Cardiac Involvement In Systemic Sclerosis Research Articles

Assessment of cardiac arrhythmias, P wave and QT dispersion in systemic sclerosis

Cardiac involvement in systemic sclerosis (SSc) is significant cause of SSc-related mortality. The objective of the study was to assess cardiac arrhythmias, P wave and QT dispersion in SSc patients. 40 SSc patients and 40 healthy participants who had similar sociodemographic characteristics with the patients were enrolled in the study. P wave dispersion (Pd), QT dispersion (QTd), and corrected QT (QTc) dispersion (QTcd) were calculated by measuring maximum (max) and minimum (min) of P wave, QT interval, QTc in 12-leads electrocardiography (ECG). Cardiac arrhythmias and conduction disorders were assessed by ECG and Holter-ECG. The mean age of SSc patients (92.5% of females) was 50.9±13.9 years similar with the healthy group (51.4±8.9). Abnormal ECG findings were found in 27.5% of SSc patients and remarkably higher in comparison to the healthy participants (p=0.019). The most frequently reported abnormal ECG findings were left anterior fascicular block (15%), ventricular premature beat (10%) and first-degree atrioventricular heart block (5%). The comparison of dispersion showed no statistically important difference in Pd between two groups (p=0.69) while QTd, QTc, QTcd, QTc min, and QTc max were markedly prolonged in patients with SSc (p=0.039; p<0.001; p=0.021; p<0.001; p<0.001, respectively). The assessment of Holter-ECG demonstrated that supraventricular tachycardia was frequently detected in patients with SSc (22.5% vs 2.5%; p=0.007). This study indicated a significantly elevated incidence of abnormal ECG results and SVT, an arrhythmia not typically identifiable through standard ECG but detectable via Holter monitoring, in patients with SSc. In the study, QTd, QTc, and QTcd intervals were significantly longer in SSc patients, which may indicate a susceptibility to arrhythmias.

Digital Ulcers and Ventricular Arrhythmias as Red Flags to Predict Replacement Myocardial Fibrosis in Systemic Sclerosis.

Cardiac involvement in systemic sclerosis (SSc) affects the prognosis of the disease. Echocardiography is the first line imaging tool to detect cardiac involvement, but it is not able to routinely detect myocardial fibrosis. Late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) is the gold standard for replacement myocardial fibrosis assessment, but its availability is currently limited. We aimed to assess the clinical and instrumental parameters that would be useful for predicting the presence of LGE-CMR, to achieve a better selection of patients with SSc that could benefit from third-level CMR imaging. 344 SSc patients underwent a comprehensive echocardiogram and LGE-CMR on the same day; for 189 patients, a 24 h ECG Holter monitoring was available. CMR showed non-junctional replacement myocardial fibrosis via LGE in 25.1% patients. A history of digital ulcers (OR 2.188; 95% C.I. 1.069-4.481) and ventricular arrhythmias at ECG Holter monitoring (OR 3.086; 95% C.I. 1.191-7.998) were independent predictors of replacement myocardial fibrosis. CMR can detect patterns of clinical and subclinical cardiac involvement, which are frequent in SSc. A history of digital ulcers and evidence of ventricular arrhythmias at ECG Holter monitoring are red flags for the presence of replacement myocardial fibrosis in CMR. The association between digital ulcers and myocardial fibrosis suggests that a similar pathological substrate of abnormal vascular function may underlie peripheral vascular and cardiac complications.

