scholarly journals Experience with tafamidis in a patient with transthyretin amyloidosis

Kardiologiia ◽  
2020 ◽  
Vol 60 (3) ◽  
pp. 155-160
Author(s):  
S. N. Nasonova ◽  
I. V. Zhirov ◽  
M. M. Magomedov ◽  
M. A. Saidova ◽  
Yu. F. Osmolovskaya ◽  
...  

Transthyretin amyloidosis (ATTR) is a threatening and severe genetic disease characterized by damages to organs and systems caused by a pathological protein transthyretin produced in the liver. Clinical manifestations of this disease vary from injuries of the nervous system to injuries of the cardiovascular system. Prognosis for ATTR-amyloidosis remains unfavorable. The absence of pathognomonic symptoms complicates diagnostics of this disease, which tends to simulate other conditions. At present, medicines exist, which are pathogenetic in the treatment of ATTR-amyloidosis. The article describes a clinical case of ATTR-amyloidosis with primary heart injury complicated with functional class III chronic heart failure during the tafamidis treatment.

2019 ◽  
Vol 28 (1) ◽  
pp. 3-13 ◽  
Author(s):  
J. F. Veenis ◽  
J. J. Brugts

AbstractExacerbations of chronic heart failure (HF) with the necessity for hospitalisation impact hospital resources significantly. Despite all of the achievements in medical management and non-pharmacological therapy that improve the outcome in HF, new strategies are needed to prevent HF-related hospitalisations by keeping stable HF patients out of the hospital and focusing resources on unstable HF patients. Remote monitoring of these patients could provide the physicians with an additional tool to intervene adequately and promptly. Results of telemonitoring to date are inconsistent, especially those of telemonitoring with traditional non-haemodynamic parameters. Recently, the CardioMEMS device (Abbott Inc., Atlanta, GA, USA), an implantable haemodynamic remote monitoring sensor, has shown promising results in preventing HF-related hospitalisations in chronic HF patients hospitalised in the previous year and in New York Heart Association functional class III in the United States. This review provides an overview of the available evidence on remote monitoring in chronic HF patients and future perspectives for the efficacy and cost-effectiveness of these strategies.


2015 ◽  
Vol 87 (2) ◽  
pp. 1033-1040 ◽  
Author(s):  
Felipe C. Souza ◽  
Emiliana B. Marques ◽  
Rogério B.M. Scaramello ◽  
B.V. Christianne

Digoxin is used for heart failure associated to systolic dysfunction and high ventricular rate. It has a narrow therapeutic range and intoxication may occur due to drug interactions or comorbidities. The aim of this work was to study digoxin use in a public health unit delineating the profile of patients susceptible to digitalis intoxication. Medical records belonging to patients admitted to the cardiomyopathy ward of the health unit (2009-2010) and in use of digoxin were analyzed. Among 647 patients admitted, 185 individuals using digoxin and possessed records available. The registration of plasma digoxin concentration was found in 80 records and it was out of the therapeutic range in 42 patients (52.5%). This group of individuals was constituted mainly by males patients (79%), functional class III of heart failure (65%), exhibiting renal failure (33%). The evaluated sample reflects the epidemiology of heart failure in Brazil and, although pharmacotherapy had been according to Brazilian Guidelines, apparently the monitoring was not performed as recommended. This work highlighs the necessity of plasma digoxin constant monitoring during pharmacotherapy and the development of protocols that enable a safer use, especially in male patients, functional class III and with renal dysfunction.


1994 ◽  
Vol 267 (2) ◽  
pp. H443-H448 ◽  
Author(s):  
H. Wroblewski

Baroreceptor-induced peripheral reflex vasoconstriction during upright posture is an important edema-prevention mechanism in humans. Congestive heart failure (CHF) has been associated with blunted baroreceptor control of regional blood flow during short-term head-up tilt. The effect of prolonged unloading of baroreceptors on subcutaneous blood flow of the calf was investigated in 12 healthy subjects and in 13 patients with severe idiopathic dilated cardiomyopathy (New York Heart Association functional class III or IV). The subjects were studied both supine and sitting for 3-h periods. When sitting, subcutaneous vascular resistance decreased -26 +/- 19% in CHF patients and increased 90 +/- 69% in control subjects (P < 0.0001). The corresponding subcutaneous blood flow increased 43 +/- 29% in patients with CHF compared with the decrease of -42 +/- 17% in control subjects (P < 0.0001). I conclude that patients with CHF secondary to idiopathic dilated cardiomyopathy have an abnormal baroreceptor-mediated peripheral vasodilation during orthostatic stress that is sustained for hours. This extended paradoxical vasodilation may participate as an additional pathophysiological mechanism contributing to lower extremity edema in patients with CHF.


