scholarly journals What’s New in Cirrhotic Cardiomyopathy?—Review Article

2021 ◽  
Vol 11 (12) ◽  
pp. 1285
Author(s):  
Aleksandra Bodys-Pełka ◽  
Maciej Kusztal ◽  
Joanna Raszeja-Wyszomirska ◽  
Renata Główczyńska ◽  
Marcin Grabowski

Cirrhotic cardiomyopathy (CCM) is a relatively new medical term. The constant development of novel diagnostic and clinical tools continuously delivers new data and findings about this broad disorder. The purpose of this review is to summarize current facts about CCM, identify gaps of knowledge, and indicate the direction in which to prepare an updated definition of CCM. We performed a review of the literature using scientific data sources with an emphasis on the latest findings. CCM is a clinical manifestation of disorders in the circulatory system in the course of portal hypertension. It is characterized by impaired left ventricular systolic and diastolic dysfunction, and electrophysiological abnormalities, especially QT interval prolongation. However, signs and symptoms reported by patients are non-specific and include reduced exercise tolerance, fatigue, peripheral oedema, and ascites. The disease usually remains asymptomatic with almost normal heart function, unless patients are exposed to stress or exertion. Unfortunately, due to the subclinical course, CCM is rarely recognized. Orthotopic liver transplantation (OLTx) seems to improve circulatory function although there is no consensus about its positive effect, with reported cases of heart failure onset after transplantation. Researchers indicate a careful pre-, peri-, and post-transplant cardiac assessment as a crucial point in detecting CCM and improving patients’ prognosis. There is also an urgent need to update the CCM definition and establish a diagnostic algorithm for early diagnosis of CCM as well as a specific treatment of this condition.

2000 ◽  
Vol 23 (5) ◽  
pp. 325-330 ◽  
Author(s):  
D. Mihaylov ◽  
H. Reintke ◽  
P. Blanksma ◽  
E.D. De Jong ◽  
J. Elstrodt ◽  
...  

The goal of the present study was to develop a large animal model of acute ischemic left ventricular heart failure (LVHF) that can be used to assess the influence of the PUCA pump on the heart and circulatory system under realistic conditions. We tested the hypothesis that mild stenosis of the coronary artery in combination with mild ventricular pacing induces an acute heart failure condition, whereas the separate phenomena themselves do not lead to impaired heart function. Mean aortic pressure (AoP), left ventricular end-diastolic pressure (LVEDP), stroke volume (SV) and myocardial systolic shortening (MSS) were compared 30 minutes after a pacemaker (PM) induced tachycardia in anaesthetized sheep (n=3) without and with ± 50% stenosis of the proximal LCx. All parameters measured restored to basic levels when stenosis was absent. When the LCx was partially occluded, mild PM-induced tachycardia resulted in decreased AoP (P=0.045) as well as in decreased SV (P=0.048); the LVEDP remained high (P=0.002). Also the recovery of MSS was impaired when stenosis was present (P=0.002). These values indicate that acute heart failure conditions were present. The technique used proved to be safe and allowd fine-tuning of the demand ischemia by adapting heart frequency to the required heart failure conditions. The model can be used to study the effect of LV mechanical support during acute heart failure conditions.


