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The authors report the case of a 44 year old man with a giant leiomyoma of the lower third of the esophagus. The patient presented with chest pain and the tumour was detected by echocardiography. The diagnosis was confirmed by computerised tomography and histological examination of the surgical specimen weighing 501.5 g. The surgeon performed a large esophago-gastric resection and reestablished the continuity of the digestive track by interposing a section of colon. A good result was obtained with a follow-up of 4 years. The authors underline the potential value of a simple barium swallow during cardiological assessment.

Background: Global longitudinal strain (GLS) is a useful marker for the echocardiographic evaluation of left ventricular (LV) systolic dysfunction. Presently GLS is derived from speckle tracking of LV images, but speckle tracking software is not always available. We seek to determine if manually measured GLS (MM-GLS) by assessing mid-myocardial lengths can be a reliable alternative to speckle tracking GLS (ST-GLS). Methods: Transthoracic echocardiogram images of a tertiary hospital in Australia were retrospectively analyzed to study the relationships between ST-GLS, MM-GLS, and LV ejection fraction (LVEF). We further evaluated the impact of image quality and regional wall motion abnormalities on those relationships. Results: Echocardiography studies from 154 patients were included (female sex, 36%; mean age, 61.7 ± 14.8 years). The average LVEF was 51.3% ± 11.3% and the average ST-GLS was 16.7 ± 3.8. MM-GLS strongly correlated with ST-GLS (intraclass correlation coefficient, 0.986; P < 0.001) and with LVEF regardless of the presence of regional wall motion abnormalities. If using GLS cutoff of more than 18% as normal, 97.5% of studies with normal ST-GLS had normal MM-GLS. If using GLS cutoff as less than 16% as abnormal, 95.5% of studies with abnormal ST-GLS had abnormal MM-GLS. There was no case with ST-GLS > 18% and MM-GLS < 16%, nor were there any case in with ST-GLS < 16% and MM-GLS > 18%. Conclusions: MM-GLS correlates strongly with ST-GLS. If ST-GLS cannot be accurately assessed, MM-GLS may be a useful alternative to provide GLS values in both clinical and research studies. [ABSTRACT FROM AUTHOR]

Background: Severe aortic stenosis (AS) may present with different flow, gradient and left ventricular ejection fraction (LVEF) patterns. Paradoxical low-flow low-gradient (PLF-LG) severe AS has a specific clinical profile, but its prognosis and management remain controversial. Our aim is to evaluate the impact of different AS patterns in the incidence of major clinical events. Methods: A retrospective observational study was carried out on all the consecutive patients diagnosed with severe AS at our tertiary hospital centre in 2021. Echocardiographic measurements were carefully reviewed, and patients were classified following current guidelines into four categories: high gradient (HG), concordant low-flow low-gradient (CLF-LG), paradoxical low-flow low-gradient (PLF-LG) and normal-flow low-gradient (NF-LG). The baseline characteristics and clinical events (heart failure admission, intervention and death) at 1-year follow-up were collected from medical records. The association between categories and events was established using Student's t test or ANOVA as required. Results: 205 patients with severe AS were included in the study (81 ± 10 years old, 52.7% female). Category distribution was as follows: HG (138, 67.3%), PLF-LG (34, 19.8%), CLF-LG (21, 10.2%) and NF-LG (12, 5.9%). During the follow-up, 24.8% were admitted due to heart failure, 68.3% received valve replacement (51.7% TAVR) and 22% died. Severe tricuspid regurgitation was more frequent in patients with PLF-LG than in HG AS (14.7% vs. 2.2%; p < 0.01). Despite no differences in intervention rate, more patients with PLF-LG (32.4% vs. 15.9%; p = 0.049) died during the evolution. Conclusions: The PLF-LG pattern was the second most common pattern of severe AS in our cohort, and it was related to a higher mortality with no differences in intervention rate. Thus, this controversial category, rather than being underestimated, should be followed closely and considered for early intervention. [ABSTRACT FROM AUTHOR]

Cardiac amyloidosis (CA) is a cardiac storage disease caused by the progressive extracellular deposition of misfolded proteins in the myocardium. Despite the increasing interest in this pathology, it remains an underdiagnosed condition. Non-invasive diagnostic techniques play a central role in the suspicion and detection of CA, also thanks to the continuous scientific and technological advances in these tools. The 12-lead electrocardiography is an inexpensive and reproducible test with a diagnostic accuracy that, in some cases, exceeds that of imaging techniques, as recent studies have shown. Echocardiography is the first-line imaging modality, although none of its parameters are pathognomonic. According to the 2023 ESC Guidelines, a left ventricular wall thickness ≥ 12 mm is mandatory for the suspicion of CA, making this technique crucial. Cardiac magnetic resonance provides high-resolution images associated with tissue characterization. The use of contrast and non-contrast sequences enhances the diagnostic power of this imaging modality. Nuclear imaging techniques, including bone scintigraphy and positron emission tomography, allow the detection of amyloid deposition in the heart, and their role is also central in assessing the prognosis and response to therapy. The role of computed tomography was recently evaluated by several studies, above in population affected by aortic stenosis undergoing transcatheter aortic valve replacement, with promising results. Finally, machine learning and artificial intelligence-derived algorithms are gaining ground in this scenario and provide the basis for future research. Understanding the new insights into non-invasive diagnostic techniques is critical to better diagnose and manage patients with CA and improve their survival. [ABSTRACT FROM AUTHOR]