Epicardial adipose tissue in patients with systemic sclerosis

Abstract Aims Epicardial adipose tissue (EAT) has emerged as a mediator between systemic inflammatory disorders and cardiovascular disease, and may therefore play a role in the pathophysiology of cardiac involvement in systemic sclerosis (SSc). The aim of this study was to assess the correlation between EAT and left ventricular (LV) function, and to determine the prognostic value of EAT in patients with SSc. Methods and results Consecutive patients with SSc who underwent non-contrast thorax computed tomography and echocardiography were included. EAT mass was quantified using dedicated software. The study endpoint was all-cause mortality. A total of 230 SSc patients (age 53 ± 15 years, 14% male) were included. The median value of EAT mass was 67 g (interquartile range: 45–101 g). Patients with increased EAT mass (≥67 g) showed more impaired LV diastolic function as compared with patients with less EAT mass (<67 g), and even after adjusting for age and comorbidities, EAT mass was independently associated with LV diastolic function parameters. During a median follow-up of 8 years, 42 deaths occurred. Kaplan–Meier analysis showed that patients with increased EAT mass had higher all-cause mortality rate as compared with patients with less EAT mass (29% vs. 7%; P < 0.001). In the multivariable analysis, EAT was independently associated with all-cause mortality after adjusting for important covariates (HR: 1.006; 95% CI: 1.001–1.010). Conclusion In patients with SSc, EAT is independently associated with LV diastolic dysfunction and higher mortality rate.

Serum markers in systemic sclerosis with cardiac involvement.

Cardiac involvement in systemic sclerosis is a common clinical entity that may range from subclinical to life-threatening complications. The classification of cardiac involvement may be expressed as either primary or secondary involvement. Primary systemic sclerosis heart involvement (SSc-pHI) refers to cardiac pathologies primarily ascribed to systemic sclerosis rather than concomitant conditions like ischemic heart disease and pulmonary hypertension. The timely recognition of cardiac involvement holds significant clinical relevance. Therefore, numerous screening or diagnostic tools have been evaluated to forecast the likelihood of cardiac involvement, particularly in the absence of clinically evident cardiac symptoms. Of these modalities, serum biomarkers are often preferred due to their expeditiousness and non-invasive nature. Hence, the crucial goal of this narrative review is to review serum biomarkers that can be a valuable or promising tool in diagnosing cardiac involvement, especially SSc-pHI, in the early stages or predicting disease prognosis.

Cardiovascular Evaluation in Patients with Systemic Sclerosis in the Turkish Population

Cardiac involvement in systemic sclerosis (SSc) can lead to high morbidity and mortality. Therefore, early diagnosis and treatment are required. The aim of this study was to investigate the clinical, laboratory, and echocardiographic features of SSc, evaluate the proportion of patients with cardiovascular disease, and compare them with other population-based studies in the Turkish population. This study included 150 patients who were previously diagnosed and treated in the rheumatology clinic. Their age, sex, clinical signs and symptoms, laboratory and echocardiography findings, and concomitant diseases were evaluated. The results showed that the disease duration was <1 year and, at most, 11 years, with only one patient having elevated pulmonary artery pressure. In addition, patients with SSc in our region had similar demographic, clinical, laboratory, and echocardiographic features with those in other countries. This study demonstrated that hypertension is positively correlated with disease duration and the incidence of pulmonary hypertension is very low in patients with SSc.

Epicardial adipose tissue in patients with systemic sclerosis

Abstract Background Cardiac involvement is a major cause of death in patients with systemic sclerosis (SSc). Epicardial adipose tissue (EAT) has recently emerged as a mediator between systemic inflammatory disorders and cardiovascular disease, and may therefore play a role in the pathophysiology of cardiac involvement in SSc. Purpose To assess the correlation between EAT mass and left ventricular (LV) function, and to determine the prognostic value of EAT in patients with SSc. Methods Consecutive patients with SSc who underwent non-contrast thorax computed tomography and echocardiography were included. EAT mass was quantified using dedicated software (Figure A). The study endpoint was all-cause mortality. Results A total of 230 SSc patients [age 53±15 years, 14% male] were included. The median value of EAT mass was 67g (IQR: 45–101g). Patients with increased EAT mass (≥67g) showed specifically more impaired LV diastolic function as compared to patients with less EAT mass (<67g). After adjusting for age and comorbidities, EAT remained independently associated with LV diastolic function parameters including left atrial volume index (B=0.031, P=0.025), LV mass index (B=0.139, P=0.036), E/E$'$ (B=0.025, P<0.001) and E' (B=−0.012, P<0.001). During a median follow-up of 8 years 42 patients died, and by Kaplan-Meier analysis patients with increased EAT mass showed higher all-cause mortality rate as compared to patients with less EAT mass (log-rank p<0.001, Figure B). On multivariate analysis, EAT mass was independently associated with all-cause mortality (HR: 1.006; 95% CI: 1.001–1.010; p=0.010). Conclusion In patients with SSc, EAT mass is independently associated with LV diastolic dysfunction and higher mortality rate. Funding Acknowledgement Type of funding sources: None.

