Myocardial and vascular dysfunction in systemic sclerosis: The potential role of noninvasive assessment in asymptomatic patients

doi:10.1016/j.ijcard.2006.08.119

International Journal of Cardiology

Volume 121, Issue 3, 18 October 2007, Pages 298-301

https://doi.org/10.1016/j.ijcard.2006.08.119 Get rights and content

Abstract

Systemic sclerosis (SSc) is a multi-system disorder characterized by widespread vascular lesions and fibrosis of skin and distinct internal organs. The aim of the present study was to analyze possible associations of left ventricular (LV) myocardial function with coronary flow reserve (CFR) and endothelial function in asymptomatic patients with SSc. Thirty healthy subjects and 33 age- and sex-comparable asymptomatic SSc patients underwent standard Doppler Myocardial Imaging, Strain Rate (SR) Imaging of interventricular septum (IVS) and LV lateral wall, transthoracic CFR of left anterior descending coronary vessel, and brachial artery vasodilatation measurement. In SSc patients, LV myocardial early diastolic peak velocity, peak systolic SR and strain were both reduced in basal and middle IVS, and in basal and middle LV lateral wall (p < 0.001). In addition, both CFR (p < 0.0001) and endothelial flow-mediated dilatation (p < 0.001) were significantly lower in SSc patients. By stepwise forward multivariate analyses, CFR (p < 0.001) and endothelial function (p < 0.001) were powerful independent determinants of middle LV strain of SSc patients. In conclusion, SR Imaging, transthoracic CFR and brachial artery flow-mediated dilatation are valuable non-invasive and easy-repeatable tools for detecting early LV myocardial and vascular involvement caused by SSc.