End-stage renal disease (ESRD) is a common but profound clinical condition, and it is associated with extremely increased morbidity and mortality. ESRD can represent four major echocardiographic findings―myocardial hypertrophy, heart failure, valvular calcification, and pericardial effusion. Multiple factors interplay leading to these abnormalities, including pressure/volume overload, oxidative stress, and neurohormonal imbalances. Uremic cardiomyopathy is characterized by left ventricular (LV) hypertrophy and marked diastolic dysfunction. In ESRD patients on hemodialysis, LV geometry is changeable bidirectionally between concentric and eccentric hypertrophy, depending upon changes in corporal fluid volume and arterial pressure, which eventually results in a characteristic of LV systolic dysfunction. Speckle tracking echocardiography enabling to detect subclinical disease might help prevent future advancement to heart failure. Heart valve calcification also is common in ESRD, keeping in mind which progresses faster than expected. In a modern era, pericardial effusion observed in ESRD patients tends to result from volume overload, rather than pericarditis. In this review, we introduce and discuss those four echocardiography-assessed findings of ESRD, with which known and conceivable pathophysiologies for each are incorporated. [ABSTRACT FROM AUTHOR]

Amyloidosis is a systemic disease caused by low molecular weight protein accumulation in the extracellular space, which can lead to different degrees of damage, depending of the organ or tissue involved. The condition is defined cardiac amyloidosis (CA) when heart is affected, and it is associated with an unfavorable outcome. Different types of CA have been recognized, the most common (98%) are those associated with deposition of light chain (AL-CA), and the form secondary to transthyretin deposit. The latter can be classified into two types, a wild type (transthyretin amyloidosis wild type (ATTRwt)-CA), which mainly affects older adults, and the hereditary or variant type (ATTRh-CA or ATTRv-CA), which instead affects more often young people and is associated with genetic alterations. The atrial involvement can be isolated or linked to CA with a nonspecific clinical presentation represented by new onset atrial fibrillation (AF), diastolic dysfunction and heart failure with preserved ejection fraction, or thromboembolism and stroke. Untreated patients have a median survival rate of 9 years for AL-CA and 7 years for ATTR-CA. By contrast, AL-CA and ATTR-CA treated patients have a median survival rate of 24 and 10 years, respectively. Atrial involvement in CA is a common but poor studied event, and alterations of performance can anticipate the anatomical damage. Recently, numerous advances have been made in the diagnostic field with improvements in the available techniques. An early diagnosis therefore allows a more effective therapeutic strategy with a positive impact on prognosis and mortality rate. A multimodality approach to the diagnosis of atrial involvement from CA is therefore recommended, and standard echocardiography, advanced Doppler-echocardiography (DE) and cardiac magnetic resonance (CMR) can be useful to detect early signs of CA and to estabilish an appropriate treatment. [ABSTRACT FROM AUTHOR]

Aims: The prognosis of light-chain (AL) amyloidosis, a plasma cell dyscrasia, is largely determined by the presence of cardiac involvement. Conventional staging is achieved using cardiac biomarkers (high-sensitivity troponin, N-terminal pro-beta natriuretic peptide) and free light-chain difference (Mayo staging). We sought to evaluate the role of echocardiographic parameters as prognostic markers in AL amyloidosis and examine their utility compared with conventional staging. Methods and results: Seventy-five consecutive patients with AL amyloidosis reviewed at a referral amyloid clinic who underwent comprehensive echocardiographic assessment were retrospectively identified. The evaluated echocardiographic parameters included left ventricular (LV) ejection fraction, mass, diastolic function parameters, global longitudinal strain (GLS), and left atrial (LA) volume. Mortality was assessed through a review of clinical records. During a median follow-up of 51 months, 29/75 (39%) patients died. Patients who died had a larger LA volume (47 ± 12 vs. 35 ± 10 mL/m2, P < 0.001) and a higher E/e′ (18 ± 10 vs. 14 ± 6, P = 0.026). Univariate clinical and echocardiographic predictors of survival included LA volume, E/e′, e′, LVGLS, and Mayo stage (at significance of P < 0.1). Left atrial volume and LVGLS were significant determinants of mortality when examined using clinical cut-offs, although E/e′ was not. A composite echocardiographic risk score comprising LA volume and LVGLS provided similar prognostic performance to Mayo stage [area under the curve (AUC) 0.75, 95% confidence interval (CI) 0.64–0.85 vs. AUC 0.75, 95% CI 0.65–0.858, P = 0.91]. Conclusion: Left atrial volume and LVGLS were independent predictors of mortality in AL amyloidosis. A composite echocardiographic score combining LA volume and LVGLS has similar prognostic power to Mayo stage for all-cause mortality. Graphical Abstract An echocardiographic risk score was derived using a cohort of 74 patients with light-chain amyloidosis from a single centre. A composite score using left atrial volume and global longitudinal strain, stratified patients into three distinct risk groups. Patients were allocated one point for left atrial dilatation (indexed left atrial volume >42 mL/m2) and reduced left ventricular global longitudinal strain (>−12%). Prognostic power in predicting all-cause mortality was similar to the existing Mayo staging system [area under the curve (AUC) 0.75, 95% confidence interval (CI) 0.64–0.85 vs. AUC 0.75, 95% CI 0.65–0.858, P = 0.91]. [ABSTRACT FROM AUTHOR]

