How to Image Cardiac Amyloidosis: A Practical Approach.
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ABSTRACT:
SUBMITTER: Dorbala S
PROVIDER: S-EPMC7148180 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Amyloid light chain (AL) amyloidosis is among the more common and more severe of the amyloidoses usually involving the slow proliferation of a bone-marrow-residing plasma cell (PC) clone and the secretion of unstable immunoglobulin-free light chains (FLC) that infiltrate peripheral tissues and result in detrimental end-organ damage. Disease presentation is rather vague, and the hallmark of treatment is early diagnosis before irreversible end-organ damage. Once diagnosed, treatment decision is transplant-driven whereby ~20% of patients are eligible for autologous stem cell transplantation (ASCT) with or without bortezomib-based induction. In the setting of ASCT-ineligibility, bortezomib plays a central role in upfront treatment with the recent addition of daratumumab to the current emerging standard of care. In general, management of AL amyloidosis is aimed at achieving deep, durable responses with very close monitoring for early detection of relapse/refractory disease. This article provides a comprehensive review of the management of patients with AL amyloidosis including goals of therapy, current treatment guidelines in the setting of both ASCT-eligibility and ineligibility, treatment response monitoring recommendations, toxicity management, and treatment of relapse/refractory disease.
| S-EPMC8130794 | biostudies-literature
Project description:Myocardial amyloidosis (CA) differs from other etiological pathologies of left ventricular hypertrophy in that transthoracic echocardiography is challenging to assess the texture features based on human visual observation. There are few studies on myocardial texture based on echocardiography. Therefore, this paper proposes an adaptive machine learning method based on ultrasonic image texture features to identify CA. In this retrospective study, a total of 289 participants (50 cases of myocardial amyloidosis; Hypertrophic cardiomyopathy: 70 cases; Uremic cardiomyopathy: 92 cases; Hypertensive heart disease: 77 cases). We extracted the myocardial ultrasonic imaging features of these patients and screened the features, and four models of random forest (RF), support vector machine (SVM), logistic regression (LR) and gradient decision-making lifting tree (GBDT) were established to distinguish myocardial amyloidosis from other diseases. Finally, the diagnostic efficiency of the model was evaluated and compared with the traditional ultrasonic diagnostic methods. In the overall population, the four machine learning models we established could effectively distinguish CA from nonCA diseases, AUC (RF 0.77, SVM 0.81, LR 0.81, GBDT 0.71). The LR model had the best diagnostic efficiency with recall, F1-score, sensitivity and specificity of 0.21, 0.34, 0.21 and 1.0, respectively. Slightly better than the traditional ultrasonic diagnosis model. In further subgroup analysis, the myocardial amyloidosis group was compared one-by-one with the patients with hypertrophic cardiomyopathy, uremic cardiomyopathy, and hypertensive heart disease groups, and the same method was used for feature extraction and data modeling. The diagnostic efficiency of the model was further improved. Notably, in identifying of the CA group and HHD group, AUC values reached more than 0.92, accuracy reached more than 0.87, sensitivity equal to or greater than 0.81, specificity 0.91, and F1 score higher than 0.84. This novel method based on echocardiography combined with machine learning may have the potential to be used in the diagnosis of CA.
| S-EPMC10159959 | biostudies-literature
Project description:Identification of new small molecules that regulate the step-wise differentiation of hPSC into dopaminergic neurons population. Furthermore, the naturally occurring steroid, guggulsterone, was found to be the most effective inducer of neural stem cells into dopaminergic neurons.
