Subclinical progression of systemic sclerosis-related cardiomyopathy

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.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1098.1-1099
Author(s):  
D. Benfaremo ◽  
G. Stronati ◽  
L. Manfredi ◽  
L. Zuliani ◽  
A. Ferrarini ◽  
...  

Background:Systemic sclerosis (SSc) is a progressive autoimmune disease affecting the skin as well as internal organs, including the heart. A few studies have identified a subclinical heart involvement in patients with no pulmonary hypertension. Changes in myocardial deformation are consistent with the idea of SSc-related cardiomyopathy as a primary condition affecting the heart globally through microvascular dysfunction and subsequent myocardial fibrosis.Objectives:The aim of the present study is to describe the progression of myocardial deformation in patients with SSc and no overt cardiac disease.Methods:Prospective longitudinal study enrolling consecutive SSc patients referred to the Clinica Medica, University Hospital ‘Ospedali Riuniti’, Ancona, Italy, from February 2016 to December 2018. All patients fulfilled the 2013 ACR/EULAR classification criteria for SSc. Patients with structural heart disease, heart failure, atrial fibrillation or pulmonary hypertension were excluded. Disease subset, antibodies pattern, cardiovascular risk factors and involvement of other organ systems were recorded for each patient. An echocardiographic exam was performed for all patients at baseline and during their follow-up evaluation. Standard and speckle-tracking derived variables for the systolic and diastolic function of the left ventricle (LV) and right ventricle (RV) were acquired. Speckle tracking analysis software (EchoPAC 13.0; GE Medical Systems, Milwaukee, USA) was used to assess the GLS of the left and right ventricle, excluding the ventricular septum from right ventricular GLS calculations.Results:Seventy-two patients (68 females, age 56.6±15.4 years) were enrolled. Common echocardiographic parameters of left and right systolic function were within normal range at baseline and did not change during follow-up. Mean GLS, however, worsened for both left (from -19.8±3.5% to -18.7±3.5%, p=.034) and right ventricle (from -20.9±6.1% to -18.7±5.4%, p=.013) during a median follow-up of 20 months (1st-3rd quartile 12-24 months). The increased impairment registered in SSc patients was homogenous across endocardial layers (LV from -22.5±-3.9 to -21.4±3.9, p=.041; RV from -24.2±6.2 to -20.6±5.9, p=.001), mesocardial layers (LV -19.7±3.6 to -18.7±3.5, p=.043; RV from -21.3±5.9 to -18.8±5.7, p=.012) and epicardial layers (LV from -17.1±3.0 to -16.4±3.1, p=.112, RV -18.8±6.3 to -16.0±8.4, p=.035), as well as myocardial segments. No difference in progression rate was observed stratifying patients according to disease subset or other clinical parameters.Conclusion:GLS impairment progressed over a 20-month follow-up period in a cohort of SSc patients without clinically overt cardiac involvement. Further studies are needed to assess the significance of subclinical heart involvement and its progression in patients with SSc.Disclosure of Interests:None declared


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.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
G Stronati ◽  
F Guerra ◽  
L Zuliani ◽  
L Manfredi ◽  
A Ferrarini ◽  
...  

Abstract Background Systemic sclerosis (SSc) is a progressive autoimmune disease which has been proven to affect the heart. While it is widely known that the disease can cause pulmonary artery hypertension and therefore secondary right heart impairment, new studies have detected a subclinical heart involvement of both the left and right ventricles. The similar changes in myocardial deformation of both chambers assessed by speckle tracking imaging are consistent with the definition of SSc-related cardiomyopathy as a standalone entity with peculiar characteristics. Purpose The aim of the present study is to describe the progression of myocardial deformation as assessed through speckle tracking in patients with SSc and no pulmonary hypertension. Methods Prospective, longitudinal study on 48 patients affected by SSc. Patients with a history of heart failure, known structural heart disease, atrial fibrillation, and pulmonary hypertension were excluded. For every patient, standard echocardiographical and speckle-tracking derived variables for the systolic and diastolic function of the left ventricle (LV) and right ventricle (RV) were acquired at baseline and after 15±6 months. Results While common parameters of systolic function (Simpons's left ventricular ejection fraction, right ventricular fractional area change, TAPSE, tricuspidal S') did not change during follow-up, mean global longitudinal strain (GLS) significantly worsened for both left (from −19.1%±4.2% to −17.2%±5.0%) and right ventricle (from −17.9%±5.2% to −15.9%±4.8%) over 15 months. The increased impairment seen in SSc patients was homogeneous across endocardial layers (LV: from −21.8%±4.8% to −18.8%±5.2%; RV: from −20.6%±4.5% to −19.4%±4.9%), midventricular layers (LV: from −19.2%±4.5% to −17.7%±4.9%; RV: from −17.7%±4.7% to −16.7%±4.6%) and epicardial layers (LV: from −16.3%±4.7% to −16.0%±4.3%; RV: from −15.4%±5.0% to −14.6%±4.1%), as well as across myocardial segments (Figure 1). No difference in progression rate was seen between the diffuse and limited version of SSc, nor between different serotypes. Figure 1 Conclusions While traditional echocardiographical parameters are useless in order to follow the natural history of SSc-related cardiomyopathy during its early stages, GLS impairment progresses during a 15-month follow-up and involves similarly both the left and right ventricle. Whether, how, and how much the altered myocardial deformation contributes to the incidence of pulmonary arterial hypertension in these patients is still to be assessed. Acknowledgement/Funding Marche Polytechnic University