Cardiac amyloidosis is a life-threatening disease that occurs when amyloid proteins, most commonly immunoglobulin light chain or transthyretin, mutate or become unstable, misfold, deposit as amyloid fibrils, and accumulate in the myocardium. Early diagnosis of cardiac amyloidosis is hindered by insufficient awareness, specifically regarding clinical red flags and diagnostic pathways. Cardiac amyloidosis diagnosis comprises two important phases, clinical suspicion (phase one) followed by definitive diagnosis (phase two). Each phase is associated with specific clinical techniques. For example, clinical features, electrocardiography, echocardiography, and cardiac magnetic resonance imaging serve to raise suspicion of cardiac amyloidosis and facilitate early diagnosis, whereas laboratory tests (i.e., blood or urine electrophoresis with immunofixation), biopsy, scintigraphy-based nuclear imaging, and genetic testing provide a definitive diagnosis of cardiac amyloidosis. In Egypt, both the lack of cardiac amyloidosis awareness amongst healthcare providers and the unavailability of clinical expertise for the use of diagnostic techniques must be overcome to improve the prognosis of cardiac amyloidosis in the region. Previously published diagnostic algorithms for cardiac amyloidosis have amalgamated techniques that can raise clinical suspicions of cardiac amyloidosis with those that definitively diagnose cardiac amyloidosis. Though such algorithms have been successful in developed countries, diagnostic tools like echocardiography, scintigraphy, and cardiac magnetic resonance imaging are not ubiquitously available across Egyptian facilities. This review presents the current state of knowledge regarding cardiac amyloidosis in Egypt and outlines a new diagnostic algorithm which leverages regional nuclear imaging expertise. Importantly, the proposed diagnostic algorithm guides accurate amyloid-typing to mitigate misdiagnosis and erroneous treatment selection and improve the cardiac amyloidosis diagnostic accuracy in Egypt. Plain Language Summary: Diagnostic algorithms are useful tools for guiding clinical diagnosis by summarizing diagnostic approaches and defining the patient pathway. The diagnostic algorithms for cardiac amyloidosis amalgamate techniques that raise suspicion of the disease with those that can definitively diagnose the disease. These algorithms, for the early detection and diagnosis of cardiac amyloidosis, are designed in accordance with developed healthcare systems that have the resources and infrastructure for diagnostic equipment and clinical expertise. There are limited financial resources across healthcare facilities in Egypt for diagnostic equipment like echocardiograms (ECHO), scintigraphy, and cardiac magnetic resonance imaging (cMRI), and the required clinical training for the diagnosis of cardiac amyloidosis. This reduces the possibility of early diagnosis of the disease and subsequent early intervention. Evidently, there is a significant unmet clinical need to develop an algorithm for the diagnosis of cardiac amyloidosis in accordance with the Egyptian healthcare system. This review article details the current awareness regarding the diagnosis of cardiac amyloidosis and the associated challenges in Egypt. Accordingly, a diagnostic algorithm that leverages nuclear imaging expertise to guide accurate amyloid-typing in order to mitigate misdiagnosis and erroneous treatment, and also improve the diagnostic accuracy of cardiac amyloidosis, has been proposed. [ABSTRACT FROM AUTHOR]

Background: Light-chain amyloidosis is a plasma cell disorder associated with poor outcomes, especially when the heart is involved. The characteristics of left atrial (LA) function and its prognostic implications in cardiac amyloidosis (CA) have not been fully investigated.Methods: Between April 2014 and June 2019, 93 patients with a diagnosis of CA, normal left ventricular ejection fraction (LVEF) and sinus rhythm were included. Their clinical, baseline echocardiographic and follow-up data were investigated. LA function, including LA strain and strain rate, was assessed using 2D speckle tracking echocardiography in different LA functional phases.Results: Among all patients, 38 (40.9%) died. Multivariate Cox regression analyses showed that LA mechanics regarding LA reservoir and booster pump functions were independent predictors for overall survival. Traditional echocardiographic parameters for LA structure like LA volume index and LA width were not associated with mortality. Moreover, LA strain and strain rate in reservoir and contractile phases improved the discrimination and goodness of fit of the conventional prognostic model, the Mayo criteria 2004 and 2012, in our study population. Decreased LA mechanics were associated with impaired left ventricular (LV) systolic and diastolic function, and LA reservoir and contractile functions were associated with LA structure.Conclusions: Assessment of LA reservoir and contractile functions via 2D speckle tracking echocardiographic LA mechanical indices provide clinical and prognostic insights into cardiac light-chain amyloidosis patients, especially those with preserved EF and sinus rhythm. Emphasizing the monitoring of LA function may be beneficial for the prognosis prediction of CA. [ABSTRACT FROM AUTHOR]

