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Condition: Amyloidosis
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Total 63 results found since Jan 2013.
Characterization of Fabry Disease cardiac involvement according to longitudinal strain, cardiometabolic exercise test, and T1 mapping
AbstractIn Anderson-Fabry disease (FD), we sought to evaluate relation between left ventricular (LV) hypertrophy, longitudinal strain (LS), myocardial T1 mapping and cardiopulmonary exercise parameters, and their prognostic value in term of cardiovascular outcomes. In this prospective, observational, monocentric study called “FABRY-Image”, we evaluated consecutive adult FD patients by echocardiography, cardiac magnetic resonance, and cardiopulmonary exercise testing. We investigated regional LS, the relations between LV hypertrophy, LS, T1 mapping, and VO2 peak and VE/VCO2, and the prediction of cardiovascular event s ...
Source: The International Journal of Cardiovascular Imaging - May 7, 2020 Category: Radiology Source Type: research
Light ‐chain and transthyretin cardiac amyloidosis in severe aortic stenosis: prevalence, screening possibilities, and outcome
ConclusionBoth TTR ‐ and AL‐CA can accompany severe AS. Parameters solely based on ECG and echocardiography allow for the identification of the majority of CA‐AS. In the present cohort, CA did not significantly worsen prognosis 15.3 months after TAVR.
Source: European Journal of Heart Failure - March 4, 2020 Category: Cardiology Authors: Christian Nitsche,
Stefan Aschauer,
Andreas A. Kammerlander,
Matthias Schneider,
Thomas Poschner,
Franz Duca,
Christina Binder,
Matthias Koschutnik,
Julian Stiftinger,
Georg Goliasch,
Jolanta Siller ‐Matula,
Max‐Paul Winter,
Anahit Anvari Tags: Research Article Source Type: research
Stroke risk and atrial mechanical dysfunction in cardiac amyloidosis
We present a 42 ‐year‐old male patient with familial transthyretin amyloidosis who suffered an embolic stroke that originated from a left atrial appendage thrombus in the absence of any documented atrial fibrillation. This case highlights atrial mechanical dysfunction in patients with cardiac amyloidosis and th e need to better stratify thrombotic risk in this population with integration of echocardiographic parameters and transesophageal echocardiography.
Source: ESC Heart Failure - January 20, 2020 Category: Cardiology Authors: Brennan Ballantyne,
Usha Manian,
Olusegun Sheyin,
Ryan Davey,
Sabe De Tags: Case Report Source Type: research
Occult cardiac amyloidosis: the last chapter of a 2-year long story
AbstractAmyloidosis is characterized by deposition of insoluble fibrillar proteins in organs and tissues. The prognosis depends on where in the body amyloid deposition occurs, the amount of deposition, and the symptoms, which are often unspecific. Cardiac involvement is commonly associated with the immunoglobulin light-chain amyloidosis (AL) and may lead to sudden death. The case of a 53-year-old man ’s death due to unsuspected, undiagnosed AL predominantly involving the myocardium has been reported. His medical history had begun with gastroenterological symptoms. Angina pectoris and brain stroke occurred in the followin...
Source: Forensic Science, Medicine, and Pathology - July 30, 2019 Category: Forensic Medicine Source Type: research
Potential Applications of Remote Limb Ischemic Conditioning for Chronic Cerebral Circulation Insufficiency
Conclusion
Due to its long-term and often invisible course, CCCI has received less attention than acute cerebral ischemic stroke. However, without appropriate intervention, CCCI may lead to a variety of adverse events. Because the pathophysiological changes associated with CCCI are complex, pharmacological research in this area has been disappointing. Recent research suggests that RLIC, which is less invasive and more well-tolerated than drug treatment, can activate endogenous protective mechanisms during CCCI. In the present report, we reviewed studies related to CCCI (Table 1), as well as those related to stroke and sta...