MODERATE PERICARDIAL EFFUSION WITH PULMONARY ARTERY HYPERTENSION IN A CASE OF SYSTEMIC SCLEROSIS: A CASE STUDY

A case of a 40 years old woman who was a known case of systemic sclerosis, presented with cardiac menifestations of moderate pericardial effusion with pulmonary hypertension. Cardiac involvement in systemic sclerosis is a poor prognostic factor and pulmonary arterial hypertension (PAH) is an important clinical complication and a leading cause of mortality in this disease. Although, there is no specic treatment for pericardial diseases in systemic sclerosis, but pericardial effusion is associated with PAH or renal crisis, once pericardial effusion is diagnosed, echocardiography is vital to evaluate whether the effusion is progressive, to assess the hemodynamic effects, and to monitor the response to anti-inammatory therapy in acute setting. Cautious pericardial drainage should be considered.

Serum Organ-Specific Anti-Heart and Anti-Intercalated Disk Autoantibodies as New Autoimmune Markers of Cardiac Involvement in Systemic Sclerosis: Frequency, Clinical and Prognostic Correlates.

Background: Heart involvement (HInv) in systemic sclerosis (SSc) may relate to myocarditis and is associated with poor prognosis. Serum anti-heart (AHA) and anti-intercalated disk autoantibodies (AIDA) are organ and disease-specific markers of isolated autoimmune myocarditis. We assessed frequencies, clinical correlates, and prognostic impacts of AHA and AIDA in SSc. Methods: The study included consecutive SSc patients (n = 116, aged 53 ± 13 years, 83.6% females, median disease duration 7 years) with clinically suspected heart involvement (symptoms, abnormal ECG, abnormal troponin I or natriuretic peptides, and abnormal echocardiography). All SSc patients underwent CMR. Serum AHA and AIDA were measured by indirect immunofluorescence in SSc and in control groups of non-inflammatory cardiac disease (NICD) (n = 160), ischemic heart failure (IHF) (n = 141), and normal blood donors (NBD) (n = 270). AHA and AIDA status in SSc was correlated with baseline clinical, diagnostic features, and outcome. Results: The frequency of AHA was higher in SSc (57/116, 49%, p < 0.00001) than in NICD (2/160, 1%), IHF (2/141, 1%), or NBD (7/270, 2.5%). The frequency of AIDA was higher (65/116, 56%, p < 0.00001) in SSc than in NICD (6/160, 3.75%), IHF (3/141, 2%), or NBD (1/270, 0.37%). AHAs were associated with interstitial lung disease (p = 0.04), history of chest pain (p = 0.026), abnormal troponin (p = 0.006), AIDA (p = 0.000), and current immunosuppression (p = 0.01). AHAs were associated with death (p = 0.02) and overall cardiac events during follow-up (p = 0.017). Conclusions: The high frequencies of AHA and AIDA suggest a high burden of underdiagnosed autoimmune HInv in SSc. In keeping with the negative prognostic impact of HInv in SSc, AHAs were associated with dismal prognosis.

Myocardial Involvement Detected Using Cardiac Magnetic Resonance Imaging in Patients with Systemic Sclerosis: A Prospective Observational Study.