Aims: This study was performed to investigate whether left atrial (LA) strain by echocardiography provides prognostic information in patients with wild‐type transthyretin amyloid cardiomyopathy (ATTRwt‐CM). Methods and results: Among 129 patients who were diagnosed with ATTRwt‐CM at Kumamoto University Hospital from December 2002 to December 2019, 113 patients who had enough information for two‐dimensional speckle tracking echocardiography were enrolled in this study. During a median follow‐up of 668 days, 28 cardiovascular deaths occurred. Compared with patients in the non‐event group, those in the cardiovascular death group were significantly older (81.5 ± 7.4 vs. 78.1 ± 6.1 years, P < 0.01), had a lower incidence of carpal tunnel syndrome (21% vs. 47%, P < 0.05), and had a higher high‐sensitivity cardiac troponin T [0.085 (0.063–0.105) vs. 0.049 (0.036–0.079) ng/mL, P < 0.01] and B‐type natriuretic peptide concentrations [419 (239–541) vs. 271 (155–462) pg/mL, P < 0.01] and lower estimated glomerular filtration rate (41.8 ± 15.4 vs. 53.4 ± 14.6 mL/min/1.73 m2, P < 0.01). Electrocardiography showed higher rate of a V1–V3 QS pattern (52% vs. 24%, P < 0.01) and complete left bundle branch block (27% vs. 6%, P < 0.01), and echocardiography showed a significantly lower peak LA strain rate during the contraction phase (0.16 ± 0.13 vs. 0.28 ± 0.27 S−1, P < 0.05), LA strain during the reservoir phase (LASr) (5.84 ± 2.41 vs. 8.22 ± 4.05%, P < 0.01), and peak LA strain rate during the reservoir phase (0.26 ± 0.09 vs. 0.33 ± 0.15 S−1, P < 0.05) in the cardiovascular death group than in non‐event group. By contrast, conventional echocardiographic findings were not significantly different between these two groups. After adjusting for conventional predictive factors of ATTRwt‐CM (age, high‐sensitivity cardiac troponin T and B‐type natriuretic peptide concentrations, and estimated glomerular filtration rate), multivariable Cox proportional hazard analyses showed that LASr was significantly and independently associated with cardiovascular death in patients with ATTRwt‐CM (odds ratio, 0.84; 95% confidence interval, 0.72–0.98; P < 0.05). After adjusting for age and echocardiographic findings associated with cardiovascular death (LA volume index and peak LA strain rate during the contraction phase), LASr was significantly and independently associated with cardiovascular death in patients with ATTRwt‐CM (odds ratio, 0.83; 95% confidence interval, 0.70–0.98; P < 0.05). Receiver operating characteristic curve analysis showed that the area under the curve of LASr for cardiovascular death was 0.686 and that the best cut‐off value of LASr was 6.69% (sensitivity, 62.4%; specificity, 64.3%). In the Kaplan–Meier analysis, patients with low LASr (<6.69%) had a significantly higher probability of total cardiovascular death (P < 0.05) and heart failure‐related hospitalization (P < 0.05). Conclusions: Left atrial strain during the reservoir phase provides significant prognostic value in patients with ATTRwt‐CM even after adjusting for conventional predictive factors. [ABSTRACT FROM AUTHOR]

Purpose of Review: Cardiac amyloidosis is an increasingly recognized condition with a growing range of targeted therapies, but diagnosis requires a high index of suspicion and multimodality imaging expertise. Early diagnosis remains key to improving quality of life and survival. This article reviews the multimodality imaging approach to the diagnosis, differentiation, and prognosis of cardiac amyloidosis. Recent Findings: Recent advances in multimodality cardiac imaging have allowed for earlier diagnosis of cardiac amyloidosis resulting in earlier initiation of life-saving therapy in cases of light chain amyloidosis and life-prolonging therapy in transthyretin amyloidosis. Summary: With these advances in multimodality imaging, it is important for cardiologists and cardiac imagers to be aware of the subtleties of early disease, the appropriate diagnostic approach as well as understanding the practicalities and pitfalls that are encountered with each modality. [ABSTRACT FROM AUTHOR]

Speckle tracking echocardiography is an imaging method, based on analysis of 2D echocardiographic images, that is useful in providing information on tissue deformation in different cardiac diseases. Cardiac amyloidosis is a complex pathological entity that can be confused with other causes responsible for cardiac hypertrophy, and for this reason, it can be misdiagnosed, especially in the early stages. Cardiac magnetic resonance and endomyocardial biopsy are reference methods for specific diagnosis, but their use is limited by a number of factors, both of a logistical and technical nature. Considering the limits of standard 2D echocardiography, speckle tracking echocardiography can be a useful method to enhance the clinical suspicion, to provide prognostic information, and to address patients more appropriately towards reference methods for definitive diagnosis. [ABSTRACT FROM AUTHOR]

