Cardiac Amyloidosis and its New Clinical Phenotype: Heart Failure with Preserved Ejection Fraction.

Existing Reviews

Please note, new claims can take a short while to show up.

No claims yet.

Annotation Summary

Term Occurence Count Dictionary
amyloidosis 69 endocrinologydiseases
bortezomib 1 endocrinologydiseasesdrugs
cholic acid 1 endocrinologydiseasesdrugs
cyclophosphamide 1 endocrinologydiseasesdrugs
dexamethasone 2 endocrinologydiseasesdrugs
melphalan 2 endocrinologydiseasesdrugs
obesity 2 endocrinologydiseases

Graph of close proximity drug and disease terms (within 200 characters).

Note: If this graph is empty, then there are no terms that meet the proximity constraint.

Review

Having read the paper, please pick a pair of statements from the paper to indicate that a drug and disease are related.

Select Drug Character Offset Drug Term Instance
bortezomib 26288 Clinic group, in the case of three patients initially ineligible for stem cell therapy, the use of bortezomib made the procedure possible. Stem cell transplantation is used in 25% of patients with cardiac amyloidosis.
cholic acid 28582 function were observed, as well as biomarkers.[32],[55],[56]The use of doxycycline and tauroursodeoxy cholic acid was carried out with a small number of patients, and a possible clinical improvement was identified.
cyclophosphamide 26062 who took MelDex.[32],[46]-[48]Bortezomib has been shown to be an effective drug when combined with cyclophosphamide and dexamethasone, with a significant hematological response (71%) after two months of use. According
dexamethasone 26083 took MelDex.[32],[46]-[48]Bortezomib has been shown to be an effective drug when combined with cyclophosphamide and dexamethasone , with a significant hematological response (71%) after two months of use. According to Mayo Clinic group,
dexamethasone 26693 stem cell therapy is approximately 56%.[16],[19],[27],[32],[33]A study evaluating the use of bortezomid, dexamethasone and alkylating agents (BDEX + AA) in 106 patients with symptomatic HF due to AL cardiac amyloidosis showed
melphalan 25616 months, confirming a better prognosis in these cases.[19],[21],[31],[33],[34]Dexamethasone-associated melphalan therapy (MelDex) in patients ineligible for autologous stem cell transplantation had a response rate
melphalan 25873 advanced cardiac involvement. In a recent randomized clinical trial comparing MelDex with high doses of melphalan followed by stem cell transplantation showed a better survival rate in patients who took MelDex.[32],[46]-[48]Bortezomib
Select Disease Character Offset Disease Term Instance
amyloidosis 812 insoluble fibrils originating from proteins that exhibit anomalous folding. Recently, pictures of senile amyloidosis have been described in patients with HFpEF, demonstrating the need for clinical cardiologists to investigate
amyloidosis 991 for clinical cardiologists to investigate this etiology in suspect cases. The clinical suspicion of amyloidosis should be increased in cases of HFPS where the cardio imaging methods are compatible with infiltrative
amyloidosis 1407 management of the diagnosed patients can be done in partnership with centers specialized in the study of amyloidosis , which, together with the new technologies, investigate the possibility of organ or bone marrow transplantation
amyloidosis 2092 as hypertension, diabetes, obesity, and chronic kidney disease.[1]-[6] Recently, a picture of senile amyloidosis has been described in patients with HFpEF, demonstrating the need for clinical cardiologists to investigate
amyloidosis 3127 determines numerous dysfunctions of irreversible, progressive and indolent course, as observed in cardiac amyloidosis .[7],[10],[16]-[23]Cardiac involvement may lead to the development of a restrictive HF model. Deposits in
amyloidosis 3461 is the main cause of the reduction of survival of these patients.[7],[10],[16]-[23]The diagnosis of amyloidosis presents important non-invasive advances in characterizing its presence and its type. In the past, the
amyloidosis 4284 survival and quality of life.[24],[25]According to data obtained in MedLine, publications on the cardiac amyloidosis framework date back to 1948, totaling over 1000 articles indexed in several languages. Over the last
amyloidosis 5043 disease provides sufficient evidence for physicians to increase their clinical suspicion of cardiac amyloidosis , especially of the senile type, in cases of HFpEF, and referral of these patients to specialized centers
amyloidosis 5450 this present review we will discuss the recent advances in the etiology and pathophysiology of cardiac amyloidosis , especially the senile form, in a systematized way for the evaluation of individuals suspected of amyloidosis
amyloidosis 5560 amyloidosis, especially the senile form, in a systematized way for the evaluation of individuals suspected of amyloidosis in the context of HFpEF and the emerging therapies currently available in clinical practice.Classification