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Systemic sclerosis and macrovascular involvement: Status of the issue in 2019
2019, JMV-Journal de Medecine Vasculaire
La Sclérodermie Systémique (ScS) est une maladie auto-immune rare responsable d’une fibrose cutanée et viscérale. Alors que la microangiopathie de la ScS est bien connue, certains patients présentent des complications macrovasculaires graves, cérébrales, cardiaques, ou périphériques. À ce jour, l’existence d’une atteinte macrovasculaire spécifique de la ScS reste controversée. De nombreuses études ont cependant rapporté une augmentation de la prévalence de l’atteinte macrovasculaire chez les patients atteints de ScS par rapport à des témoins appariés sur les facteurs de risque cardiovasculaires. Les modalités d’évaluation de l’atteinte macrovasculaire sont très variables selon les études : clinique, index de pression systolique, épaisseur intima media, imagerie, score calcique coronaire, mesure de la vitesse de l’onde de pouls, ou encore l’étude de la dilatation induite par le flux. À ce jour, la physiopathologie de l’atteinte macrovasculaire de la ScS reste inconnue et probablement multifactorielle : athérosclérose accélérée, dysfonction endothéliale dans le contexte dysimmunitaire, ou retentissement d’amont de l’atteinte microvasculaire oblitérante. L’objectif de ce travail est de réaliser une revue de la littérature la plus exhaustive possible, en étudiant les différentes topographies de l’atteinte macrovasculaire chez les patients atteints de ScS. Les résultats des principales études sont synthétisés sous forme de tableau selon le mode d’investigation utilisé.
Systemic sclerosis (SSc) is a rare immune disease leading to fibrosis of the skin and internal organs. Microvasculopathy is a hallmark of SSc. However, some patients have severe macrovascular complications as affecting cerebral, cardiac or peripheral vessels. To date, macrovascular involvement in SSc remains a matter of debate. Many studies have shown an increased prevalence of macrovascular involvement in SSc in comparison with controlled subjects with similar cardiovascular risk factors. Various methods were used: ankle brachial pressure index, intima media thickness, imagery, coronary calcium score, pulse wave velocity, or flow mediated dilation. The pathophysiology of macrovascular involvement remains unknown and is probably multifactorial: accelerated atherosclerosis, endothelial dysfunction, or reflected wave of microvessel obliteration. The aim of this study was to perform a comprehensible review of the literature, through the study of different types of involved vessels. Results of the main studies are summarized in tables according to the method of investigation used.
Chronic Inflammatory Disease Is an Independent Risk Factor for Coronary Flow Velocity Reserve Impairment Unrelated to the Processes of Coronary Artery Calcium Deposition
2016, Journal of the American Society of Echocardiography
Citation Excerpt :
These results suggest that CID may have specific pathophysiologic pathways responsible for the development of CMD unrelated to the classical pathways that lead to coronary atherosclerosis and calcification. There is accumulating evidence that patients with various CIDs present a high prevalence of CMD.4-7,15,18 Montisci et al.5 demonstrated for the first time that CFVR assessed by TTDE was impaired in patients with SSc, even in the absence of clinical signs of cardiac disease.
Chronic inflammatory disease (CID) is a complex multisystem disease characterized by chronic inflammation, which can lead to coronary microvascular dysfunction (CMD) and can also predispose to coronary artery calcium deposition, even in the absence of obstructive coronary artery disease.
Twenty-one patients with systemic lupus erythematosus (SLE; mean age, 60 ± 11 years), 21 patients with systemic sclerosis (SSc; mean age, 66 ± 11 years), 32 patients with rheumatoid arthritis (RA; mean age, 65 ± 9 years), and 23 control subjects with comparable traditional risk factors for coronary artery disease (mean age, 65 ± 10 years) were prospectively enrolled in the outpatient clinic. All study participants underwent transthoracic Doppler-derived echocardiography for coronary flow velocity reserve (CFVR) measurement in the left anterior descending coronary artery; CFVR < 2.5 defined CMD. Coronary artery calcium score in the left anterior descending coronary artery was also assessed by computed tomography.
None of study participants had obstructive coronary artery disease. The prevalence of CMD was 26% in the control group, 67% in the SLE group, 76% in the SSc group, and 63% in the RA group (P < .05, CID groups vs control group). CFVR was significantly lower in all three CID groups than in the control group (control group, 3.01 ± 0.72; SLE group, 2.23 ± 0.71; SSc group, 2.14 ± 0.54; RA group, 2.33 ± 0.62; P < .05, CID groups vs control group). In contrast, coronary artery calcium scores were similar in the four groups and had no relation to CMD. The odds ratios for CMD in patients with SLE, SSc, and RA were 16.70, 25.78, and 8.44 (P < .05) after adjusting for age, body mass index, the presence or absence of anemia, and hemoglobin level. Multiple linear regression analysis showed that only the presence of CID was independently associated with reduced CFVR among all study participants.
CID strongly contributes to CMD identified by qualitative evaluation of CFVR independently of traditional coronary risk factors of atherosclerosis but does not predispose to coronary artery calcification.
Ischemic heart disease in systemic inflammatory diseases. An appraisal
2014, International Journal of Cardiology
Citation Excerpt :