The mechanism of reduced stroke volume index (SVi) in paradoxical low-flow, low-pressure gradient (PLFLPG) aortic stenosis (AS) remains unclarified. Guyton et al. ( 21 ) demonstrated that SVi is determined by whole body O2 consumption (Ṿo2) in many subjects, including patients with heart disease. We hypothesized that reduced SVi in PLFLPG AS is associated with reduced Ṿo2 by the whole body. This study investigated the relationship between Ṿo2, SVi, and AS severity in patients with AS to examine the association between reduced Ṿo2 and PLFLPG AS. In 59 patients (24 men and 35 women, mean age: 78 ± 7 yr old) with severe AS, SVi, AS severity, and type were evaluated by echocardiography, and Ṿo2 was measured by the fraction of O2 in expired gases. SVi and Ṿo2 were significantly decreased in 20 patients with PLFLPG AS compared with 39 patients with non-PLFLPG AS (30 ± 4 vs. 41 ± 7 ml/m2 and 2.4 ± 0.5 vs. 3.0 ± 0.5 ml·min-1·kg-1, respectively, P < 0.01). The SVi-to-Ṿo2 ratio was not different between the two groups (13.1 ± 2.6 vs. 13.6 ± 2.1, not significant). SVi was independently correlated with Ṿo2 ( r = 0.74, P < 0.01) but not with the aortic valve area index. Categorized PLFLPG AS was also significantly associated with reduced Ṿo2 ( P < 0.001). PLFLPG AS is associated with reduced Ṿo2 by the whole body, which may offer insights into the mechanism of PLFLPG AS. NEW & NOTEWORTHY Paradoxical low-flow, low-pressure gradient severe aortic stenosis (PLFLPG AS) is an important and problematic subtype, and its central pathophysiology with reduced stroke volume is yet to be clarified. We hypothesized and subsequently clarified that reduced stroke volume in PLFLPG AS is associated with reduced O2 consumption by the whole body. This study suggests important insights into the mechanism of PLFLPG AS and may further promote studies to investigate further mechanisms and novel treatment. [ABSTRACT FROM AUTHOR]

BACKGROUND: Amyloidosis is a severe systemic disorder produces by the accumulation of inappropriately amyloid deposition in tissues. Cardiac involvement, as a main type of amyloidosis, has a major impact on prognosis. We describe a biopsy-proven cardiac amyloidosis in an old man with unexpected presentation. CASE REPORT: A 70-year-old man, with a complaint of severe weakness, lightheadedness, and lower limb paresthesia, was admitted to the emergency department. Electrocardiography revealed right bundle branch block and Trifascicular block. Echocardiography study showed a moderately increased thickness of left ventricular wall with concentric pattern as well. Laboratory investigations including serum and urine electrophoresis, and serum free light chain examination as immunofixation assay revealed that κ chains predominated over λ chains in a ratio of 3:2. Our patient with final diagnosis of amyloid light-chain (AL) amyloidosis underwent chemotherapy with melphalan combined with high-dose dexamethasone, CPHPC and monoclonal antibodies for 2 weeks. CONCLUSION: It shows that rapid diagnosis of AL amyloidosis can enhance the prognosis. Applying an optimal strategy for the treatment leads to effective therapy, too. [ABSTRACT FROM AUTHOR]

The clinical management of asymptomatic patients with severe aortic valve stenosis (AS) may be challenging. Indeed, there is substantial controversy over the optimal timing of intervention for these patients, as some advocate early intervention while others urge for a conservative management until symptom onset. In the meantime of randomized clinical trials aiming to compare both strategies of management, an integrative approach including several imaging modalities as well as biomarkers of the myocardial damage may help to improve the risk stratification of patients with asymptomatic severe AS and individualize strategy of treatment. The extent of aortic valve calcification, semi-quantitatively assessed by echocardiography but better fully quantitatively measured by computed tomography (CT), provides incremental value to predict rapid disease progression and adverse event and thus could be a potential trigger for early referral to intervention or guide the timing of clinical follow-up. In addition, the assessment of disease activity using positron emission tomography, combined with CT calcium scoring, may also help to better predict the disease progression. The comprehensive assessment of left ventricular (LV) function using speckle-tracking echocardiography and/or cardiac magnetic resonance may provide useful information regarding the actual repercussion of AS on the myocardium. Besides imaging modalities, the measurement of several circulating biomarkers associated with the extent of the myocardial damage may also improve the risk stratification of asymptomatic patients with severe AS and help guiding management. Finally, it should be emphasized that a personalized medicine, including this comprehensive integrative approach in heart valve clinics, should be advocated for the management of these challenging patients. [ABSTRACT FROM AUTHOR]