amyloidosis 5703 emerging therapies currently available in clinical practice.Classification and etiopathogenesis of amyloidosis In the face of the complexity associated with the disease and its multiplicity of presentations, specific
amyloidosis 5980 to the predisposing condition and to the type of amyloid fibril deposited in the tissue. In general, amyloidosis can be classified as primary, secondary, related to dialysis and associated with transthyretin.[27]-[30]It
amyloidosis 6124 primary, secondary, related to dialysis and associated with transthyretin.[27]-[30]It is classified as primary amyloidosis (AL) when it is defined by the production of amyloid protein composed of light chain immunoglobulins
amyloidosis 7976 presence of a compromised liver and a possible acquired von Willebrand's disease.[27],[32]-[34]Secondary amyloidosis (AA) is identified in chronic inflammatory clinical conditions such as rheumatoid arthritis, psoriasis
amyloidosis 8929 affected by AA. However, there are also reports of cardiac involvement.[27],[35]-[37]Dialysis-related amyloidosis occurs as a function of the deposition of fibrils originating from beta-2 microglobulin proteins, which
amyloidosis 9316 is osteoarticular involvement, such as carpal tunnel syndrome and rotator cuff involvement.[38],[39]Cardiac amyloidosis associated with transthyretin (TTR) is the second form of amyloidosis with a higher prevalence of cardiac
amyloidosis 9386 cuff involvement.[38],[39]Cardiac amyloidosis associated with transthyretin (TTR) is the second form of amyloidosis with a higher prevalence of cardiac involvement, and may be divided into hereditary and senile forms.
amyloidosis 10151 patients.[22] Data from the Mayo Clinic group indicate that the prevalence of this form among patients with amyloidosis is approximately 8,5%, with a mean age of 77 years and the male sex representing 82% of the affected
amyloidosis 10867 than 70 mutations associated with this protein have been identified. In view of the suspicion of an amyloidosis by TTR, the sequencing of this protein from tissue or blood sample should be performed for the diagnosis
amyloidosis 12263 cardiac dysfunction with increased filling pressures, pulmonary edema is an infrequent condition in amyloidosis cardiomyopathy.[7],[8],[10],[14]A rarer phenotype is the involvement of the interventricular septum
amyloidosis 12779 genetic origin.[21],[24],[41]The report of syncope, due to autonomic nervous system involvement by amyloidosis , is a common finding in these patients and their presence in relation to physical exertion is associated
amyloidosis 13286 episodes of hypoperfusion. Diseases of the conduction system may be present in the different forms of amyloidosis . However, they are more frequently found in the form associated with TTR, both senile and hereditary.
amyloidosis 13433 found in the form associated with TTR, both senile and hereditary. Syncope in patients with cardiac amyloidosis is mainly associated with hypotension due to dysautonomia and bradyarrhythmias and less related to ventricular
amyloidosis 13669 Malignant ventricular arrhythmia, when present, is a common cause of death in patients with cardiac amyloidosis , and these patients are strong candidates for implantation of cardio-defibrillators.[24]Involvement
amyloidosis 13954 pericardial effusion which, most of the time, does not develop cardiac tamponade. Due to the alterations of cardiac amyloidosis itself, this condition may be masked and not have echocardiographic signs such as atrial and right ventricular compression.[24],[41]The
amyloidosis 14332 the risk of intracavitary thrombus formation. This process is evident, especially in patients with AL amyloidosis type and is an independent factor of atrial fibrillation, and when both factors are present, the risk
amyloidosis 14580 high. Therefore, the use of anticoagulants should be considered in these individuals.[24],[41]Cardiac amyloidosis and its new clinical phenotypeA new insight into HFpEF becomes critical, given its increasing relevance
amyloidosis 15062 study, among the population with HF, 59% had the HFpEF phenotype.[5]Data from a specialized center in amyloidosis in Brazil point to a high prevalence of myocardial involvement in patients with amyloid polyneuropathy from
amyloidosis 15631 worth highlighting the possibility that a portion of the patients with HFpEF actually present a cardiac amyloidosis . This is confirmed by recent studies with patients with a diagnosis of HFpEF, in the absence of arterial
amyloidosis 16066 patients with HF.[7],[8],[15],[45]In order to facilitate the identification of a suspected cardiac amyloidosis , regardless of the type of fibril deposited, we must consider some clinical evidence and complementary