The Chornobyl accident in Ukraine affected more than 3 million people. The impact of the disaster on human health is multifaceted and is associated with both the direct effects of ionizing radiation and other negative factors of the accident. Diseases of the circulatory system are one of the leading causes of death in this population. The combination of hypertension disease and gastroesophageal reflux disease reveals forms of structural and functional indicators of the heart, characterized by a higher level of left ventricular myocardial hypertrophy, regardless of the stage of HD and the degree of blood pressure. The aim was to determine the features of structural and functional indicators of the heart in the combined course of HD and GERD in the liquidators of the Chornobyl accident, as well as to establish differences from the indicators of patients with isolated HD. Materials and methods of research. A comprehensive survey of 105 liquidators of the Chornobyl accident aged 52 to 69 years (mean age 58.5 ± 0.8 years), 85.7 % of men and 14.3 % of women was conducted. All of them were hospitalized in therapeutic and / or observed on an outpatient basis in the outpatient departments of the «Regional Clinical Specialized Dispensary for Radiation Protection of the Population» of Kharkiv. Patients were divided into 2 groups. The first group – 52 patients with a combined course of HD and GERD, 44 (84.6 %) men and 8 (15.4 %) women. The second – 53 patients with isolated HD, 46 (86.8 %) men and 7 (13.2 %) women. All patients in outpatient settings underwent a comprehensive medical examination, including Doppler echocardiography. Results and conclusions. When comparing the structural and functional parameters of the heart in the combined course of HD and GERD in the liquidators of the Chornobyl accident with an isolated course of HD, it was found that almost all indicators of systolic, diastolic and pumping heart function are statistically significantly increased, p ≤ 0.05, except for aortic pressure size gradient and diameter of right ventricule. Thus, patients with a combined course of pathology have more pronounced changes in the structural and functional parameters of the heart, thereby increasing the risk of cardiovascular complications. Prospects for the study are relevant and necessary for further prediction of the risk of complications and individual therapeutic approach in comorbid pathology in the liquidators of the Chernobyl accident.


2021 ◽  
Vol 12 (1) ◽  
pp. 76-88
Author(s):  
Kieran Longley ◽  
Tuan Tran ◽  
Vincent Ho

Cirrhotic cardiomyopathy (CCM), cardiac dysfunction in end-stage liver disease in the absence of prior heart disease, is an important clinical entity that contributes significantly to morbidity and mortality. The original definition for CCM, established in 2005 at the World Congress of Gastroenterology (WCG), was based upon known echocardiographic parameters to identify subclinical cardiac dysfunction in the absence of overt structural abnormalities. Subsequent advances in cardiovascular imaging and in particular myocardial deformation imaging have rendered the WCG criteria outdated. A number of investigations have explored other factors relevant to CCM, including serum markers, electrocardiography, and magnetic resonance imaging. CCM characteristics include a hyperdynamic circulatory state, impaired contractility, altered diastolic relaxation, and electrophysiological abnormalities, particularly QT interval prolongation. It is now known that cardiac dysfunction worsens with the progression of cirrhosis. Treatment for CCM has traditionally been limited to supportive efforts, but new pharmacological studies appear promising. Left ventricular diastolic dysfunction in CCM can be improved by targeted heart rate reduction. Ivabradine combined with carvedilol improves left ventricular diastolic dysfunction through targeted heart rate reduction, and this regimen can improve survival in patients with cirrhosis. Orthotopic liver transplantation also appears to improve CCM. Here, we canvass diagnostic challenges associated with CCM, introduce cardiac physiology principles and the application of echocardiographic techniques, and discuss the evidence behind therapeutic interventions in CCM.


2018 ◽  
Vol 69 (8) ◽  
pp. 2209-2212
Author(s):  
Alexandru Radu Mihailovici ◽  
Vlad Padureanu ◽  
Carmen Valeria Albu ◽  
Venera Cristina Dinescu ◽  
Mihai Cristian Pirlog ◽  
...  

Left ventricular noncompaction is a primary cardiomyopathy with genetic transmission in the vast majority of autosomal dominant cases. It is characterized by the presence of excessive myocardial trabecularities that generally affect the left ventricle. In diagnosing this condition, echocardiography is the gold standard, although this method involves an increased risk of overdiagnosis and underdiagnosis. There are also uncertain cases where echocardiography is inconclusive, a multimodal approach is needed, correlating echocardiographic results with those obtained by magnetic resonance imaging. The clinical picture may range from asymptomatic patients to patients with heart failure, supraventricular or ventricular arrhythmias, thromboembolic events and even sudden cardiac death. There is no specific treatment of left ventricular noncompaction, but the treatment is aimed at preventing and treating the complications of the disease. We will present the case of a young patient with left ventricular noncompactioncardiomyopathy and highlight the essential role of transthoracic echocardiography in diagnosing this rare heart disease.