An important proportion of patients with aortic stenosis (AS) have a 'low-gradient' AS, i.e. a small aortic valve area (AVA, 1.0 cm²) consistent with severe AS but a low mean transvalvular gradient (,40 mmHg) consistent with non-severe AS. The management of this subset of patients is particularly challenging because the AVA-gradient discrepancy raises uncertainty about the actual stenosis severity and thus about the indication for aortic valve replacement (AVR) if the patient has symptoms and/or left ventricular (LV) systolic dysfunction. The most frequent cause of low-gradient (LG) AS is the presence of a low LV outflow state, which may occur with reduced left ventricular ejection fraction (LVEF), i.e. classical low-flow, low-gradient (LF-LG), or preserved LVEF, i.e. paradoxical LF-LG. Furthermore, a substantial proportion of patients with AS may have a normal-flow, low-gradient (NF-LG) AS: i.e. a small AVA--low-gradient combination but with a normal flow. One of the most important clinical challenges in these three categories of patients with LG AS (classical LF-LG, paradoxical LF-LG, and NF-LG) is to differentiate a true-severe AS that generally benefits from AVR vs. a pseudo-severe AS that should be managed conservatively. A low-dose dobutamine stress echocardiography may be used for this purpose in patients with classical LF-LG AS, whereas aortic valve calcium scoring by multi-detector computed tomography is the preferred modality in those with paradoxical LF-LG or NF-LG AS. Although patients with LF-LG severe AS have worse outcomes than those with high-gradient AS following AVR, they nonetheless display an important survival benefit with this intervention. Some studies suggest that transcatheter AVR may be superior to surgical AVR in patients with LF-LG AS. [ABSTRACT FROM AUTHOR]

Background:The prognosis of amyloidosis is known to depend heavily on cardiac function and may be improved by identifying patients at highest risk for adverse cardiac events. Aims: Identify predictors of mortality in patients with cardiac light-chain amyloidosis (AL), hereditary transthyretin amyloidosis (m-TTR), or wild-type transthyretin amyloidosis (WT-TTR) to prompt physician to refer these patients to dedicated centers. Methods and results: Observational study. About 266 patients referred for suspected cardiac amyloidosis (CA) in two French university centers were included. About 198 patients had CA (AL = 118, m-TTR = 57, and WT-TTR = 23). Their median (25th–75th percentile) age, NT-proBNP left ventricular ejection fraction were, respectively, 68 years (59–76), 2339 pg mL−1(424–5974), and 60% (48–66). About 31% were in NYHA class III–IV. Interventricular septal thickness was greater in the m-TTR and WT-TTR groups than in the AL group (p < 0.0001). Median follow-up in survivor was 26 months (15–44) and 87 (44%) patients died. By multivariate analysis, independent predictors of mortality for AL amyloidosis were the following: age, cardiac output and NT-proBNP; for TTR amyloidosis was: NT-proBNP. When all amyloidosis were combined NT-proBNP, low cardiac output and pericardial effusion were independently associated with mortality. Conclusion: NT-proBNP is a strong prognosticator in the three types of cardiac amyloidosis. High NT-proBNP, low cardiac output, and pericardial effusion at the time of screening should prompt physician to refer the patients to amyloidosis referral center. [ABSTRACT FROM AUTHOR]

Background: Right ventricular (RV) dysfunction is a strong predictor of poor outcomes in heart failure. Its prognostic meaning in cardiac amyloidosis (CA) is under-investigated. Methods: Hundred and twenty nine patients with suspected CA and an interventricular septum thickness (IVST) ≥ 12 mm underwent echocardiography with measurement of left ventricular (LV) and RV longitudinal strain (LS), late gadolinium-enhancement (LGE) cardiac MRI, and standard evaluation. Results: Among 82 confirmed CA, types were immunoglobulin light chain (AL,n = 26), hereditary transthyretin (m-TTR,n = 37) and senile (WT-TTR,n = 19). Compared to those without, CA patients had significantly lower RV fractional shortening (RV-FS), tricuspid annular plane systolic excursion (TAPSE), tissue Doppler systolic velocity, and global RV-LS, without any difference among the CA types. RV-LGE, observed in 62% of CA patients, was associated with lower global and basal RV-FS. Median follow-up was 8(2; 16) months. Using multivariate analysis, NYHA-class and low TAPSE independently predicted major adverse cardiac event (MACE) defined as death, heart transplantation and acute heart failure. Independent determinants of TAPSE < 14 mm, the best cut-off value, were LV ejection fraction (LVEF), estimated filling pressure (E/E′), NT-proBNP and pulmonary artery pressure, but not RV-LGE. Conclusions: RV dysfunction is common in CA. Its routine evaluation by a simple TAPSE may be an aid in assessing the prognosis of CA patients. [ABSTRACT FROM AUTHOR]