Introduction and objectives: Cardiac involvement in systemic sclerosis (SSc) patients affects mortality. Cardiac magnetic resonance (CMR) is capable of detecting structural changes, including diffuse myocardial fibrosis that may develop over time. Our aim was to evaluate myocardial structure and function changes using CMR in patients with SSc without known cardiac disease during a 5-year follow-up and find possible correlations with selected biomarkers. Methods: A total of 25 patients underwent baseline and follow-up CMR examinations according to a pre-specified protocol. Standard biochemistry, five biomarkers (hsTnI, NT-proBNP, galectin-3, sST2, and GDF-15), and disease-specific functional parameters enabling the classification of disease severity were also measured. Results: After five years, no patient suffered from manifest heart disease. Mean extracellular volume (ECV) and T1 mapping values did not change significantly (p ≥ 0.073). However, individual increases in native T1 time and ECV correlated with increased galectin-3 serum levels (r = 0.56; p = 0.0050, and r = 0.71; p = 0.0001, respectively). The progression of skin involvement assessed using the Rodnan skin score and a decrease in the diffusing capacity of the lungs were associated with increased GDF-15 values (r = 0.63; p = 0.0009, and r = −0.51; p = 0.011, respectively). Conclusions: During the 5-year follow-up, there was no new onset of heart disease observed in patients with SSc. However, in some patients, CMR detected progression of sub-clinical myocardial fibrosis that significantly correlated with elevated galectin-3 levels. GDF-15 values were found to be associated with disease severity progression.

Abstract 14424: Association of Beta-1 Adrenergic Receptor Autoantibodies With Heart Failure Risk in Systemic Sclerosis

Introduction: Systemic sclerosis (SSc) is associated with the greatest independent risk of heart failure (HF) among systemic autoimmune diseases. Cardiac involvement in SSc also includes cardiomyopathy (CM), diastolic dysfunction (DD), and arrhythmias. Diffuse cutaneous SSc (dcSSc) is associated with greater cardiac risk than limited cutaneous SSc (lcSSc). Prior studies have examined the pathogenic roles of anti-beta-1 adrenergic receptor autoantibodies (β1AR-AAbs) and the protective role of the IgG3 subclass of β1AR-AAbs in HF. We aimed to investigate the associations between β1AR-AAb IgG/IgG3 seropositivity and disease phenotype in an SSc cohort. Methods: Serum samples were collected with informed consent from SSc patients with and without cardiac involvement, as approved by the Cleveland Clinic Institutional Review Board. Enzyme-linked immunosorbent assays were performed to investigate the presence of circulating IgG and IgG3 subtype β1AR-AAbs. Average optical density (OD) was compared between SSc and healthy control samples to establish seropositivity at the 2x control threshold level. Proportions of patients positive for β1AR-AAbs in different groups were compared using Fisher’s exact test. Results: Samples were collected from 36 SSc patients, of whom 20 (56%) had lcSSc and 15 (42%) had dcSSc. Cardiac involvement included HF, CM, DD, and arrhythmia, at least one of which was documented in 9 patients (25%). Of the total SSc cohort, 11 (31%) and 7 (19%) were positive for anti-β1AR IgG and IgG3 respectively. Between SSc patients with and without cardiac involvement, there was no significant difference in IgG or IgG3 positivity. There was a significant increase in IgG3 positivity in the lcSSc group compared to the dcSSc group at the 2x control threshold (p = 0.0125). Conclusions: High anti-β1AR IgG3 positivity is associated with less severe disease category (lcSSc vs. dcSSc) in our SSc cohort, supporting prior evidence of the protective role of the IgG3 subtype. A larger sample size will allow for further investigation of the associations between IgG/IgG3 β1AR-AAbs and cardiac involvement in SSc.

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

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&amp;lt;0.001) and hospitalization for CV causes (ARR 73%; p&amp;lt;0.001). Moreover, a diagnosis of HF was associated with an increased risk of death (ARR 50%; p&amp;lt;0.001) and hospitalization for CV causes (ARR 61%; p&amp;lt;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. Funding Acknowledgement Type of funding sources: None.

Prevalence and risk factors for left ventricular diastolic dysfunction in systemic sclerosis: a multi-center study of CRDC cohort in China.

