scholarly journals Long term prognosis and cardiovascular complications of patients with systemic sclerosis-related cardiomiopathy

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
G Stronati ◽  
F Ribichini ◽  
D Benfaremo ◽  
C Dichiara ◽  
M Casella ◽  
...  

Abstract Background Both primary (SSc related cardiomyopathy) and secondary cardiac involvement in systemic sclerosis (SSc) is frequent albeit mostly asymptomatic. It represents a negative prognostic factor as almost 25% of SSc patients die from either heart failure or arrhythmia complications. Speckle tracking global longitudinal strain has been proven to be an effective tool both to identify and detect the progress of subclinical heart disease in SSc. The aim of our study was to assess the association between SSc diagnosis and the development of heart failure, pulmonary hypertension, death and need for hospitalization. Materials and methods We conducted an observational prospective study enrolling all patients with a diagnosis of SSc and no overt cardiac disease. We excluded all patients with a known diagnosis of pulmonary hypertension and atrial fibrillation. For each patient standard echocardiogram and GLS variables were collected. Results We enrolled 70 patients (61 females, age 56.2±15.4 years) who were followed for a median of 3 years. 68% of the patients had a limited variant of the disease. All-cause mortality was 10% in a 5-year follow-up. During the same period, PH was reached in 13% of all patients, HF in 7% and 18% required at least one hospital admission for cardiovascular causes. A diagnosis of PH was associated with an increased risk of death (ARR 34%; p<0.001) and hospitalization for CV causes (ARR 73%; p<0.001). Moreover, a diagnosis of HF was associated with an increased risk of death (ARR 50%; p<0.001) and hospitalization for CV causes (ARR 61%; p<0.001). A 1% worsening of GLS was associated with an increased risk to develop PH in the following 5 years, both for the left ventricle (OR 1.2; 95% CI: 1.1–1.4; p=0.043) and for the right ventricle (OR 1.1; 95% CI: 1.1–1.3; p=0.045). Conclusions Pulmonary hypertension and heart failure often occur in patients with cardiac involvement. Both proved themselves to be associated with an increased risk of death and hospitalization for CV causes. Moreover, GLS worsening of both the left and right ventricles may allow us to predict the diagnosis of PH and therefore preemptively start appropriate management. FUNDunding Acknowledgement Type of funding sources: None.

2020 ◽  
Vol 27 (17) ◽  
pp. 1876-1886
Author(s):  
Giulia Stronati ◽  
Lucia Manfredi ◽  
Alessia Ferrarini ◽  
Lucia Zuliani ◽  
Marco Fogante ◽  
...  

Aims Cardiac involvement in patients with systemic sclerosis (SSc) is frequent and represents a negative prognostic factor. Recent studies have described subclinical heart involvement of both the right ventricle (RV) and left ventricle (LV) via speckle-tracking-derived global longitudinal strain (GLS). It is currently unknown if SSc-related cardiomyopathy progresses through time. Our aim was to assess the progression of subclinical cardiac involvement in patients with SSc via speckle-tracking-derived GLS. Methods This was a prospective longitudinal study enrolling 72 consecutive patients with a diagnosis of SSc and no structural heart disease nor pulmonary hypertension. A standard echocardiographic exam and GLS calculations were performed at baseline and at follow-up. Results Traditional echocardiographic parameters did not differ from baseline to 20-month follow-up. LV GLS, despite being already impaired at baseline, worsened significantly during follow-up (from –19.8 ± 3.5% to –18.7 ± 3.5%, p = .034). RV GLS impairment progressed through the follow-up period (from –20.9 ± 6.1% to –18.7 ± 5.4%, p = .013). The impairment was more pronounced for the endocardial layers of both LV (from –22.5 ± 3.9% to –21.4 ± 3.9%, p = .041) and RV (–24.2 ± 6.2% to –20.6 ± 5.9%, p = .001). A 1% worsening in RV GLS was associated with an 18% increased risk of all-cause death or major cardiovascular event ( p = .03) and with a 55% increased risk of pulmonary hypertension ( p = .043). Conclusion SSC-related cardiomyopathy progresses over time and can be detected by speckle-tracking GLS. The highest progression towards reduced deformation was registered for the endocardial layers, which supports the hypothesis that microvascular dysfunction is the main determinant of heart involvement in SSc patients and starts well before overt pulmonary hypertension.