Background: Amyloidosis is characterized by extracellular deposits of insoluble proteins that cause tissue damage. The three main types are monoclonal light chain (AL), wild-type transthyretin (wt-TTR) and mutated transthyretin (m-TTR) amyloidosis. Cardiac amyloidosis (CA) raises diagnostic challenges. Objective: To assess the diagnostic accuracy of99mTc-HMDP-scintigraphy for typing CA, differentiating CA from non-amyloid left ventricle hypertrophy (LVH), and predicting outcomes. Methods: 121 patients with suspected CA underwent99mTc-HMDP-scintigraphy in addition to standard investigations. Results: CA was diagnosed in all AL (n = 14) and wt-TTR (n = 21). Among m-TTR (n = 34), 26 had CA, 4 neuropathy without CA and 4 were asymptomatic carriers. Of the 52 patients with non-amyloid heart disease, 37 had LVH and served as controls.99mTc-HMDP cardiac uptake occurred in all wt-TTR, in m-TTR with CA except two and in one AL. A visual score ≥ 2 was 100% specific for diagnosing TTR-CA. Among TTR-CA, heart-to-skull retention (HR/SR) correlated with CA severity (LVEF and NT-proBNP). Median follow-up was 111 days (50;343). In a multivariate Cox model including clinical, echocardiographic and scintigraphic variables, NYHA III-IV and HR/SR > 1.94 predicted acute heart failure and/or death. Conclusions: This preliminary study suggests that99mTc-HMDP-scintigraphy may aid differentiation between transthyretin and AL-CA as well as CA from other LVHs.99mTc-HMDP-scintigraphy appears to provide prognostic information in CA. [ABSTRACT FROM AUTHOR]

Aims: Low gradient severe aortic stenosis (AS) with preserved left ventricular ejection fraction (LVEF) may be attributed to aortic valve area index (AVAi) underestimation due to the assumption of a circular shape of the left ventricular outflow tract (LVOT) with 2-dimensional echocardiography. The current study evaluated whether fusing Doppler and multidetector computed tomography (MDCT) data to calculate AVAi results in significant reclassification of inconsistently graded severe AS. Methods and results: In total, 191 patients with AVAi < 0.6 cm2/m2 and LVEF ≥ 50% (mean age 80±7 years, 48% male) were included in the current analysis. Patients were classified according to flow (stroke volume index <35 or ≥35 mL/m2) and gradient (mean transaortic pressure gradient ≤40 or >40 mmHg) into four groups: normal flow-high gradient (n = 72), low flow-high gradient (n = 31), normal flow-low gradient (n = 46), and low flow-low gradient (n = 42). Left ventricular outflow tract area was measured by planimetry on MDCT and combined with Doppler haemodynamics on continuity equation to obtain the fusion AVAi. The group of patients with normal flow-low gradient had significantly larger AVAi and LVOT area index compared with the other groups. Although MDCT-derived LVOT area index was comparable among the four groups, the fusion AVAi was significantly larger in the normal flow-low gradient group. By using the fusion AVAi, 52% (n = 24) of patients with normal flow-low gradient and 12% (n = 5) of patients with low flow-low gradient would have been reclassified into moderate AS due to AVAi ≥ 0.6 cm2/m2. Conclusion The fusion AVAi reclassifies 52% of normal flow-low gradient and 12% of low flow-low gradient severe AS into true moderate AS, by providing true cross-sectional LVOT area. [ABSTRACT FROM AUTHOR]

Aims We reported that patients with small aortic valve area (AVA) and low flow despite preserved left ventricular ejection fraction (LVEF), i.e. ‘paradoxical’ low flow (PLF), have worse outcomes compared with patients with normal flow (NF), although they generally have a lower mean gradient (MG). The aortic valve weight (AVW) excised at the time of valve replacement is a flow-independent marker of stenosis severity. The objective of this study was to compare the AVW of patients with PLF and MG<40 mmHg with the AVW of patients with NF and MG≥40 mmHg. Methods and results We recruited 250 consecutive patients undergoing valve replacement (Cohort A) for severe stenosis. Among them, 33 (13%) were in PLF [LVEF > 50% but stroke volume index (SVi) ≤ 35 mL/m2] with MG < 40 mmHg (PLF-LG group) and 105 (42%) were in NF (LVEF > 50% and SVi > 35 mL/m2) with MG ≥ 40 mmHg (NF-HG group). Despite a much lower MG (29 ± 7 vs. 53 ± 10 mmHg; P < 0.0001), patients in the PLF-LG group had a similar AVA (0.73 ± 0.12 vs. 0.69 ± 0.13; P = 0.19) compared with those in the NF-HG group. The AVW [median (interquartile): 1.90 (1.63–2.50) vs. 2.60 (1.66–3.32)] and prevalence of bicuspid phenotype (15 vs. 42%) were lower in the PLF-LG group than in the NF-HG group. However, AVWs analysed separately in the tricuspid and bicuspid valves were similar in both groups [tricuspid valves: 1.80 (1.63–2.50) vs. 2.30 (1.58–3.00) g; P = 0.26 and bicuspid valves: 2.72 (1.73–3.61) vs. 2.60 (2.10–3.55) g; P = 0.93]. When using cut-point values of AVW established in another series of non-consecutive patients (n = 150, Cohort B) with NF and concordant Doppler-echocardiographic findings, we found that the percentage of patients with evidence of severe stenosis in Cohort A was 70% in patients with PLF-LG and 86% in patients with NF-HG. Conclusion The aortic valve weight data reported in this study provide evidence that a large proportion of patients with PLF and low-gradient have a severe stenosis and that the gradient may substantially underestimate stenosis severity in these patients. A multi-parametric approach including all Doppler-echocardiographic parameters of valve function as well as other complementary diagnostic tests may help correctly identify these patients. [ABSTRACT FROM PUBLISHER]

