| Cardiac (Heart) Involvement |
| This page was written by Shelley Ensz, and has not yet been medically edited. See Disclaimer. |
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| Diagnosis of Systemic Scleroderma Heart Involvement |
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| Overview of Heart Assessment for Systemic Sclerosis (Scleroderma) |
| It is common for patients with systemic scleroderma to develop heart involvement even without any overt cardiac symptoms, so mandatory assessment of systemic scleroderma heart involvement requires a thorough baseline screening and follow-up monitoring. Diagnostic tests for scleroderma heart involvement may include CT scans, echocardiography, Holter monitoring, and MRI's. |
| Cardiac imaging techniques in systemic autoimmune diseases. Systemic autoimmune disorders are frequently associated with cardiac involvement and to a high prevalence of ischemic coronary events, often occurring at a younger age than in the normal population. PubMed. Lupus. 2005;14(9):727-31. |
| Screening for heart involvement is mandatory for all systemic scleroderma patients! | Assessment of Negative Prognostic Factors in Patients Affected by Systemic Sclerosis with Cardiac Involvement: A Retrospective Study. Baseline screening of heart functioning and appropriate follow-up monitoring is mandatory in all SSc patients. Furthermore, if low ejection fraction, inversion of E/A ratio, pulmonary hypertension and dangerous arrhythmias (IV - V Lown class) are present simultaneously at the baseline screening, they must be considered additive risk factors for shortening of survival due to cardiac involvement. A. Soldi. FRI0139 EULAR 2005. |
| Atherosclerosis in Patients With Autoimmune Disorders. Treatment of patients with autoimmune disease should also include CVD (cardiovascular disease) aspects and be focused on traditional risk factors as well as on disease-related factors. PubMed. Arterioscler Thromb Vasc Biol. 2005 Jun 23. |
| Assessment of heart involvement. Cardiac involvement frequently occurs in systemic sclerosis (SSc), contributing to the occurrence of symptoms, namely dyspnoea, fatigue, palpitations, and in some instances to the clinical evolution and prognosis of the disease. A thorough baseline screening of heart functioning and appropriate follow-up monitoring is therefore mandatory in all SSc patients. PubMed. Clin Exp Rheumatol. 2003;21(3 Suppl 29):S24-8. |
| Diagnostic tests for cardiac problems include Electrocardiogram (ECG) and Holter Monitor. (See Medical Tests) |
| Significance of the cardiovascular function tests in patients with collagen diseases, especially for SSc (systemic scleroderma). The cardiovascular function tests are useful to find cardiovascular abnormalities in these collagen diseases. PubMed. Rinsho Byori. 2004 May;52(5):401-5. (Also see: Sjogren's Syndrome) |
| CT Scans |
| Usefulness of technetium-99 m sestamibi myocardial perfusion SPECT in detection of cardiovascular involvement in patients with systemic lupus erythematosus or systemic sclerosis. Tc-99m sestamibi SPECT is a useful noninvasive imaging modality to detect cardiac involvement in symptomatic or asymptomatic SLE or SS patients. PubMed. Int J Cardiol. 2003 Dec;92(2-3):157-61. |
| Echocardiography (ECG) |
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| ECG (General) |
| Evaluation of Asymptomatic Changes in Left Ventricular Function in Scleroderma Patients Using Echocardiographic Strain Imaging. Besides being a cause of pulmonary hypertension, severity of pulmonary involvement may be a contributing factor to subtle ventricular abnormalities observed in scleroderma patients as strain values were found to correlate significantly with DLCO (carbon monoxide diffusion capacity). A. Kepez. FRI0364 EULAR 2006. (Also see: Pulmonary Hypertension ) |
| Lung lesion is a most common organ change in systemic scleroderma (SSD) detectable in approximately 70% of the patients at autopsy. This study has provided evidence that echocardiography is of high informative value in detecting pulmonary hypertension (PH) and right cardiac changes in patients with systemic scleroderma, which shows this technique to be a valuable screening in these cases. PubMed. Klin Med (Mosk). 2004;82(5):47-50. (Also see: Pulmonary Hypertension) |