Left ventricular diastolic dysfunction (LVDD) is a common manifestation of cardiac involvement in systemic sclerosis (SSc), which is associated with increased mortality, but little is known about the risk factors. The aim is to determine the frequency and potential predictors of SSc-LVDD. We conducted a prospective multi-center cohort study, enrolling 784 SSc patients assessed by echocardiography between April 2008 and June 2019. Diagnosis of systemic sclerosis was according to the 2013 American College of Rheumatology (ACR)/the European League Against Rheumatism (EULAR) classification criteria. Data were compared between patients with and without LVDD, while univariate and multivariate regression analysis was performed to determine the factors independently associated with LVDD. LV diastolic dysfunction was present in 246/784 (31.4%) of the subjects. There were no significant differences in gender, BMI, or disease duration between the two groups. Around 40% of the patients in the SSc-LVDD group and in the SSc-non LVDD group had diffused cutaneous involvements. Factors independently associated with LV diastolic dysfunction in multivariable analysis included age at onset (OR 1.053, 95%CI 1.021-1.086, p = 0.001), pulmonary arterial hypertension (OR 3.057, 95%CI 1.468-6.367, p = 0.003), positivity of anti-RNP antibody (OR 2.455, 95%CI 1.049-5.745, p = 0.038), increased WBC count (OR 1.156, 95%CI 1.037-1.287, p = 0.009), elevated levels of uric acid (OR 1.003, 95%CI 1.000-1.006, p = 0.036), and triglyceride (OR 1.515, 95%CI 1.106-2.077, p = 0.010). LV diastolic dysfunction was prevalent in the SSc population. Advanced onset age, PAH, positive anti-RNP antibody, increased WBC count, and adverse metabolic status were independent risk factors for SSc-related LVDD. Key Points • In this Chinese multi-center cohort of systemic sclerosis, LVDD is not a rare complication, with a prevalence of 31.4%. • The presence of advanced onset age, PAH, positive anti-RNP antibody, increased WBC count and adverse metabolic status were baseline predictors of developing LVDD in SSc.

Clinical Outcomes of Asymptomatic Cardiac Involvement in Systemic Sclerosis Patients After a 2-Year Follow-Up (Extended Study)

Asymptomatic cardiac involvement in systemic sclerosis (SSc) has been reported. Long-term follow-up might elucidate the clinical implications of these abnormalities. The aim was to identify the clinical outcomes of asymptomatic cardiac involvement in SSc patients after 2 years of follow-up. A cohort study was done at Khon Kaen University, Thailand, on adult patients with SSc who completed the preliminary study. Repeated investigations included electrocardiography, chest radiography, echocardiography, and blood tests for creatine kinase-MB, high sensitivity cardiac troponin-T, and N-terminal prohormone of brain natriuretic peptide. Seventy-four of the 103 patients from the previous study were enrolled. The mean duration of follow-up was 3.1±0.9 years. Five patients developed symptomatic cardiac involvement-all of whom had pulmonary arterial hypertension (PAH). The incidence of symptomatic cardiac involvement for the combined 315 person-years was 1.6 per 100-person-years (95%CI 0.7-3.4). Fourteen patients died resulting in a mortality incidence of 4.4 per 100-person-years (95%CI 4.3-5.4). Persistent cardiac involvement was found in 35 patients for an incidence of 11.1 per 100-person-years (95%CI 8.0-15.5). Two of the patients who had persistent elevated cardiac enzyme developed PAH at a respective 3.7 and 39.4 months after the initial evaluation. None of the clinical parameters were predictive of symptomatic and persistent cardiac involvement. Only male sex was associated with mortality (hazard ratio 3.70; 95%CI 1.22-11.11). Cardiac involvement in SSc can progress slowly or even be reversed. Based on a previous test, the incidence of symptomatic cardiac involvement after 2 years was low despite its being a persistent involvement. If symptomatic cardiac involvement develops, PAH is the most prevalent symptom.

QT Interval Prolongation in Patients with Systemic Sclerosis-Are the Holter ECG Recordings a Better Option for QT Interval Evaluation?

Background and Objectives: Cardiac involvement in systemic sclerosis has important consequences on patient survival. Myocardial fibrosis and microcirculation involvement can generate arrhythmic complications, which can be associated with a higher death risk. QT interval prolongation is considered as a risk factor for ectopic ventricular events and can be evaluated using standard short ECG recordings or 24-h Holter ECG recordings. Materials and Methods: 39 patients with systemic sclerosis were submitted to a standard ECG recording at admission and 24-h Holter ECG monitoring. Results: QT interval values resulted from Holter ECG monitoring are higher than the values generated by the short-term ECG recordings. Holter ECG monitoring permits the detection of ventricular ectopy in patients with no events on standard ECG. Conclusions: In patients with systemic sclerosis, 24-h Holter ECG recordings can realize a more precise evaluation of the extent of QTc interval prolongation and ventricular ectopic events associated with myocardial involvement.