Background and Purpose We have shown that infusions of apolipoprotein A- I ( ApoA- I) mimetic peptide induced regression of aortic valve stenosis ( AVS) in rabbits. This study aimed at determining the effects of ApoA- I mimetic therapy in mice with calcific or fibrotic AVS. Experimental Approach Apolipoprotein E-deficient ( ApoE−/−) mice and mice with Werner progeria gene deletion ( WrnΔhel/Δhel) received high-fat diets for 20 weeks. After developing AVS, mice were randomized to receive saline (placebo group) or ApoA- I mimetic peptide infusions ( ApoA- I treated groups, 100 mg·kg−1 for ApoE−/− mice; 50 mg·kg−1 for Wrn mice), three times per week for 4 weeks. We evaluated effects on AVS using serial echocardiograms and valve histology. Key Results Aortic valve area ( AVA) increased in both ApoE−/− and Wrn mice treated with the ApoA- I mimetic compared with placebo. Maximal sinus wall thickness was lower in ApoA- I treated ApoE−/− mice. The type I/ III collagen ratio was lower in the sinus wall of ApoA- I treated ApoE−/− mice compared with placebo. Total collagen content was reduced in aortic valves of ApoA- I treated Wrn mice. Our 3D computer model and numerical simulations confirmed that the reduction in aortic root wall thickness resulted in improved AVA. Conclusions and Implications ApoA- I mimetic treatment reduced AVS by decreasing remodelling and fibrosis of the aortic root and valve in mice. [ABSTRACT FROM AUTHOR]

Aims: To characterize patients with cardiac amyloidosis using echocardiography, electrocardiogram (ECG) and right heart catheterization (RHC). Methods and results: Fourteen patients with biopsy verified light chain or transthyretin cardiac amyloidosis were included. All patients had heart failure with markedly elevated NT-proBNP. Echocardiography demonstrated biventricular hypertrophy, left atrial enlargement and normal to slightly reduced left ventricular ejection fraction. Tissue Doppler septal e´ was low and median E/e´ was high. Within 6 months RHC was performed in eight of the patients. The restrictive filling pattern demonstrated by echocardiography corresponded well to median pulmonary wedge pressure (21 mmHg). Systolic pulmonary artery pressure (SPAP) was increased, whereas cardiac output and stroke volume were seen to be decreased with both methods. ECG demonstrated: low voltage (36%), abnormal R-progression (65%), ST-T abnormalities (71%) and high incidence of fibrillation (36%). In addition, a case report following the treatment of melphalan and dexamethasone is presented with improvement of hypertrophy, SPAP, left ventricular mass and e´. Conclusion: These findings should lead to a suspicion of cardiac amyloidosis and suggest further investigation. [ABSTRACT FROM AUTHOR]

We report the usefulness of live three-dimensional transthoracic echocardiography (3DTTE) in the morphological assessment of a left ventricular thrombus. Using live 3DTTE, the thrombus could be easily viewed end-on and from the sides. In addition, by cropping the 3D images sequentially in transverse (horizontal or short axis), longitudinal (vertical or long axis), frontal, and oblique planes, the degree and extent of lysis within the thrombus, which represents an integral part of the clot-resolution process, could be comprehensively assessed. The site of attachment of the thrombus in the left ventricular apex and its morphology could also be fully evaluated in three dimensions by live 3DTTE.(ECHOCARDIOGRAPHY, Volume 21, October 2004) [ABSTRACT FROM AUTHOR]

In three of nine children with dilated cardiomyopathy (aged 1-9 years), left ventricular thrombi were diagnosed and followed by echocardiography. Thrombi recurred in two patients, in one of them, embolized to the cerebral arteries. Resolution of the thrombi was observed under therapy directed against platelet aggregation. However, this did not prevent thrombus formation. [ABSTRACT FROM AUTHOR]

Purpose of Review: The aim is to provide a description of the most important echocardiographic features in systemic amyloidosis. Recent Findings: Amyloidosis is a heterogeneous group of multisystem disorders, characterized by an extracellular deposition of amyloid fibrils. Several imaging tests are available for the diagnosis; however, echocardiography is the cornerstone of the non-invasive imaging modality for cardiac amyloidosis. So far, little is known about the diagnosis of cardiac amyloidosis through imaging modalities. Summary: We summarized the most important echocardiographic findings in cardiac amyloidosis. Hence, we offered a systematic report of the diagnostic performance of cardiac amyloidosis using echocardiography. [ABSTRACT FROM AUTHOR]