amyloidosis 16295 presented in Table 1.[7]Table 1Clinical criteria and complementary tests in the investigation of cardiac amyloidosis CategoriesCriteriaHistoryAge of onset of HFpEF > 60 yearsFamily history of unexplained HF at age 60Peripheral
amyloidosis 17184 this scenario where the most common etiologies applicable to the HFpEF are not confirmed, a cardiac amyloidosis scenario should be suspected. Therefore, we propose a guideline flow chart for the management of these
amyloidosis 17412 (FIGURES 2 and 3).[18],[22]Figure 2Flowchart for the evaluation of patients with HFpEF and suspicion of amyloidosis . HfpEF: heart failure with preserved ejection fraction; Echo: doppler echocardiography; ECG: electrocardiogram;
amyloidosis 17631 electrocardiogram; DPD: 2,3-dicarboxypropane-1,1-diphosphonate.Figure 3Flowchart for investigation of the type of amyloidosis . TTR: transthyretin.Diagnostic approachCardiac amyloidosis presents an indolent course and the diagnosis
amyloidosis 17690 3Flowchart for investigation of the type of amyloidosis. TTR: transthyretin.Diagnostic approachCardiac amyloidosis presents an indolent course and the diagnosis is made, often late, thus contributing to a worse prognosis.
amyloidosis 18052 conditions where there is the characterization of restrictive cardiopathy.[7]In clinical history, cardiac amyloidosis may be suspected in patients 50 years of age or older who have signs and symptoms of HF, with an ejection
amyloidosis 18422 manifestations in the patient's history should be considered as guiding principles for the diagnosis of HF by amyloidosis , such as peripheral neuropathy and carpal tunnel syndrome, especially recurrent and bilateral. Signs
amyloidosis 18851 present.[7],[29]The ECG is an easily accessible examination that may offer changes that raise the suspicion of amyloidosis in the presence of a CHF. The finding of a low voltage QRS complex, electrical axis deviations and branch
amyloidosis 19339 left ventricular hypertrophy associated with ECG with low-voltage QRS may lead to suspicion of cardiac amyloidosis . Another criterion for suspicion of cardiac amyloidosis is thickening of the left ventricular wall above
amyloidosis 19395 low-voltage QRS may lead to suspicion of cardiac amyloidosis. Another criterion for suspicion of cardiac amyloidosis is thickening of the left ventricular wall above 12 mm in the absence of a history of systemic arterial
amyloidosis 19826 cardiomyopathy.[9],[14],[22]Measurement of the thickness of the interventricular septum may suggest the type of amyloidosis present in the patient, and is often greater in cases of amyloidosis by TTR than the AL form, and may
amyloidosis 19895 septum may suggest the type of amyloidosis present in the patient, and is often greater in cases of amyloidosis by TTR than the AL form, and may in many cases be greater than 20 mm. However, the separation between
amyloidosis 20111 these two forms on a clinical basis is not always possible.[9],[14],[22]In some patients with cardiac amyloidosis , we can observe the clinical phenotype similar to obstructive hypertrophic cardiomyopathy due to the
amyloidosis 20475 longitudinal systolic strain has been used for the diagnosis of systolic dysfunction in patients with cardiac amyloidosis , and may show the preservation of the heart tip in relation to other walls. In the presence of ECG and
amyloidosis 20704 alterations we should resort to complementary tests. In a first step in the elucidation of a case of cardiac amyloidosis , we investigated the renal function status of this patient through the nitrogenous excoriations and,
amyloidosis 21064 protein electrophoresis. This stage in the investigation allows the identification of the primary form of amyloidosis and consists in the identification of the light chains that are in high titers in these patients. Immunofixation,
amyloidosis 21712 abdominal fat aspirate, right ventricular endomyocardial biopsy may be essential for the diagnosis of cardiac amyloidosis . Through this method, the amyloid protein is stained by congo-red. Other tissues can also be evaluated
amyloidosis 22169 prognosis.[14],[22],[29]Magnetic resonance imaging appears as another alternative for the diagnosis of cardiac amyloidosis , with a sensitivity of 87% and specificity of 96% for the form associated with TTR. Through this examination,
amyloidosis 22802 the light chain derivatives, being an effective method of evaluating the mutant or wild forms of cardiac amyloidosis associated with TTR. Positron emission tomography in conjunction with the C-RiB plotter may be a new
amyloidosis 23106 immunohistochemical staining or flow cytometry analysis is critical in patients in whom the type of AL- amyloidosis has been identified. This will demonstrate a clonal population of plasma cells, which are producing defective
amyloidosis 23448 disease.[29],[33]There are new studies involving omic sciences that aim to increase the diagnostic accuracy of amyloidosis . Proteomics involves the study of all the protein expression of a cell in different conditions, being