Cardiac involvement in systemic sclerosis: Getting to the heart of the matter.

Primary systemic sclerosis heart involvement (pSHI) is an important disease manifestation that accounts for a significant proportion of systemic sclerosis (SSc)-associated mortality. A broad clinical spectrum of pSHI exists, which ranges from asymptomatic perfusion abnormalities to diastolic dysfunction or acute myocarditis and congestive heart failure. With improving sensitivity of cardiac investigations, it is increasingly recognized that there is a large burden of subclinical cardiac disease in patients with SSc. Early signs of pSHI can be subtle and determining the etiology of cardiac abnormalities from other causes of cardiomyopathy such as hypertension, ischemic heart disease (IHD), and pulmonary vascular disease remain challenging. Early identification of pSHI potentially provides clinicians with a window of opportunity for intervention to avert progression to heart failure. However, optimal screening and treatment guidelines are lacking, and it is an area of much needed further clinical research.

Increased Risk of Valvular Heart Disease in Systemic Sclerosis: An Underrecognized Cardiac Complication.

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 the time of SSc diagnosis and incidence of VHD during follow-up compared to non-SSc subjects. 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. The study included 78 incident SSc cases and 156 non-SSc comparators (56 yrs [± 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 0.7-7.0) in non-SSc subjects (HR 4.23, 95% CI 2.03-8.83). Coronary artery disease was the only significant risk factor for VHD. Patients with SSc 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 patients with SSc, besides secondary tricuspid regurgitation. Underlying mechanisms for this association require further elucidation.

Essential Hypertension Worsens Left Ventricular Contractility in Systemic Sclerosis.

Primary cardiac involvement in systemic sclerosis (SSc) is prevalent and morbid; however, the influence of traditional cardiovascular (CV) risk factors, such as essential hypertension (HTN), are unclear. In the present study, we sought to understand the effects of HTN on left ventricular (LV) contractility in patients with SSc using echocardiographic speckle-derived global longitudinal strain (GLS). Fifty-six SSc patients with HTN (SSc+HTN+) and 82 SSc patients without HTN (SSc+ HTN-) were compared with 40 non-SSc controls with HTN (SSc-HTN+) and 40 non-SSc controls without HTN (SSc-HTN-), matched by age and sex. All HTN patients were on stable antihypertensive therapies. Echocardiographic measures included LV (LV) ejection fraction (LVEF), left atrial volume index (LAVI), and LV diastolic function. LV contractility was assessed by GLS, averaged across the 18 LV segments. Patients with SSc had diminished GLS regardless of HTN status when compared to both control groups, despite normal LVEF (P < 0.001). SSc+HTN+ had the highest prevalence of diastolic dysfunction, with significantly higher septal E/e´, a marker of LV filling pressures (P < 0.05), as well as the largest reduction in GLS compared to SSc+HTN- and both control groups. Speckle-derived strain revealed diminished LV contractility in patients with SSc, despite normal LVEF. SSc+HTN+ had more prominent reductions in GLS associated with evidence of LV remodeling and worsened diastolic function. Our findings demonstrate the presence of subclinical LV contractile dysfunction in SSc that is further exacerbated by concomitant HTN, thereby identifying HTN as an important modifiable CV risk factor that should be managed aggressively in this at-risk population.

Significant and Early Cardiac Involvement in Systemic Sclerosis Detected by Cardiac Magnetic Resonance

Heart involvement in systemic sclerosis (SSc) is common and often diagnosed late in the disease course. It may involve the myocardium, pericardium and conducting system, though there is heterogeneity in presentation. We sought to characterise cardiac involvement in SSc using cardiac Magnetic Resonance imaging (CMR).

Early myocardial damage and microvascular dysfunction in asymptomatic patients with systemic sclerosis: A cardiovascular magnetic resonance study with cold pressor test.