Reumatismo ◽  
2020 ◽  
Vol 72 (1) ◽  
pp. 44-51
Author(s):  
A. Javinani ◽  
S. Mostafaei ◽  
F. Gharibdoost ◽  
A.R. Jamshidi ◽  
R. Atef Yekta ◽  
...  

Systemic sclerosis (SSc) is a collagen-vascular disorder characterized by fibrosis and vasculopathy. Delta finger to palm distance (delta FTP) is an index measuring the distance between the tip of the third finger to the distal palmar crease in the flexed and extended position. The present study aimed to evaluate the clinical value of delta FTP and to assess the correlation of delta FTP with modified Rodnan skin score (mRSS) and forced vital capacity (FVC) over the 12-month follow-up. This prospective longitudinal study began with 50 participants who were followed for twelve months. Lowess smoothing and linear regression were applied to detect and assess the relationship between delta FTP and mRSS. p-values were adjusted by the Benjamini-Hochberg method (BHM) as a control for false discovery rate. Delta FTP was lower among patients with higher disease duration (p-valueadj: 0.008), diffuse cutaneous SSc (p-valueadj: 0.006), digital ulcers (p-valueadj: 0.003), telangiectasia (p-valueadj: 0.006) and dysphagia (p-valueadj: 0.036). The mRSS has a significant negative linear effect on the delta FTP at the baseline and the end of the follow-up (r: -0.31 and -0.40, respectively). Moreover, changes of mRSS and delta FTP showed a negative linear association over time (r: -0.22). These linear effects remained significant after regrouping the patients based on their SSc subtype. Delta FTP and FVC were not correlated either at the baseline or at the end. It seems that the delta FTP can be a valuable clinical index, supported by its correlated changes with mRSS and other SSc clinical manifestations over the one-year follow-up.


2002 ◽  
pp. 59-63 ◽  
Author(s):  
CW le Roux ◽  
PJ Jenkins ◽  
SL Chew ◽  
C Camacho-Hubner ◽  
AB Grossman ◽  
...  

OBJECTIVE: Epidemiological studies have shown an increased risk for prostate carcinoma in men with serum IGF-I in the upper part of the age-related reference range. Recombinant human GH (rhGH) is widely used in patients with GH deficiency, usually raising the serum IGF-I levels into the normal range: safety surveillance is therefore mandatory, with particular regard to neoplasia. The aim was to examine whether rhGH replacement in hypopituitary adults is associated with changes in serum prostate-specific antigen (PSA) as a surrogate marker of changes in prostatic growth. DESIGN AND METHODS: A prospective longitudinal study was used with a median follow-up of 22 (range 2.5-32) months, in which 41 men aged over 50 years with adult onset hypopituitarism and GH deficiency during rhGH replacement were examined. Serum PSA and IGF-I were measured at baseline and at latest follow-up. RESULTS: Mean serum PSA remained unchanged during rhGH replacement, with a median follow-up of 2 years. No correlation was found between the individual changes in serum IGF-I and changes in serum PSA. CONCLUSIONS: These data are reassuring thus far regarding the safety of GH replacement in relation to the prostate in this patient group.


2021 ◽  
pp. jrheum.201005
Author(s):  
Reto D. Kurmann ◽  
Edward A. El-Am ◽  
Yasser A. Radwan ◽  
Avneek S. Sandhu ◽  
Cynthia S. Crowson ◽  
...  

