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Transthyretin amyloidosis is a debilitating systemic disease that is often associated with symptomatic cardiac involvement. Relative apical sparing of longitudinal strain on echocardiography may be able to identify such patients. We wanted to review electrocardiogram and echocardiogram results, as well as relative apical sparing of longitudinal strain in ATTR amyloidosis patients treated non-invasively with 99m Tc-PYP imaging. From left ventricular longitudinal strain measurements, a predictive apical longitudinal strain was calculated. No ECG factors were consistently associated with the diagnosis of 99 m Tc-PYP positive patients. Patients with diabetes can be referred to indefinitely without longitudinal strain and may be able to confidently advise clinicians in triaging whom patients should consider ordering 99m Tc-PYP imaging for the noninvasive diagnosis of wild type cardiac amyloidosis. To look for ATTR cardiac amyloidosis, a patient with clinically suggestive features and a LV LS relative apical sparing ratio of 0. 8 can be considered for 99 million Tc-PYP imaging with a patient with clinically suggestive features and a 0. 8 percent relative apical sparing ratio > 0. 8.
Source link: https://doi.org/10.1007/s10554-022-02692-y
Background: Transthyretin cardiac amyloidosis is a life-threatening, debilitating disorder related to abnormal synthesis and deposit of transthyretin protein in a variety of organs, including myocardial extracellular matrix. In addition, several TTR mutations exist in particular ethnicities. Interestingly, our patient was found to have a very unusual Gly67Ala TTR mutation that is normally not present in people of Asian descent. Although costly, there are now new, emerging therapies for symptom management of cardiac amyloidosis, making early diagnosis, especially quality of life, particularly for these patients. Case summary: We outline the case of a 50-year-old Asian female who was first hospitalized for nausea and vomiting, as well as persistent orthostatic hypotension. Our goal is to outline numerous TTR mutations, existing diagnostic imaging techniques, and commercial treatments, as well as highlight the importance of early diagnosis and monitoring of cardiac amyloidosis, enabling faster diagnosis and treatment of the condition.
Source link: https://doi.org/10.3390/jcdd10010013
The purpose of Study Amyloidosis is a protein deposition disorder that causes organ dysfunction and, in some cases, death by a combination of precursor proteins. A diagnostic tool for patients with suspected amyloidosis with or without cardiomyopathy is shown to help diagnose disease and enable medical diagnosis and management. Patients with light chain disease have recently started treatments from multiple myeloma therapies, improving progression-free and overall survival as well as organ response.
Source link: https://doi.org/10.1007/s11899-021-00626-4
Transthyretin cardiac amyloidosis is a rare cardiac protein deposition disorder characterized by progressive thickening of both ventricles, the inter-atrial-ventricular septum, and the atrioventricular valves. We examined 58 patients suspected of cardiac amyloidosis based on electrocardiogram and ultrasonography results, who had been sent for bone scintigraphy in order to determine the presence of ATTR cardiac amyloidosis. However, the final diagnosis was determined by correlating the bone scan with genetic analysis, free light chain administration, or soft tissue biopsy.
Source link: https://doi.org/10.3390/diagnostics13010083
Abstract amyloid fibrils are prevalent in AL amyloid fibrils and are characterized by widespread deposition of immunoglobulin light chains. In addition to the tissue modification caused by fibrils, scientific and experimental data shows that cardiac injury can also be caused by proteotoxicity of prefibrillar amyloidogenic species. We have characterized the cellular shifts in primary human cardiac fibroblasts exposed in vitro to soluble amyloidogenic cardiotoxic LCs from AL cardiomyopathy patients. To determine the generalizability of our findings, we investigated the effect of multiple LCs on hCF's availability and on a subset of cellular proteins.
Source link: https://doi.org/10.1038/s41598-017-15424-3
The aim of this research was to determine if this abnormal myocardial deformation parameters are related to impaired LV myocardial dysfunction. Chronic LVEF patients with normal LVEF maintained longitudinal PS at apical and significantly reduced longitudinal PS at mid and basal segments, according to chronic and basal segments. The myocardial deformation was related to coronary microvascular dysfunction in AL-CA patients, according to this report, even in those with preserved LVEF; and myocardial deformation was linked to coronary microvascular dysfunction.
Source link: https://doi.org/10.1038/s41598-017-03699-5
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