2019 ◽  
Vol 26 (11) ◽  
pp. 1946-1959 ◽  
Author(s):  
Le Minh Tu Phan ◽  
Lemma Teshome Tufa ◽  
Hwa-Jung Kim ◽  
Jaebeom Lee ◽  
Tae Jung Park

Background:Tuberculosis (TB), one of the leading causes of death worldwide, is difficult to diagnose based only on signs and symptoms. Methods for TB detection are continuously being researched to design novel effective clinical tools for the diagnosis of TB.Objective:This article reviews the methods to diagnose TB at the latent and active stages and to recognize prospective TB diagnostic methods based on nanomaterials.Methods:The current methods for TB diagnosis were reviewed by evaluating their advantages and disadvantages. Furthermore, the trends in TB detection using nanomaterials were discussed regarding their performance capacity for clinical diagnostic applications.Results:Current methods such as microscopy, culture, and tuberculin skin test are still being employed to diagnose TB, however, a highly sensitive point of care tool without false results is still needed. The utilization of nanomaterials to detect the specific TB biomarkers with high sensitivity and specificity can provide a possible strategy to rapidly diagnose TB. Although it is challenging for nanodiagnostic platforms to be assessed in clinical trials, active TB diagnosis using nanomaterials is highly expected to achieve clinical significance for regular application. In addition, aspects and future directions in developing the high-efficiency tools to diagnose active TB using advanced nanomaterials are expounded.Conclusion:This review suggests that nanomaterials have high potential as rapid, costeffective tools to enhance the diagnostic sensitivity and specificity for the accurate diagnosis, treatment, and prevention of TB. Hence, portable nanobiosensors can be alternative effective tests to be exploited globally after clinical trial execution.


2021 ◽  
pp. 1-21
Author(s):  
Antonio Atalaia ◽  
Rabah Ben Yaou ◽  
Karim Wahbi ◽  
Annachiara De Sandre-Giovannoli ◽  
Corinne Vigouroux ◽  
...  

Background: Variants in the LMNA gene, encoding lamins A/C, are responsible for a growing number of diseases, all of which complying with the definition of rare diseases. LMNA-related disorders have a varied phenotypic expression with more than 15 syndromes described, belonging to five phenotypic groups: Muscular Dystrophies, Neuropathies, Cardiomyopathies, Lipodystrophies and Progeroid Syndromes. Overlapping phenotypes are also reported. Linking gene and variants with phenotypic expression, disease mechanisms, and corresponding treatments is particularly challenging in laminopathies. Treatment recommendations are limited, and very few are variant-based. Objective: The Treatabolome initiative aims to provide a shareable dataset of existing variant-specific treatment for rare diseases within the Solve-RD EU project. As part of this project, we gathered evidence of specific treatments for laminopathies via a systematic literature review adopting the FAIR (Findable, Accessible, Interoperable, and Reusable) guidelines for scientific data production. Methods: Treatments for LMNA-related conditions were systematically collected from MEDLINE and Embase bibliographic databases and clinical trial registries (Cochrane Central Registry of Controlled Trials, clinicaltrial.gov and EudraCT). Two investigators extracted and analyzed the literature data independently. The included papers were assessed using the Oxford Centre for Evidence-Based Medicine 2011 Levels of Evidence. Results: From the 4783 selected articles by a systematic approach, we identified 78 papers for our final analysis that corresponded to the profile of data defined in the inclusion and exclusion criteria. These papers include 2 guidelines/consensus papers, 4 meta-analyses, 14 single-arm trials, 15 case series, 13 cohort studies, 21 case reports, 8 expert reviews and 1 expert opinion. The treatments were summarized electronically according to significant phenome-genome associations. The specificity of treatments according to the different laminopathic phenotypical presentations is variable. Conclusions: We have extracted Treatabolome-worthy treatment recommendations for patients with different forms of laminopathies based on significant phenome-genome parings. This dataset will be available on the Treatabolome website and, through interoperability, on genetic diagnosis and treatment support tools like the RD-Connect’s Genome Phenome Analysis Platform.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Yankun Lyu ◽  
Vipin K. Verma ◽  
Younjee Lee ◽  
Iosif Taleb ◽  
Rachit Badolia ◽  
...  

