920048)