2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Meghan J. Arwood ◽  
Nasim Vahabi ◽  
Christelle Lteif ◽  
Ravindra K. Sharma ◽  
Roberto F. Machado ◽  
...  

AbstractHeart failure with preserved ejection fraction (HFpEF) patients who develop pulmonary hypertension (PH) have an increased risk of death, with combined pre- and post-capillary PH (CpcPH) having the highest risk. However, the mechanism behind PH development in HFpEF is poorly understood. We aimed to identify transcriptomic associations with PH development in HFpEF. Blood was collected from 30 HFpEF patients: 10 without PH, 10 with isolated post-capillary PH, and 10 with CpcPH. Gene expression measurements were completed using transcriptome-wide RNA sequencing. Gene expression differences were compared using a quasi-likelihood method adjusting for age, sex, race, and smoking-status. Biological pathways were compared using global gene expression differences. A replication in 34 additional heart failure patients and a validation in lung tissue from a representative mouse model were completed using quantitative PCR. Six differentially expressed genes were identified when comparing transcriptomics between subjects with CpcPH and those without PH. When tested in additional subjects, only the association with ID2 replicated. Consistent with clinical findings, Id2 expression was also upregulated in mice with HFpEF and PH. Pathway analysis identified proliferative and mitochondrial pathways associated with CpcPH. Thus, these patients may possess systemic pathophysiological differences similar to those observed in pulmonary arterial hypertension patients.


Medicina ◽  
2020 ◽  
Vol 56 (4) ◽  
pp. 167
Author(s):  
Lina Padervinskiene ◽  
Deimante Hoppenot ◽  
Ausra Krivickiene ◽  
Birute Gumauskiene ◽  
Irena Nedzelskiene ◽  
...  

Background and objectives: Non-invasive imaging of the heart has an important place in the diagnosis and management of pulmonary arterial hypertension (PAH). The aim of this study was to establish the thresholds of cardiac magnetic resonance imaging (CMRI)-derived biventricular deformation, function parameters, and levels of N-terminal pro brain natriuretic peptide (NT-proBNP) for the prediction of survival of pre-capillary pulmonary hypertension (PHprecap) patients. Materials and Methods: In total, 64 incident PHprecap cases, who underwent CMRI, were consecutively enrolled in a prospective cohort study. Patients underwent a systemic evaluation, including measurement of NT-proBNP, two-dimensional (2D) echocardiography, six-minute walk test (6MWT), CMRI with feature tracking (FT), and right-heart catheterization (RHC). Patients were divided into two groups according to one-year survival (survival and non-survival groups). Survival analysis was performed. Results: One-year survival was 79.6%. The distribution between age, sex, mean pulmonary artery pressure (mPAP), New York Heart Association (NYHA) functional class, and 6MWT did not differ between the groups. Survival was significantly lower in the PAH group associated with connective tissue disease (CTD-PAH), where 44% (n = 4) of patients died during the first year. Univariate analysis revealed that severely reduced right-ventricle (RV) ejection fraction (EF) <25.5%, left-ventricle global longitudinal strain (LV GLS) >−14.18%, and right pulmonary artery (RPA) relative area change (RAC) <19%, and severely increased NT-proBNP level >1738 (ng/L) indicate an increased risk of death in PHprecap patients. Conclusions: Impaired RV systolic function and LV global longitudinal strain, decrease of pulmonary artery distensibility, and CTD-PAH etiology, together with high NT-proBNP level, impair prognosis in pre-capillary PH patients. These findings are important for the risk stratification and management of pre-capillary pulmonary hypertension patients.