| A Longitudinal Study of Estimated Right Ventricular Systolic Pressure (RVSP) by Doppler Echocardiography (ECHO) as a Predictor of Mortality in Systemic Sclerosis (Scleroderma). Any change in RVSP over time is associated with a small increase in risk of death. However, a greater than 20 mmHg increase in RVSP is associated with a substantial risk (6-fold) of death not explainable by the other clinical factors studied. Echocardiographic assessment of patients is a valuable, noninvasive tool that should be performed periodically to detect patients with an increased risk of death, likely secondary to pulmonary vascular disease. Sangeeta D. Sule. 1049/429. ACR 2004. |
| Blunted coronary flow reserve (CFR) in systemic sclerosis (SSc). CFR is often reduced in SSc patients. CFR was lower in patients with diffuse systemic sclerosis (dSSc) than in those affected by limited systemic sclerosis (lSSc). A reduced CFR value should be considered an indirect sign of heart involvement in scleroderma, but its clinical and prognostic implications need to be clarified. PubMed. Rheumatology (Oxford). 2004 Jan 20. |
| Reduced Coronary Flow Reserve In Systemic Sclerosis: A Sign Of Cardiac Involvement In Not-Symptomatic Patients. We tested the CFR estimation as an efficient, non-invasive and reliable tool to assess the cardiac involvement in systemic sclerosis. The reduced CFR value should be considered an indirect sign of scleroderma heart involvement, even if its clinical and prognostic implication needs to be clarified. Alberto Sulli. ACR Conference Oct. 2003. |
| Detection of early impairment of coronary flow reserve in patients with systemic sclerosis. This impairment in coronary microvasculature is detectable by a non-invasive echocardiographic method and in this study was more common in diffuse scleroderma. PubMed. Ann Rheum Dis. 2003 Sep;62(9): 890-3. |
| Tissue Doppler Analysis ECG |
| Tissue Doppler Echocardiography and Cardiopulmonary Exercise Tests: The New Insight Into Heart and Respiratory Failure in Systemic Sclerosis Patients. Fibrosing of connective tissue in SSc patients leads to left ventricle diastolic dysfunction and restrictive ventilatory disturbances. This is a cause of severe cardiopulmonary system failure and exercise intolerance. W. Plazak. SAT0227 EULAR 2006. (Also see: Pulmonary Involvement ) |
| Myocardial Systolic Dysfunction as Early Sign of Cardiac Involvement in Asymptomatic Patients with Systemic Sclerosis: A Strain and Strain Rate Tissue Doppler Analysis. Systemic sclerosis is characterized by an early impairment in systolic function at a time when fractional shortening, TAPSE and LV ejection fraction remain normal. This abnormality precedes the onset of symptoms and can be detected by Strain and SR but is not apparent by TD imaging. Therefore Strain analysis may represent a useful tool for the non-invasive follow-up of SSc patients, better selecting patients with early RV systolic impairment. S. Bellissimo. FRI0340 EULAR 2004. |
| Right Ventricular Function in Patients with Systemic Sclerosis: Relationship Between Skin Involvement and Pulsed Doppler Echocardiography Combined with DTI (Doppler Tissue Imaging) in Different Autoantibodies Subsets. Cardiac involvement in SSc is common and has a strong negative impact on the prognosis, especially when Scl-70 antibodies are present. DTI may represent a useful tool for non invasive follow-up of SSc patients for RV functional study, especially in Scl-70 subset autoantibodies. S. Stisi. FRI0334 EULAR 2004. |
| Reliable Detection of Myocardial Dysfunction by Tissue Doppler Echocardiography in Patients with Systemic Sclerosis. Patients with SSc have frequently reduced systolic and diastolic myocardial function evaluated by TDE despite normal radionuclide LVEF. TDE, a non-invasive new technology for cardiac evaluation, may be reliable in order to detect SSc cardiac involvement. Y. Allanore. FRI0309 EULAR 2004. |
| Noninvasive Assessment of Cardiac Structures and Function in Diffuse and Limited Systemic Sclerosis - Doppler Echocardiography Comparative Study. Patchy myocardial fibrosis (found in about 81% of scleroderma patients) leads to different type of abnormalities in cardiac structures and progressive myocardial dysfunction. Symptomatic cardiac involvement in patients, especially with diffuse systemic sclerosis, predicts a very poor prognosis. Echocardiography is absolutely the most important diagnostic and prognostic procedure for early detection of cardiac involvement in systemic sclerosis. B. N. Stamenkovic. FRI0117 EULAR 2003. |
| XL-ECG |