Objective Cardiac involvement is a poor prognostic marker in systemic sclerosis (SSc). While diastolic dysfunction, myocardial fibrosis, and arrhythmias are traditionally considered features of primary cardiac involvement in SSc, the incidence of valvular heart disease (VHD) is not well reported. Our objective was to examine the prevalence of VHD at time of SSc diagnosis and incidence of VHD during follow up compared to non-SSc subjects. Methods Medical records of patients with suspicion of SSc were reviewed to identify incident cases. SSc subjects were matched 1:2 by age- and sex to non-SSc subjects. Results The study included 78 incident SSc cases and 156 non-SSc comparators [56 years (± 15.7), 91% female]. A nearly 4-fold increase in the prevalence of moderate/severe VHD prior to SSc diagnosis compared to non-SSc subjects (6% vs. 0%; P=0.004) was identified. During follow up, 18 SSc and 12 non-SSc patients developed moderate/severe VHD. The cumulative incidence of VHD at 10 years after SSc incidence/index was 17.9% (95% CI: 10.7-29.9%) in patients with SSc compared with 2.3% (95% CI: 0.7-6.3%) in non-SSc subjects (HR: 4.23; 95% CI: 2.03-8.83). Coronary heart disease was the only significant risk factor for VHD. Conclusion SSc patients have a 4-fold increase in the prevalence of moderate/severe VHD at diagnosis compared to non-SSc patients. They also have a 4-fold increased risk of developing moderate/severe VHD after diagnosis of SSc. Aortic stenosis and mitral regurgitation have a much higher prevalence in SSc patients, besides secondary tricuspid regurgitation. Underlying mechanisms for this association require further elucidation.


PLoS ONE ◽  
2020 ◽  
Vol 15 (12) ◽  
pp. e0243651
Author(s):  
Ivan Castellví ◽  
Carmen Pilar Simeón ◽  
Monica Sarmiento ◽  
Jordi Casademont ◽  
Hèctor Corominas ◽  
...  

Systemic sclerosis is a disease where microcirculation damage is critical in their beginning and vascular complications have similar pathogenic findings. Digital ulcers are a frequent complication in systemic sclerosis patients and pulmonary hypertension is one of the leading causes of death. The use of bosentan has been shown to be useful for the treatment of pulmonary arterial hypertension and to prevent new digital ulcers. However, is unknown if bosentan can prevent pulmonary hypertension. Our objective was to determine if bosentan is useful to prevent pulmonary hypertension in SSc patients. A retrospective study in 237 systemic sclerosis patients with digital ulcers history treated or not with bosentan to prevent it was made. We analyzed the occurrence of pulmonary hypertension defined by an echocardiogram pulmonary arterial pressure > 40 mmHg in the entire cohort. Demographic, clinical, and treatment variables were recorded for all patients. Statistical significance was denoted by p values < 0.05. Fifty-nine patients were treated with bosentan a median of 34 months. 13.8% of treated patients had pulmonary hypertension vs 23.7% of untreated patients (p 0.13) during the follow up. In multivariate analysis patients not treated with bosentan had 3.9fold-increased risk of pulmonary hypertension compared with patients under bosentan treatment (p < 0.02). Moreover the percentage carbon monoxide diffusing capacity (DLCO) in bosentan treated patients did not decrease from baseline to the end of follow-up (61.8±14% vs 57±20.1%, p = 0.89). We concluded that Systemic sclerosis patients with digital ulcers treated with bosentan seems to have less risk to develop pulmonary hypertension and to stabilize DLCO


2021 ◽  
pp. 088626052110374
Author(s):  
Reeve S. Kennedy ◽  
Sarah A. Font ◽  
Ann-Christin Haag ◽  
Jennie G. Noll

Females exposed to child sexual abuse (CSA) are at an increased risk of experiencing further victimization in adolescence. Associations between CSA and several forms of cyber and in-person peer bullying victimization were assessed in a prospective, longitudinal study. Females exposed to substantiated CSA and a matched comparison group (N = 422) were followed over a two-year period. Bullying experiences were assessed in both survey and qualitative interviews. Qualitative data were coded and used to describe the types (e.g., cyber, physical, verbal), and foci (e.g., threats, physical appearance) of bullying victimization. Logistic regression was used to assess the odds that CSA was associated with subsequent bullying victimization, adjusted for demographics, social networking use, and prior bullying. CSA-exposed females were at an increased risk of multiple forms of bullying victimization with a persistent risk of bullying victimization over time. Specifically, they had 2.6 times higher odds of experiencing any bullying at follow-up, 2.9 times higher odds of experiencing cyberbullying at follow-up, and 2 times higher odds of experiencing combined cyber/in-person bullying at follow-up. CSA-exposed females were more likely than comparison females to experience bullying regarding their appearance/weight and dating relationships. Findings provide further insight into the unique circumstances of the cyberbullying and in-person bullying experienced by CSA-exposed females. Females exposed to child sexual abuse (CSA) are at an increased risk of experiencing bullying victimization, specifically cyberbullying and combined cyber/in-person bullying, as well as bullying about their appearance and dating relationships. These findings indicate that bullying prevention needs to include trauma-focused components to target these uniquely vulnerable females.