2013-04-24 | GSE42265 | GEO
Project description:Identification of new small molecules that regulate the step-wise differentiation of hPSC into dopaminergic neurons population. Furthermore, the naturally occurring steroid, guggulsterone, was found to be the most effective inducer of neural stem cells into dopaminergic neurons. Total RNA extracted at different stages of neural differentiation from human pluripotent stem cells
2013-04-24 | E-GEOD-42265 | biostudies-arrayexpress
Project description:Cardiac amyloidosis is a form of restrictive cardiomyopathy resulting in heart failure and potential risk on arrhythmia, due to amyloid infiltration of the nerve conduction system and the myocardial tissue. The prognosis in this progressive disease is poor, probably due the development of cardiac arrhythmias. Early detection of cardiac sympathetic innervation disturbances has become of major clinical interest, because its occurrence and severity limits the choice of treatment. The use of iodine-123 labelled metaiodobenzylguanidine ([I-123]MIBG), a chemical modified analogue of norepinephrine, is well established in patients with heart failure and plays an important role in evaluation of sympathetic innervation in cardiac amyloidosis. [I-123]MIBG is stored in vesicles in the sympathetic nerve terminals and is not catabolized like norepinephrine. Decreased heart-to-mediastinum ratios on late planar images and increased wash-out rates indicate cardiac sympathetic denervation and are associated with poor prognosis. Single photon emission computed tomography provides additional information and has advantages for evaluating abnormalities in regional distribution in the myocardium. [I-123]MIBG is mainly useful in patients with hereditary and wild-type ATTR cardiac amyloidosis, not in AA and AL amyloidosis. The potential role of positron emission tomography for cardiac sympathetic innervation in amyloidosis has not yet been identified.
| S-EPMC6394628 | biostudies-literature
Project description:Cardiac involvement has a profound effect on the prognosis of patients with systemic amyloidosis. Therapeutic methods for suppressing the production of causative proteins have been developed for ATTR amyloidosis and AL amyloidosis, which show cardiac involvement, and the prognosis has been improved. However, a method for removing deposited amyloid has not been established. Methods for reducing cytotoxicity caused by amyloid deposition and amyloid precursor protein to protect cardiovascular cells are also needed. In this review, we outline the molecular mechanisms and treatments of cardiac amyloidosis.
| S-EPMC8744761 | biostudies-literature
Project description:Amyloidosis refers to a range of protein misfolding disorders that can cause organ dysfunction through progressive fibril deposition. Cardiac involvement often leads to significant morbidity and mortality and increasingly has been recognized as an important cause of heart failure. The two main forms of cardiac amyloidosis, light chain (AL) and transthyretin (ATTR) amyloidosis, have distinct mechanisms of pathogenesis. Recent insights have led to the development of novel pharmacotherapies with the potential to significantly impact each disease. This review will summarize the preclinical and clinical data for these emerging treatments for AL and ATTR amyloidosis.
| S-EPMC5832446 | biostudies-literature
Project description:AimsCardiac resynchronization therapy (CRT) is highly effective in dilated cardiomyopathy (DCM) patients with impaired left ventricular ejection fraction (LVEF) and left bundle block branch. In cardiac amyloidosis (CA) patients, left ventricular dysfunction and conduction defects are common, but the potential of CRT to improve cardiac remodelling and survival in this particular setting remains undefined. We investigated cardiovascular outcomes in CA patients after CRT implantation in terms of CRT echocardiographic response and major cardiovascular events (MACEs).Methods and resultsOur retrospective study included 47 CA patients implanted with CRT devices from January 2012 to February 2020, in nine French university hospitals (77 ± 6 years old, baseline LVEF 30 ± 8%) compared with propensity-matched (1:1 for age, LVEF at implantation, and CRT indication) DCM patients with a CRT device. CA patients had lower rates of CRT response (absolute delta LVEF ≥ 10%) compared with DCM patients (36% vs. 70%, P = 0.002). After multivariate Cox analysis, CA was independently associated with MACE (hospitalization for heart failure/cardiovascular death) [hazard ratio (HR) 3.73, 95% confidence interval (CI) 1.85-7.54, P < 0.001], along with the absence of CRT response (HR 3.01, 95% CI 1.56-5.79, P = 0.001). The presence of echocardiographic CRT response (absolute delta LVEF ≥ 10%) was the only predictive factor of MACE-free survival in CA patients (HR 0.36, 95% CI 0.15-0.86, P = 0.002).ConclusionCompared with a matched cohort of DCM patients, CA patients had a lower rate of CRT response and consequently a worse cardiovascular prognosis after CRT implantation. However, CRT could be beneficial even in CA patients given that CRT response was associated with better cardiac outcomes in this population.
| S-EPMC8787999 | biostudies-literature
Project description: Not available
| S-EPMC9731992 | biostudies-literature