AbstractIt is well established that the aging heart progressively remodels towards a senescent phenotype, but alterations of cellular microstructure and their differences to chronic heart failure (HF) associated remodeling remain ill-defined. Here, we show that the transverse tubular system (t-system) and proteins underlying excitation-contraction coupling in cardiomyocytes are characteristically remodeled with age. We shed light on mechanisms of this remodeling and identified similarities and differences to chronic HF. Using left ventricular myocardium from donors and HF patients with ages between 19 and 75 years, we established a library of 3D reconstructions of the t-system as well as ryanodine receptor (RyR) and junctophilin 2 (JPH2) clusters. Aging was characterized by t-system alterations and sarcolemmal dissociation of RyR clusters. This remodeling was less pronounced than in HF and accompanied by major alterations of JPH2 arrangement. Our study indicates that targeting sarcolemmal association of JPH2 might ameliorate age-associated deficiencies of heart function.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Mohammad Amin Abazari ◽  
Deniz Rafieianzab ◽  
M. Soltani ◽  
Mona Alimohammadi

AbstractAortic dissection (AD) is one of the fatal and complex conditions. Since there is a lack of a specific treatment guideline for type-B AD, a better understanding of patient-specific hemodynamics and therapy outcomes can potentially control the progression of the disease and aid in the clinical decision-making process. In this work, a patient-specific geometry of type-B AD is reconstructed from computed tomography images, and a numerical simulation using personalised computational fluid dynamics (CFD) with three-element Windkessel model boundary condition at each outlet is implemented. According to the physiological response of beta-blockers to the reduction of left ventricular contractions, three case studies with different heart rates are created. Several hemodynamic features, including time-averaged wall shear stress (TAWSS), highly oscillatory, low magnitude shear (HOLMES), and flow pattern are investigated and compared between each case. Results show that decreasing TAWSS, which is caused by the reduction of the velocity gradient, prevents vessel wall at entry tear from rupture. Additionally, with the increase in HOLMES value at distal false lumen, calcification and plaque formation in the moderate and regular-heart rate cases are successfully controlled. This work demonstrates how CFD methods with non-invasive hemodynamic metrics can be developed to predict the hemodynamic changes before medication or other invasive operations. These consequences can be a powerful framework for clinicians and surgical communities to improve their diagnostic and pre-procedural planning.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1452.3-1453
Author(s):  
A. Martynova ◽  
T. Popkova ◽  
H. Gerasimova

Background:N-terminal pro-brain natriuretic peptide (NT-proBNP) is a known marker of heart dysfunction, mainly described in patients with high activity of rheumatoid arthritis (RA). Further knowledge of the influence of the IL-6 receptor antagonist, tocilizumab (TCZ), on NT-proBNP levels and systolic heart function is yet to be obtained.Objectives:Access the effect of 12 months TCZ therapy on NT-proBNP levels, transthoracal ehocardiography results and analyze the association between congestive heart disease progression and RA activity.Methods:37 RA patients (pts) (31F/6M); median age 56,5 [48; 63,5] years; disease duration 48 [6; 348] months; DAS28 score 6,15 [5,44; 6,45]; rheumatoid factor (RF)+100%; anti–citrullinated protein antibody (ACPA) + 79,6% were treated in an open-label study with TCZ (8 mg/kg every 4 weeks). Identification of NT-pro-BNT in blood serum, transthoracal ultrasound evaluation of left ventriculum ejection fraction (LVEF), E/A ratio performed at baseline and 12 months.Results:11 (29,7%) pts had congestive heart disease (CHD) (II functional class of NYHA), 7 (18,9%) pts having signs of mild left ventricular dysfunction (LVD) as dyspnea, shortness of breath, cardiotropic treatment remained the same in the course of the study. After 12 month TCZ treatment as RA activity lowered (DAS28 2.32 [1,75; 3,15], р<0,05), NT-proBNP levels decreased (100,95 [57.9; 117.6] pg/ml to 90,46 [33.62; 106.6] pg/ml), along with elevation of LVEF (60,75 [60; 70]% to 67,68 [62.5; 73.5], p = 0,001). Increase of E/A (0,97 [0.8; 1.17] to 1,04 [0.7; 1.42] correlated with decrease of NT-proBNP level (r = -0,63, p=0,036). Raise of LVEF over 12 months correlated with decrease of RA activity according to SDAI scale (r= -0,670, p<0,05). No significant relationship between NT-proBNP levels, LVEF, E/A and other scales measuring RA activity was found. Clinically all patients had improvement in evaluation of their health and no signs of CHD or RVD progression were found.Conclusion:Use of TCZ in patients with active RA showed none to positive influence on heart condition, specifically, lowering NT-proBNP levels, improving LVEF and reducing clinical signs of LVD.References:[1]Pan Y, Li D, Ma J, Shan L, Wei M. NT-proBNP test with improved accuracy for the diagnosis of chronic heart failure. Medicine (Baltimore). 2017 Dec;96(51):e9181.[2]D Novikova, I Kirillova, E Markelova et al. The first report of significantly improvement of NT-proBNP level in rheumatoid arthritis patients treated with tofacitinib during 12-month follow-up, European Heart Journal, Volume 40, Issue Supplement_1, October 2019, ehz745.0836.[3]Pappas DA, Nyberg F, Kremer JM et al. Clin Rheumatol. 2018 Sep;37(9):2331-2340.Disclosure of Interests:None declared