Source: Frontiers in Neurology - May 2, 2019 Category: Neurology Source Type: research
Decreased CSF Levels of ß-Amyloid in Patients With Cortical Superficial Siderosis
Conclusions: Our results demonstrate that the presence and extent of cSS are associated with reduced CSF ß-amyloid 42 levels. Further studies are needed to investigate the underlying mechanisms of this association.
Introduction
Cerebral amyloid angiopathy (CAA)—characterized by the deposition of ß-amyloid in the walls of leptomeningeal vessels—is a common cerebral small vessel disease and a major cause of intracerebral hemorrhage in the elderly (1–3). Furthermore, it has become evident that CAA is associated with cognitive impairment (4). Specifically, it has been shown that ...
Source: Frontiers in Neurology - April 25, 2019 Category: Neurology Source Type: research
Cerebral hemorrhagic stroke associated with cerebral amyloid angiopathy in young adults about 3 decades after neurosurgeries in their infancy
Recently, there have been increasing numbers of evidence that cerebral β–amyloidosis could be transmitted between individuals [1–5]. Here, we describe 2 patients with cerebral amyloid angiopathy (CAA) associated with amyloid β protein (Aβ) (Aβ-CAA) about 3 decades after neurosurgeries, suggesting transmission of Aβ-CAA via the neurosurgeries contaminated with Aβ aggregates or induction of Aβ-CAA by the disturbance of Aβ clearance caused by neurosurgeries.
Source: Journal of the Neurological Sciences - February 1, 2019 Category: Neurology Authors: Tsuyoshi Hamaguchi, Junji Komatsu, Kenji Sakai, Moeko Noguchi-Shinohara, Satoshi Aoki, Takeshi Ikeuchi, Masahito Yamada Tags: Letter to the Editor Source Type: research
Hereditary cardiac amyloidosis associated with Pro24Ser transthyretin mutation: a case report
ConclusionsHere we report the case of a patient with hereditary cardiac amyloidosis associated with a Pro24Ser mutation in transthyretin, which is the first case reported in Japan. Technetium pyrophosphate scintigraphy was extremely useful for definitive diagnosis. Thus, we propose that the nuclear imaging technique should be taken into account even for an exploratory diagnosis of transthyretin cardiac amyloidosis.
Source: Journal of Medical Case Reports - December 16, 2018 Category: General Medicine Source Type: research
Diagnosis of Cardiac Amyloidosis: Clinical and Echocardiographic Features
AbstractPurpose of ReviewThis review is geared to address the various forms of amyloidosis which have cardiac involvement and discuss the echocardiographic findings that may be present in patients with cardiac amyloidosis.Recent FindingsThe use of echocardiography has revolutionized the diagnosis of cardiac amyloidosis. The diagnosis is often first suspected due to characteristic echocardiographic findings. Although no single finding is pathognomonic, there are a constellation of features that point to the diagnosis including increased left and right ventricular wall thickness, thickened cardiac valves and atrial walls, an...
Source: Current Cardiovascular Imaging Reports - October 23, 2018 Category: Radiology Source Type: research
Taking a deeper insight into the burden of cardiac amyloidosis: Has 3D speckle tracking echocardiographic strain come of age?
Left ventricular hypertrophy (LVH) when physiological is adaptive and beneficial, pathological hypertrophy may be maladaptive because of inability to provide a stroke volume commensurate with a normal life at rest and during physical activities/exercise. Therefore, one of the most important mandates in cardiology practice today is to differentiate between physiological and pathological hypertrophy.
Source: International Journal of Cardiology - June 11, 2018 Category: Cardiology Authors: Samir Kanti Saha, Anatoli Kiotsekoglou Tags: Editorial Source Type: research
Innovative Magnetic Resonance Imaging Markers of Hereditary Cerebral Amyloid Angiopathy at 7 Tesla Brief Report
Background and Purpose—The aim of the present study is to explore whether using 7 Tesla magnetic resonance imaging, additional brain changes can be observed in hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D) patients as compared with the established magnetic resonance imaging features of sporadic cerebral amyloid angiopathy.Methods—The local institutional review board approved this prospective cohort study. In all cases, informed consent was obtained. This prospective parallel cohort study was conducted between 2012 and 2014. We performed T2*-weighted magnetic resonance imaging performed at 7 Tesla...