2013 ◽  
Vol 88 (3) ◽  
pp. 329-343 ◽  
Author(s):  
Joao Roberto Antonio ◽  
Eny Maria Goloni-Bertollo ◽  
Livia Arroyo Tridico

Neurofibromatosis, which was first described in 1882 by Von Recklinghausen, is a genetic disease characterized by a neuroectodermal abnormality and by clinical manifestations of systemic and progressive involvement which mainly affect the skin, nervous system, bones, eyes and possibly other organs. The disease may manifest in several ways and it can vary from individual to individual. Given the wealth of information about neurofibromatosis, we attempted to present this information in different ways. In the first part of this work, we present a chronological history, which describes the evolution of the disease since the early publications about the disorder until the conclusion of this work, focusing on relevant aspects which can be used by those wishing to investigate this disease. In the second part, we present an update on the various aspects that constitute this disease.


Kardiologiia ◽  
2021 ◽  
Vol 61 (10) ◽  
pp. 104-107
Author(s):  
A. A. Proshkina ◽  
N. A. Tsareva ◽  
G. V. Nekludova ◽  
S. N. Avdeev

The article presents a clinical case of successful triple combination therapy in a female patient with functional class III idiopathic pulmonary arterial hypertension. Supplementing the previous macitentan and riociguat treatment with selexipag reduced the severity of clinical manifestations of pulmonary hypertension. Also, the treatment efficacy was demonstrated by improvement of laboratory and instrumental indexes. Time-related changes were evaluated at 3 months after initiation of the selexipag treatment.


2019 ◽  
Vol 2019 ◽  
pp. 1-8 ◽  
Author(s):  
Jing Ye ◽  
Zhen Wang ◽  
Di Ye ◽  
Yuan Wang ◽  
Menglong Wang ◽  
...  

Background. Interleukin-11 (IL-11) is an important inflammatory cytokine and has been demonstrated to participate in cardiovascular diseases. However, there have been no studies about the role of IL-11 in heart failure (HF). The present study is aimed at investigating whether IL-11 levels are associated with the cardiac prognosis in patients with HF. Methods. The plasma concentrations of IL-11 were measured in 240 patients with chronic HF (CHF) and 80 control subjects without signs of significant heart disease. In addition, we prospectively followed these CHF patients to endpoints of cardiac events. Results. Compared with the control group, the plasma IL-11 concentrations were significantly increased in the CHF patients and gradually increased in the New York Heart Association (NYHA) functional class II group, the NYHA functional class III group, and the NYHA functional class IV group. The receiver operating characteristic (ROC) curve revealed that the predictive role of IL-11 in HF is not as good as N-terminal B-type natriuretic peptide (BNP), although IL-11 has a certain value in predicting cardiac events. In addition, the CHF patients were divided into 3 groups according to the plasma IL-11 concentration category (low, T1; middle, T2; and high, T3). The multivariate Cox hazard analysis showed that the high plasma IL-11 concentrations were independently associated with the presence of cardiac events after adjustment for confounding factors. Furthermore, the CHF patients were divided into two groups based on the median plasma IL-11 concentrations. The Kaplan-Meier analysis revealed that the patients with high IL-11 concentrations had a higher risk of cardiac events compared with those with low IL-11 concentrations. Conclusions. Higher plasma IL-11 levels significantly increase the presence of cardiac events and suggest a poor outcome; although the diagnostic value of IL-11 in CHF is not as good as BNP, there is a certain value in predicting cardiac events in CHF.


2019 ◽  
Vol 20 (6) ◽  
pp. 1287 ◽  
Author(s):  
Nelson Ferreira ◽  
Maria Saraiva ◽  
Maria Almeida

Transthyretin (TTR) amyloidoses (ATTR amyloidosis) are diseases associated with transthyretin (TTR) misfolding, aggregation and extracellular deposition in tissues as amyloid. Clinical manifestations of the disease are variable and include mainly polyneuropathy and/or cardiomyopathy. The reasons why TTR forms aggregates and amyloid are related with amino acid substitutions in the protein due to mutations, or with environmental alterations associated with aging, that make the protein more unstable and prone to aggregation. According to this model, several therapeutic approaches have been proposed for the diseases that range from stabilization of TTR, using chemical chaperones, to clearance of the aggregated protein deposited in tissues in the form of oligomers or small aggregates, by the action of disruptors or by activation of the immune system. Interestingly, different studies revealed that curcumin presents anti-amyloid properties, targeting multiple steps in the ATTR amyloidogenic cascade. The effects of curcumin on ATTR amyloidosis will be reviewed and discussed in the current work in order to contribute to knowledge of the molecular mechanisms involved in TTR amyloidosis and propose more efficient drugs for therapy.