2021 ◽  
Vol 50 (Supplement_1) ◽  
Author(s):  
Ingrid Stacey ◽  
Daniela Bond-Smith ◽  
Rebecca Seth ◽  
Jeffrey Cannon ◽  
Kevin Murray ◽  
...  

Abstract Background Rheumatic Heart Disease (RHD), a chronic acquired heart-valve disease, is a major contributor to morbidity and mortality among Indigenous Australians. We estimate the probability and determinants of progressing from RHD diagnosis to cardiovascular complications/death in the Australian population, addressing methodological shortcomings in existing evidence. Methods This retrospective cohort study used linked RHD register, hospital and death data from five Australian jurisdictions. Cardiovascular complications (heart failure, stroke, endocarditis, atrial fibrillation) and all-cause mortality were measured for people aged &lt;35years diagnosed with first-ever RHD (2010-2018; minimum 8.5year look-back; maximum 8year follow-up). Probability of cardiovascular complications were determined with competing risk of death. Proportional cause-specific hazard regression measured independent predictors of outcomes. Results 1718 RHD cases aged &lt;35years were identified (85% Indigenous, 11% migrant, 63% women, 40% 5-14years, 16% metropolitan). Within 8years, 22% experienced a cardiovascular complication (50% heart failure) and 3.2% died. Age ≥14years (Hazard Ratio,HR:2.0, 95% Confidence Interval,CI:1.4-3.0) and metropolitan residence (HR:3.2, 95%CI:2.0-5.1) were associated with increased risk of cardiovascular complications in cases (relative to &lt; 14years and non-metropolitan). Record of ARF prior to RHD was protective (HR:0.6, 95%CI:0.4-0.8). Conclusions Competing risks methods applied to linked data have provided the most comprehensive multi-jurisdictional estimates of RHD progression to cardiovascular complications/death in Australia. Risk of cardiovascular events was highest in metropolitan areas but clinical history of ARF was associated with reduced risk of progression. Key messages Despite access to the world-class Australian healthcare system, one-fifth of initially uncomplicated RHD diagnoses will progress to one of the complications examined within 8years


EP Europace ◽  
2021 ◽  
Vol 23 (Supplement_3) ◽  
Author(s):  
M Mpotis ◽  
A Kartas ◽  
A Samaras ◽  
E Akrivos ◽  
E Vrana ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. OnBehalf MISOAC- AF study group BACKGROUND Digoxin is widely used in atrial fibrillation (AF) and heart failure (AF). However, established evidence is conflicting regarding its association with clinical outcomes. AIM To investigate the relation between digoxin and adverse outcomes in patients with AF, with or without HF, in a contemporary AF cohort. METHODS We performed a retrospective analysis of data from 698 patients, originating from the MISOAC- AF (Motivational Interviewing to Support Oral AntiCoagulation Adherence in patients with non-valvular Atrial Fibrillation) trial, and followed over a median of 2.5 years. HF was denoted at baseline. The primary outcome was all-cause mortality and the secondary outcome was all-cause hospitalization, in a time-to-event analysis. Propensity scores were used to derive matched populations, balanced on key baseline covariates. To limit potential confounding, we also implemented inverse probability of treatment weighting (IPTW) analysis. RESULTS Among patients with HF, 10.5% (n = 39) were administered digoxin at baseline, whereas 89.5% (n = 331) were not. Digoxin administration was not associated with an increased risk of death (hazard ratio (HR) in the digoxin group, 1.21; 95% Confidence Interval (CI), 0.69 to 2.13, p = 0.5) or hospitalization of any cause (HR 1.15; 95% CI, 0.67 to 1.96; p = 0.6). Among patients without HF, 3.5% (n = 11) were administered digoxin, with neutral effects on all-cause mortality (HR: 3.25; 95% CI, 0.98 to 10.70), p = 0.06) and all-cause hospitalization (HR, 1.15; 95% CI, 0.67 to 1.96, p = 0.60). Consistent qualitatively results were observed using IPTW. CONCLUSIONS Among patients with AF, digoxin administration was not associated with an increased risk of death and hospitalization of any cause, irrespective of HF status. Abstract Figure.