PurposeCardiac involvement in Systemic Sclerosis (SSc) is increasingly recognized as a mayor cause of morbidity and mortality. The aim of present study is to investigate the early stages of cardiac involvement in SSc by Cardiovascular magnetic resonance (CMR), combining the non-invasive detection of myocardial inflammation and fibrosis using T2 and T1 mapping techniques and the assessment of microcirculatory impairment through perfusion response to cold pressor test (CPT).Methods40 SSc patients (30 females, mean age: 42.1 years) without cardiac symptoms and 10 controls underwent CMR at 1.5 T unit. CMR protocol included: native and contrast-enhanced T1 mapping, T2 mapping, T2-weighted, cineMR and late gadolinium enhancement (LGE) imaging. Microvascular function was evaluated by comparing myocardial blood flow (MBF) on perfusion imaging acquired at rest and after CPT. Native myocardial T1 and T2 relaxation times, extracellular volume fraction (ECV), T2 signal intensity ratio, biventricular volumes and LGE were assessed in each patient.ResultsSSc patients had significantly higher mean myocardial T1 (1029±32ms vs. 985±18ms, p<0.01), ECV (30.1±4.3% vs. 26.7±2.4%, p<0.05) and T2 (50.1±2.8ms vs. 47±1.5ms, p<0.01) values compared with controls. No significant differences were found between absolute MBF values at rest and after CPT; whereas lower MBF variation after CPT was observed in SSc patients (+33 ± 14% vs. +44 ± 12%, p<0.01). MBF variation had inverse correlation with native T1 values (r: -0.32, p<0.05), but not with ECV.ConclusionsMyocardial involvement in SSc at preclinical stage increases native T1, T2 and ECV values, reflecting inflammation and fibrosis, and reduces vasodilatory response to CPT, as expression of microvascular dysfunction.

Abstract 13566: Clinical Utility of Noninvasive Assessment of Flow-pressure Relationship in Scleroderma

Introduction: Cardiac involvement in Systemic sclerosis (SSc), especially left ventricular diastolic dysfunction (DD), is high prevalence and associated with high mortality. Thus, the early detection of left ventricular DD might be important to manage SSc. Recent study has shown that exercise stress test may evaluate an early stage of heart failure in patients with normal resting echocardiogram. Hypothesis: We hypothesized that SSc patients with normal resting hemodynamics may present early phase of left ventricular DD by exercise echocardiogram, leading to the model of predicting long-term outcomes. Methods: Between January 2014 and December 2018, we prospectively enrolled 140 SSc patients who underwent 6-minute walk (6MW) stress echocardiographic studies with normal range of estimated mean pulmonary arterial pressure (mPAP) (&lt;25mmHg) and mean pulmonary artery wedge pressure (mPAWP) (&lt;15mmHg) at rest. We used ΔmPAP/Δcardiac output (CO) to assess pulmonary vascular reserve and ΔmPAWP/ΔCO to assess left ventricular (LV) reserve between resting and post-6MW point. Results: During a median period of 3.6 years, 25 patients (18%) reached the composite outcome. Both ΔmPAP/ΔCO and ΔmPAWP/ΔCO in patients with events were significantly higher than in ones without events (8.9±3.8mmHg/l/min vs. 3.0±1.7mmHg/l/min; p=0.002, and 2.2±0.9mmHg/l/min vs. 0.9±0.5mmHg/l/min; p&lt;0.001, respectively). In addition, ΔmPAWP/ΔCO is strongly associated with events (p=0.04) in patients with normal ΔmPAP/ΔCO (≤median value), although ΔmPAWP/ΔCO had weak impact in patients with abnormal ΔmPAP/ΔCO (&gt;median value) (p=0.23). Conclusions: Exercise echocardiography revealed impaired LV functional reserve in SSc patients with normal resting hemodynamics. Furthermore, in the group of normal pulmonary vascular function, the patients with impaired LV functional reserve had significantly shorter event-free survival than the ones with non-impaired LV function. Thus, the evaluation of early left ventricular DD with 6MW may detect high-risk group in SSc patients.