2021 ◽  
Author(s):  
Marcos Clavero-Adell ◽  
Daniel Palanca-Arias ◽  
Marta López-Ramón ◽  
Lorenzo Jiménez-Montañés ◽  
Itziar Serrano-Viñuales ◽  
...  

Abstract Purpose: Bronchopulmonary dysplasia is still a main problem in preterm infants. The screening of secondary right ventricle (RV) failure concern neonatologist and pediatric cardiologists. Measurements of right ventricle deformation through speckle tracking analysis in echocardiography could help to early diagnosis.Methods: A prospective longitudinal study was carried out over 28 months at a tertiary care pediatric cardiology reference center. Under 32 weeks’ pre-term infants were eligible for the study. Twenty-eight days after birth, all enrolled patients were included in one group: no bronchopulmonary dysplasia (NO-BPD) or bronchopulmonary dysplasia (BPD). At 36 PMA, BPD patients were included in one group according to severity categorization (mild, moderate, severe). At three time points echocardiogram measurements were performed. Right ventricle strain was studied using speckle tracking analysis and it was compared with classical function parameters between groups and along time. Results: Fifty patients were enrolled in the study, 22 on NO-BPD group and 28 on BPD group (16 mild, 8 moderate, 4 severe). RV strain showed no statistical differences between groups. However, BDP group showed worse RV function than NO-BPD group, both in speckle tracking analysis and in classical parameters. During de follow-up, an improvement trend is shown in RV strain. Conclusions: RV longitudinal strain and strain rate derived by speckle tracking is feasible in preterm infants. Although it seems to be a good correlation between RV strain and BPD severity, authors cannot conclude it. More studies should be carried out to investigate the optimum echocardiographic screening model of RV dysfunction in BPD patients.


2018 ◽  
Vol 25 (15) ◽  
pp. 1598-1606 ◽  
Author(s):  
Federico Guerra ◽  
Giulia Stronati ◽  
Colomba Fischietti ◽  
Alessia Ferrarini ◽  
Lucia Zuliani ◽  
...  

Background Systemic sclerosis is characterised by progressive cutaneous and organ fibrosis. Among all organs, a subclinical heart involvement is difficult to detect through conventional imaging. Design We evaluated whether speckle tracking-derived global longitudinal strain could help detect early subclinical systolic dysfunction in systemic sclerosis patients without overt clinical involvement. Methods A case–control, single-centre study on 52 systemic sclerosis patients and 52 age and gender-matched controls. Patients with structural heart disease, heart failure, atrial fibrillation and pulmonary hypertension were excluded. For every patient, standard echocardiographic and speckle tracking-derived variables for the systolic and diastolic function of the left ventricle and right ventricle were acquired. Results Traditional parameters of left and right systolic function did not differ between systemic sclerosis patients and controls (all P = ns). Left and right ventricular global longitudinal strain was significantly impaired in patients with systemic sclerosis when compared to controls (–19.2% vs. –21.1%; P = 0.009 and –18.2% vs. –22.3%; P = 0.012, respectively). Systemic sclerosis patients had a 2.5-fold increased risk of subclinical left ventricular systolic impairment (odds ratio 2.5, 95% confidence interval 1.1–5.5; P = 0.027) and a 3.3-fold increased risk of subclinical right ventricular systolic impairment when compared to controls (odds ratio 3.3, 95% confidence interval 1.4–7.7; P = 0.004). Alterations in the myocardial deformation pattern of systemic sclerosis patients were homogeneous in the right ventricle and eccentric in the left ventricle. Conclusions While traditional echocardiographic parameters are ineffective in detecting subclinical systolic impairment, reduced global longitudinal strain is common in patients with systemic sclerosis and significant for both ventricles. Global longitudinal strain could become a low-cost, non-invasive and reliable tool in order to detect early cardiac involvement in systemic sclerosis patients.


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