Author(s):  
Shannon C. Kelly ◽  
Christoph D. Rau ◽  
An Ouyang ◽  
Pamela K. Thorne ◽  
T. Dylan Olver ◽  
...  

Heart failure (HF) patients with deteriorating right ventricular (RV) structure and function have a nearly two-fold increased risk of death compared to those without. Despite the well-established clinical risk, few studies have examined the molecular signature associated with this HF condition. The purpose of this study was to integrate morphological, molecular, and functional data with the transcriptome dataset in the RV of a pre-clinical model of cardio-metabolic HF. Ossabaw swine were fed either normal diet without surgery (lean control, n=5) or Western Diet and aortic-banding (WD-AB; n=4). Postmortem RV weight was increased and positively correlated with lung weight in the WD-AB group compared to CON. Total RNA-seq was performed and gene expression profiles were compared and analyzed using Principal Component Analysis, Weighted Gene Co-Expression Network Analysis, Module Enrichment Analysis, and Ingenuity Pathway Analysis. Gene networks specifically associated with RV hypertrophic remodeling identified a hub gene in MAPK8 (or JNK1) that was associated with the selective induction of the extracellular matrix (ECM) component fibronectin. JNK1 and fibronectin protein were increased in the right coronary artery (RCA) of WD-AB animals and associated with a decrease in matrix metalloproteinase 14 protein, which specifically degrades fibronectin. RCA fibronectin content was correlated with increased vascular stiffness evident as a decreased elastin elastic modulus in WD-AB animals. In conclusion, this study establishes a molecular and transcriptome signature in the RV using Ossabaw swine with cardio-metabolic HF. This signature was associated with altered ECM regulation and increased vascular stiffness in the RCA, with selective dysregulation of fibronectin.


2020 ◽  
pp. 13-17
Author(s):  
Dmitrii Aleksandrovich Lopyn ◽  
Stanislav Valerevich Rybchynskyi ◽  
Dmitrii Evgenevich Volkov

Currently the electrophysiological treatment options have been considered to be the most effective for many patients with arrhythmogenic cardiomyopathies, as well as in those with arrhythmias on the background of heart failure. Currently, the dependence of efficiency of the pacemakers on the location of the electrodes has been proven. In order to study the effect of a myocardial dysynchrony on the effectiveness of pacing depending on the location of the right ventricular electrode, an investigation has been performed. This study comprised the patients with a complete atrioventricular block, preserved ejection fraction of the left ventricle (more than 50 %), with no history of myocardial infarction, who were implanted with the two−chamber pacemaker. It has been established that the best results were achieved with a stimulation of the middle and lower septal zone of the right ventricle, the worst ones were obtained with a stimulation of its apex. It has been found that the dynamics of the magnitude of segmental strains and a global longitudinal strain coincided with the dynamics of other parameters of the pacemaker effectiveness, which indicated the pathogenetic value of myocardial dysynchrony in the progression of heart failure after implantation of the pacemaker. Therefore it could be concluded that the studying of myocardial mobility by determining a longitudinal strain for assessing the functional state of the myocardium and the effectiveness of pacing is highly advisable. It is emphasized that the use of the latest strains−dependent techniques for cardiac performance evaluation in the patients with bradyarrhythmia have a great potential to predict the development of chronic heart failure and to choose the optimal method of physiological stimulation of the heart. Key words: right ventricular lead, cardiac stimulation, myocardial dyssynchrony.