Source: Stroke - May 25, 2018 Category: Neurology Authors: Emma A. Koemans, Ellis S. van Etten, Anna M. van Opstal, Gerda Labadie, Gisela M. Terwindt, Marieke J.H. Wermer, Andrew G. Webb, Edip M. Gurol, Steven M. Greenberg, Mark A. van Buchem, Jeroen van der Grond, Sanneke van Rooden Tags: Magnetic Resonance Imaging (MRI), Cerebrovascular Disease/Stroke, Intracranial Hemorrhage, Vascular Disease Brief Reports Source Type: research
Myocardial Energetics in AL and ATTR Cardiac Amyloidosis: A C-11 Acetate PET/CT Study
Conclusions: These results suggest that myocardial structure and energy utilization patterns differ between patients with AL-CMP and ATTR-CMP. Patients with AL-CMP have much lower myocardial mass and lower energy efficiency compared to patients with ATTR-CMP, suggesting better myocardial metabolic adaptation in ATTR-CMP. These findings if confirmed in a larger cohort suggest a role for novel therapeutics to manipulate substrate utilization in AL-CMP. AL-CMP(N=20)ATTR-CMP(N=3)P-ValueAge63 ± 6.380 ± 0.8<0.0001Rate Pressure Product, systolic blood pressure X heart rate, (mm Hg bpm)9228 ± 13608318 &plus...
Source: Journal of Nuclear Medicine - May 23, 2018 Category: Nuclear Medicine Authors: El-Sady, M. S., Kijewski, M., Jacob, S., Bravo, P., Dubey, S., Nodoushani, A., Belanger, A., Park, M.-A., Falk, R., Di Carli, M., Dorbala, S. Tags: Cardiac Metabolism, Viability, and Innervation Source Type: research
Failure of Tafamidis to Halt Progression of Ala36Pro TTR Oculomeningovascular Amyloidosis
Oculomeningovascular amyloidosis is a variant of transthyretin (TTR) amyloidotic polyneuropathy, which is associated with blindness and brain ischemia, microhemorrages, and siderosis due to prominent production of the abnormal TTR in the eye and in the choroid plexuses. Tafamidis is a TTR stabilizer that is orally administered and, by interfering with amyloid fibril formation and deposition, is capable of slowing progression of TTR polyneuropathy and of early-stage cardiomyopathy. However, the ocular manifestations of amyloid deposition progressed despite tafamidis therapy in Val30Met TTR amyloidosis, and the effects of ta...
Source: Journal of Stroke and Cerebrovascular Diseases - May 17, 2018 Category: Neurology Authors: Fabrizio Salvi, Roberto Volpe, Francesca Pastorelli, Andrea Bianchi, Alessandra Vella, Claudio Rapezzi, Mario Mascalchi Tags: Case Studies Source Type: research
Independent Prognostic Value of Stroke Volume Index in Patients With Immunoglobulin Light Chain Amyloidosis Cardio-Oncology
Conclusions:
SV index prognostic performance was similar to left ventricular strain in predicting survival in AL amyloidosis, independently of biomarker staging. Because SV index is routinely calculated and widely available, it could serve as the preferred echocardiographic measure to predict outcomes in AL amyloidosis patients.
Source: Circulation: Cardiovascular Imaging - May 11, 2018 Category: Radiology Authors: Milani, P., Dispenzieri, A., Scott, C. G., Gertz, M. A., Perlini, S., Mussinelli, R., Lacy, M. Q., Buadi, F. K., Kumar, S., Maurer, M. S., Merlini, G., Hayman, S. R., Leung, N., Dingli, D., Klarich, K. W., Lust, J. A., Lin, Y., Kapoor, P., Go, R. S., Pell Tags: Cardiomyopathy, Heart Failure, Echocardiography Cardio-Oncology Source Type: research