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
M Barki ◽  
M Losito ◽  
M.M Caracciolo ◽  
F Bandera ◽  
M Rovida ◽  
...  

Abstract Background The right ventricle (RV) is extremely sensitive to hemodynamic changes and increased impedance. In acute heart failure (AHF), the development of pulmonary venous congestion and the increase of left ventricular (LV) filling pressures favors pulmonary vascular adverse remodeling and ultimately RV dysfunction, leading to the onset of symptoms and to a further decay of cardiac dynamics. Purpose The aim of the study was to evaluate RV morphology and functional dynamics at admission and discharge in patients hospitalized for AHF, analyzing the role and the response to treatment of the RV and its coupling with pulmonary circulation (PC). Methods Eighty-one AHF patients (mean age 75.75±10.6 years, 59% males) were prospectively enrolled within 24–48 hours from admission to the emergency department (ED). In either the acute phase and at pre-discharge all patients underwent M-Mode, 2-Dimensional and Doppler transthoracic echocardiography (TTE), as well as lung ultrasonography (LUS), to detect an increase of extravascular lung water (EVLW) and development of pleural effusion. Laboratory tests were performed in the acute phase and at pre-discharge including the evaluation of NT-proBNP. Results At baseline we observed a high prevalence of RV dysfunction as documented by a reduced RV systolic longitudinal function [mean tricuspid annular plane systolic excursion (TAPSE) at admission of 16.47±3.86 mm with 50% of the patients exhibiting a TAPSE&lt;16mm], a decreased DTI-derived tricuspid lateral annular systolic velocity (50% of the subjects showed a tricuspid s' wave&lt;10 cm/s) and a reduced RV fractional area change (mean FAC at admission of 36.4±14.6%). Furthermore, an increased pulmonary arterial systolic pressure (PASP) and a severe impairment in terms of RV coupling to PC was detected at initial evaluation (mean PASP at admission: 38.8±10.8 mmHg; average TAPSE/PASP at admission: 0.45±0.17 mm/mmHg). At pre-discharge a significant increment of TAPSE (16.47±3.86 mm vs. 17.45±3.88; p=0.05) and a reduction of PASP (38.8±10.8 mmHg vs. 30.5±9.6mmHg, p&lt;0.001) was observed. Furthermore, in the whole population we assisted to a significant improvement in terms of RV function and its coupling with PC as demonstrated by the significant increase of TAPSE/PASP ratio (TAPSE/PASP: 0.45±0.17 mm/mmHg vs 0.62±0.20 mm/mmHg; p&lt;0.001). Patients significantly reduced from admission to discharge the number of B-lines and NT-proBNP (B-lines: 22.2±17.1 vs. 6.5±5 p&lt;0.001; NT-proBNP: 8738±948 ng/l vs 4227±659 ng/l p&lt;0.001) (Figure 1). Nonetheless, no significant changes of left atrial and left ventricular dimensions and function were noted. Conclusions In AHF, development of congestion and EVLW significantly impact on the right heart function. Decongestion therapy is effective for restoring acute reversal of RV dysfunction, but the question remains on how to impact on the biological properties of the RV. Funding Acknowledgement Type of funding source: None


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