Author(s):  
Sheila Krishnan ◽  
Erin M. Fricke ◽  
Marcos Cordoba ◽  
Laurie A. Chalifoux ◽  
Reda E. Girgis

Abstract Purpose of review This study aims to describe the pathophysiology of pregnancy in pulmonary hypertension (PH) and review recent literature on maternal and fetal outcomes. Recent findings There is an increasing number of pregnant women with PH. Maternal mortality in pulmonary arterial hypertension (PAH) ranges from 9 to 25%, most commonly from heart failure and arrythmias. The highest risk of death is peri-partum and post-partum. Fetal/neonatal morbidity and mortality are also substantial. There are high rates of prematurity, intrauterine growth retardation, and preeclampsia. Women should be referred to expert centers for management. Combination PAH therapy with parenteral prostacyclin and a phosphodiesterase type V inhibitor is recommended. Induced vaginal delivery is preferred, except in cases of severe heart failure or obstetric indications for cesarean section. Summary Despite advances in management, pregnancy in PAH remains a high-risk condition and should be prevented.


2021 ◽  
pp. 239719832110340
Author(s):  
Yasser A Radwan ◽  
Reto D Kurmann ◽  
Avneek S Sandhu ◽  
Edward A El-Am ◽  
Cynthia S Crowson ◽  
...  

Objectives: To study the incidence, risk factors, and outcomes of conduction and rhythm disorders in a population-based cohort of patients with systemic sclerosis versus nonsystemic sclerosis comparators. Methods: An incident cohort of patients with systemic sclerosis (1980–2016) from Olmsted County, MN, was compared to age- and sex-matched nonsystemic sclerosis subjects (1:2). Electrocardiograms, Holter electrocardiograms, and a need for cardiac interventions were reviewed to determine the occurrence of any conduction or rhythm abnormalities. Results: Seventy-eight incident systemic sclerosis cases and 156 comparators were identified (mean age 56 years, 91% female). The prevalence of any conduction disorder before systemic sclerosis diagnosis compared to nonsystemic sclerosis subjects was 15% versus 7% ( p = 0.06), and any rhythm disorder was 18% versus 13% ( p = 0.33). During a median follow-up of 10.5 years in patients with systemic sclerosis and 13.0 years in nonsystemic sclerosis comparators, conduction disorders developed in 25 patients with systemic sclerosis with cumulative incidence of 20.5% (95% confidence interval: 12.4%–34.1%) versus 28 nonsystemic sclerosis patients with cumulative incidence of 10.4% (95% confidence interval: 6.2%–17.4%) (hazard ratio: 2.57; 95% confidence interval: 1.48–4.45), while rhythm disorders developed in 27 patients with systemic sclerosis with cumulative incidence of 27.3% (95% confidence interval: 17.9%–41.6%) versus 43 nonsystemic sclerosis patients with cumulative incidence of 18.0% (95% confidence interval: 12.3%–26.4%) (hazard ratio: 1.62; 95% confidence interval: 1.00–2.64). Age, pulmonary hypertension, and smoking were identified as risk factors. Conclusion: Patients with systemic sclerosis have an increased risk of conduction and rhythm disorders both at disease onset and over time, compared to nonsystemic sclerosis patients. These findings warrant increased vigilance and screening for electrocardiogram abnormalities in systemic sclerosis patients with pulmonary hypertension.


2021 ◽  
Vol 3 (6) ◽  
pp. 17-22
Author(s):  
Selia Chowdhury ◽  
Mehedi Hasan Bappy ◽  
Samia Chowdhury ◽  
Md. Shahraj Chowdhury ◽  
Nurjahan Shipa Chowdhury

A global pandemic instigated by SARS-CoV-2 virus has been going strongly for almost two years. There have been almost 240 million cases with almost 5 million fatalities all over the world so far. COVID has affected almost every aspect of people’s lives halting the world in immeasurable ways. Although COVID-19 is mostly affecting the respiratory system, it is also responsible for a significant amount of cardiovascular system involvement. On one hand, it deteriorates any pre-existing cardiovascular condition, on another hand, it triggers other inflammation-facilitated acute events. They comprise of inflammatory heart disease, severe pericardial effusion, ventricular arrhythmias, endothelial dysfunction, pulmonary hypertension, heart failure, and many more. In this article, we summarize the features, management, and treatment of several important COVID-19 induced cardiovascular complications. Recent advances in the treatment of these diseases along with the justification and concerns of specific therapeutic measures are also discussed.


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