amyloidosis 23907 are dissected and decomposed into smaller components called peptides.[25],[28]Treatment of cardiac amyloidosis Treatment of cardiac amyloidosis is best performed in specialized centers of the disease. Treatment requires
amyloidosis 23939 into smaller components called peptides.[25],[28]Treatment of cardiac amyloidosisTreatment of cardiac amyloidosis is best performed in specialized centers of the disease. Treatment requires two approaches: control
amyloidosis 24203 deposition and treatment of the underlying disease to prevent new amyloid formations.Treatment of cardiac amyloidosis aims to improve the signs and symptoms of HF. The use of low-dose diuretics improves symptoms related
amyloidosis 24456 combination of beta-blockers and angiotensin-converting enzyme inhibitors has its undefined benefit in amyloidosis .[16],[18],[21],[22],[24]The use of digitalis has no benefits in this group of patients, since the myocardium
amyloidosis 25004 survival is approximately four years after diagnosis and has improved over the past three decades. AL- amyloidosis is often the result of a clonal increase of plasma cells in the bone marrow and thus therapy with cytotoxic chemotherapeutics
amyloidosis 26394 bortezomib made the procedure possible. Stem cell transplantation is used in 25% of patients with cardiac amyloidosis . Following the procedure episodes of supraventricular tachycardia may occur, with mortality of 11% in
amyloidosis 26795 dexamethasone and alkylating agents (BDEX + AA) in 106 patients with symptomatic HF due to AL cardiac amyloidosis showed an improvement in survival after adjustment of clinical variables. (Hr: 0.209, 95% CI: 0.069
amyloidosis 27023 0.006).[32],[49]Form associated with TTRTafamidis appears as an important option for the treatment of amyloidosis , acting as a kinetic stabilizer of the TTR tetramer. The interaction of molecules at certain TTR binding
amyloidosis 27579 double-blind, placebo-controlled study, the safety and efficacy of oral Tafamidis in patients with amyloidosis and involvement of the peripheral nervous system were demonstrated. Clinical trials show that this medication
amyloidosis 28067 stabilization of transthyretin in 97% of patients with mild to moderate HF due to the wild type of cardiac amyloidosis .[7],[22],[24],[32],[50]-[54]Another drug in the evaluation process for the cardiac amyloidosis associated with
amyloidosis 28162 cardiac amyloidosis.[7],[22],[24],[32],[50]-[54]Another drug in the evaluation process for the cardiac amyloidosis associated with TTR is Diflunisal, a non-steroidal anti-inflammatory that can stabilize the tetramer,
amyloidosis 28386 amyloidogenesis. One cohort evaluated the tolerance and effects promoted in 13 patients with cardiac amyloidosis by TTR, both mutant and wild type. No significant changes in cardiac structure and function were observed,
amyloidosis 28979 28 studies are enrolled in the Clinical Trials database for interventions in patients with cardiac amyloidosis .[32],[57],[58]An alternative treatment for some types of amyloidosis would be liver transplantation
amyloidosis 29048 for interventions in patients with cardiac amyloidosis.[32],[57],[58]An alternative treatment for some types of amyloidosis would be liver transplantation with the goal of replacing the mutated TTR gene that produces the majority
amyloidosis 29695 amyloid protein and thus does not delay the progression of the disease.[22],[24],[32]ConclusionCardiac amyloidosis inaugurates a new era of personalized cardiology, where precise diagnosis through techniques involving
amyloidosis 29916 molecular genetic analysis, biomarkers and cardioimaging methods make it possible to classify the form of amyloidosis and define its clinical course and prognosis and, in the future, guide the therapeutics of these frames.The
amyloidosis 30058 and prognosis and, in the future, guide the therapeutics of these frames.The clinical suspicion of amyloidosis should be increased in cases of HFPSE in which the methods of cardiac imaging are compatible with the
amyloidosis 30299 or signs of dissociation between ECHO and ECG findings. The partnership with centers specialized in amyloidosis combined with new technologies are fundamental in the management of these patients through specialized
obesity 587 more associated with female gender, advanced age and comorbidities such as hypertension, diabetes, obesity and chronic kidney disease. Amyloidosis is a clinical disorder characterized by the deposition of aggregates
obesity 2018 more associated with female gender, advanced age, and comorbidities such as hypertension, diabetes, obesity , and chronic kidney disease.[1]-[6] Recently, a picture of senile amyloidosis has been described in

You must be authorized to submit a review.