Medicina ◽  
2020 ◽  
Vol 56 (9) ◽  
pp. 434
Author(s):  
Pungkava Sricharoen ◽  
Phichayut Phinyo ◽  
Jayanton Patumanond ◽  
Dilok Piyayotai ◽  
Yuwares Sittichanbuncha ◽  
...  

Background and objectives: Acute heart failure is a common problem encountered in the emergency department (ED). More than 80% of the patients with the condition subsequently require lengthy and repeated hospitalization. In a setting with limited in-patient capacity, the patient flow is often obstructed. Appropriate disposition decisions must be made by emergency physicians to deliver effective care and alleviate ED overcrowding. This study aimed to explore clinical predictors influencing the length of stay (LOS) in patients with acute heart failure who present to the ED. Materials and Methods: We conducted prognostic factor research with a retrospective cohort design. Medical records of patients with acute heart failure who presented to the ED of Ramathibodi Hospital from January to December 2015 were assessed for eligibility. Thirteen potential clinical predictors were selected as candidates for statistical modeling based on previous reports. Multivariable Poisson regression was used to estimate the difference in LOS between patients with and without potential predictors. Results: A total of 207 patients were included in the analysis. Most patients were male with a mean age of 74.2 ± 12.5 years. The median LOS was 54.6 h (Interquartile range 17.5, 149.3 h). From the multivariable analysis, four clinical characteristics were identified as independent predictors with an increase in LOS. These were patients with New York Heart Association (NYHA) functional class III/IV (+72.9 h, 95%Confidence interval (CI) 23.9, 121.8, p = 0.004), respiratory rate >24 per minute (+80.7 h, 95%CI 28.0, 133.3, p = 0.003), hemoglobin level <10 mg/dL (+60.4 h, 95%CI 8.6, 112.3, p = 0.022), and serum albumin <3.5 g/dL (+52.8 h, 95%CI 3.6, 102.0, p = 0.035). Conclusions: Poor NYHA functional class, tachypnea, anemia, and hypoalbuminemia are significant clinical predictors of patients with acute heart failure who required longer LOS.


2017 ◽  
Vol 98 (6) ◽  
pp. 906-909 ◽  
Author(s):  
I P Ponomareva

Aim. Comparative analysis of the profile of integral functional disorders and the degree of their deficit in elderly patients after stroke and in chronic heart failure functional class III-IV. Methods. Physical exam and interviews were used during comprehensive geriatric assessment with the analysis of the degree of functional disorders according to PULSES Profile scale and the degree of asthenia in the elderly. Results. The analysis of the degree of functional disorders in elderly and senile patients showed the diversity of issues that should be considered when providing medical and social care. In a moderate functional deficit in patients after stroke emotional and intellectual disorders prevailed - 47.2±0.1 cases, as well as upper extremities dysfunction - 29.9±0.2, and lower extremities dysfunction - 27.6±0.2. In severe heart failure inability to control excretory functions is predominant - 14.6±0.2 patients. Severe functional disorders prevailed in patients with consequences of stroke, which manifested as upper extremities dysfunction - 21.3 cases; lower extremities dysfunction - 22 cases; in severe heart failure communication disorders were prevalent - 6.4 cases. Conclusion. The more severe degree of functional deficit correlating with a high need for palliative medical and social care was observed more frequently in patients after stroke with prevailing upper and lower extremities dysfunction, while in chronic heart failure functional class III-IV communication disorders were predominant.


2018 ◽  
Vol 96 (3) ◽  
pp. 273-276
Author(s):  
N. A. Kosheleva ◽  
Elena Yu. Ponomareva ◽  
D. S. Sedov

The presented clinical case describes the development of diffuse myocarditis in a 31-year-old man with the outcome of dilated cardiomyopathy and terminal chronic heart failure, resistant to drug therapy that required heart transplantation. Discussed aspects of diagnosis, clinical manifestations, tactics of patient management and indications for heart transplantation.


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