Szűcs et al. [58] reported in SSc patients increased LDL, as well as homocysteine and C reactive protein levels, whereas other authors found normal [59] or low [60] lipid levels but significantly higher levels of lipoprotein(a) than in controls (Table 1). Finally, increased IMT and reduced brachial FMD (Table 2), despite comparable CV risk factors have been demonstrated in SSc patients compared to controls [61–63]. The impact of treatments on the relationship between SIDs and CV events is still debated since commonly used drugs have divergent effects on the atherosclerotic process (Table 3).
Systemic inflammatory diseases are inflammatory syndromes that are associated with increased cardiovascular morbidity and mortality. The link between inflammatory and cardiovascular diseases can be attributed to coexistence of classical risk factors and of inflammatory mechanisms activated in systemic inflammatory diseases and involving the immune system. Yet, clinical implications of these findings are not entirely clear and deeper knowledge and awareness of cardiac involvement in inflammatory diseases are necessary. The aims of this review are to summarize cardiac involvement in systemic inflammatory diseases and to identify areas where evidence is currently lacking that deserve further investigation in the future.
Tissue Doppler assessment of right ventricular function in female patients with limited form of systemic sclerosis
2013, Egyptian Heart Journal
Citation Excerpt :
MPI is considered to be an index of global ventricular function, including both systolic and diastolic function, with the advantage of being measured at the same site during the same cycle using TDI. D’Andrea et al.31 reported that RV MPI was elevated in 43% of their patients.32 The above results revealed that in patients with lcSSc without pulmonary hypertension, systolic and diastolic RV function was impaired and those RV dysfunction appear before any clinical sign or symptom.
Cardiac involvement in systemic sclerosis (SSc) is often clinically occult. The aim of this study was the evaluation of early subclinical right ventricular (RV) involvement in patients with limited form of systemic sclerosis by tissue Doppler.
Twenty female patients with limited cutaneous SSc and 20 control female subjects, matched for age were studied with transthoracic echocardiography and tissue Doppler imaging (TDI) to assess RV function. Patients with pulmonary hypertension, chronic renal failure, diabetes mellitus, hypertension, heart failure, left ventricular hypertrophy, ischemic or rheumatic heart disease were excluded.
Patients with limited form SSc had significant lower tricuspid annulus peak systolic velocities (S_T) (9.95 ± 1.78 vs. 11.05 ± 1.53 cm/s, p < 0.044), early (E_T) (9.65 ± 1.30 vs. 12.50 ± 1.23 cm/s, p < 0.0001), late (A_T) diastolic velocity (12.60 ± 2.01 vs. 18.15 ± 1.81 cm/s, p < 0.0001), and tricuspid annular plane systolic excursion (TAPSE) (23.05 ± 3.50 vs. 26.50 ± 1.90, p < 0.001) compared to controls. Myocardial performance index (MPI) of the RV was higher in SSc patients compared to controls (0.41 ± 0.05 vs. 0.30 ± 0.02, p < 0.0001). There were significant correlations between disease duration and S_T and RV MPI (r = −0.883, p < 0.0001; r = 0.686, p < 0.001, respectively).
Patients with limited form of SSc present with pulsed-tissue Doppler imaging indices indicative of right ventricle dysfunction, which had significant correlations with disease duration. Tissue Doppler is a valuable non-invasive tool for detecting RV myocardial involvement in patients with limited SSc.
Bosentan improves systemic sclerosis-related peripheral circulation insufficiency
2011, International Journal of Cardiology
Atherosclerosis and macrovascular involvement in systemic sclerosis: Myth or reality
2011, Autoimmunity Reviews
Citation Excerpt :
dcSSc patients had significantly lower CFR parameters compared with lcSSc [67]. Importantly, CFR in SSc patients was found to correlate to the extent and the severity of the left ventricular myocardial contractile dysfunction [63]. Nevertheless, it should be kept in mind that reduced CFR alone cannot differentiate vasospasm from anatomic arterial stenosis [12].
Systemic sclerosis (SSc) is a chronic disease of unknown etiology, characterized by enhanced fibrosis, and microvascular abnormalities. During the past several decades, the death rates due to cardiovascular disease or cerebrovascular disease in SSc patients substantially increased and are currently responsible for 20–30% of mortality. Various autoimmune rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus accelerate atherosclerosis. Although microvascular disease is a hallmark of SSc, an ongoing debate exists regarding the presence and extent of macrovascular diseases and the presence of accelerated atherosclerosis in SSc patients. Despite conflicting results as to intima-media thickness (IMT) in SSc patients, the most recent and largest study has found no difference in either plaque occurrence or IMT. Additionally, abnormal coronary flow reserve in SSc patients appears to be due to microvascular involvement rather than atherosclerosis of the epicardial coronary arteries. Angiographic findings as well as computed tomography studies have generated conflicting reports as to coronary atherosclerosis in SSc.
Herein, we review the current knowledge of macrovascular involvement and atherosclerosis in SSc. The differences between SSc and other autoimmune rheumatic diseases in the presence and extent of atherosclerosis need to be further investigated.

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Letter to the EditorMyocardial and vascular dysfunction in systemic sclerosis: The potential role of noninvasive assessment in asymptomatic patients

Abstract

J Card Fail

J Am Soc Echocardiogr

Am J Cardiol

J Am Soc Echocardiogr

Int J Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

Preliminary criteria for the classification of Systemic Sclerosis (Scleroderma)

Arthritis Rheum

Letter to the Editor
Myocardial and vascular dysfunction in systemic sclerosis: The potential role of noninvasive assessment in asymptomatic patients