| XL-ECG: Evaluation of the Arrhythmic Risk in Systemic Sclerosis (SSc) Patients (Pts): Utility of Simultaneous Assessment of Electrocardiographic (ECG) Parameters. Standard ECG, ECG Holter and echocardiography cannot always identify cardiac involvement in early stages of the disease. XL-ECG estimates the susceptibility to develop life-threatening arrhythmias and can identify, more precociously than myocardial scintigraphy, a subset of SSc pts in a preclinic phase of heart involvement that must be strictly monitored. M. L. Conforti. FRI0096 EULAR 2003. |
| Heart Rate |
| Arrhythmias: Bradycardia and Tachycardia. A slow heart rhythm with a rate below 60 beats per minute at rest is called a bradycardia. Tachycardia is a fast heart rate of more than 100 beats per minute at rest. A fast or slow heart rate does not always mean your heart rhythm is abnormal. Fast or slow heart rates are also related to anxiety, activity, medications or other normal causes. Cleveland Clinic. |
| Panic attacks. Signs and symptoms may include rapid heart rate, sweating, trembling, shortness of breath and hyperventilation, chills, hot flashes, nausea, abdominal cramping, chest pain, headache, dizziness, faintness, tightness in your throat, trouble swallowing, a sense of impending death. Other health problems— such as an impending heart attack, an overactive thyroid gland (hyperthyroidism) or drug withdrawal— can cause similar signs and symptoms. From Mayo Clinic. CNN. (Also see: Lung Involvement, Heart Involvement and Thyroid Disease ) |
| MRI and CV-MRI |
| Distribution Of Myocardial Fibrosis In Scleroderma: A Delayed-Enhanced MRI Study. Delayed-enhanced MRI can detect myocardial fibrosis in a significant portion of SSc patients. The distribution of fibrosis is mostly basal- and mid-myocardial and may account for the arrhythmias detected in these patients. G.E. Tzelepis OP0163 EULAR 2007. |
| Early Detection of Right Ventricular Involvement by MRI in Systemic Sclerosis. SSc patients without cardiac symptoms may have a low RVEF (right ventricle ejection fraction) in comparison with healthy subjects. MRI might be a potential predictive method in evaluating early myocardial involvement in SSc, along with contrastographic Doppler Echocardiography as previously demonstrated. D. Rollando. FRI0065 EULAR 2005. |
| Non-invasive diagnostic and functional evaluation of cardiac and pulmonary involvement in systemic sclerosis (SSc). Pulmonary involvement occurs both in limited and in diffuse cutaneous SSc patients and develops, in 83% of the cases, without any regional lung perfusion abnormality. Cardiac involvement is detected in 65% of the cases. Lung perfusion MRI should be considered as a complementary diagnostic method for the functional evaluation of these symptoms in systemic sclerosis. PubMed. In Vivo. 2004 Mar-Apr;18(2):229-35. (Also see: Pulmonary Involvement) |
| Acute myocarditis associated with adenoviral infection in a patient with scleroderma. To our knowledge this is the first report of viral myocarditis in scleroderma. We propose that contrast-enhanced CV-MRI is valuable in a non-invasive diagnosis of heart disease in patients with scleroderma. PubMed. Clin Rheumatol. 2003 Dec;22(6):487-90. |
| CV-MRI: Contrast-Enhanced Cardiovascular Magnetic Resonance for Evaluating Heart Involvement in Scleroderma. Heart involvement in systemic scleroderma (SSc) is often clinically under-recognized and there is a discrepancy between the reported prevalence of symptomatic heart disease (20-25% of SSc patients (pts)) and the evidence of myocardial abnormalities (30-81% of cases) seen at autopsy. CV-MRI is a novel non-invasive, easily repeatable diagnostic tool capable to detect early morphologic and functional cardiac abnormalities in SSc pts. G. Pomponio. FRI0099 EULAR 2003. (Also see: Tests) |
| Holter Monitoring |
| Systemic sclerosis is not associated with clinical or ambulatory blood pressure. Raynaud's phenomenon is often the first symptom and occurs eventually in more than 95% of patients with systemic sclerosis (SSc). Systemic sclerosis is not associated with clinical blood pressure or the parameter of 24 h blood pressure monitoring. PubMed. Clin Exp Rheumatol 2003 Mar-Apr;21(2): 199-204. (Also see: Kidney Involvement) |
| Evaluation of the Arrhythmic Risk in Systemic Sclerosis (SSc) Patients (Pts): Utility of Simultaneous Assessment of Electrocardiographic (ECG) Parameters. Standard ECG, ECG Holter and echocardiography cannot always identify cardiac involvement in early stages of the disease. XL-ECG estimates the susceptibility to develop life-threatening arrhythmias and can identify, more precociously than myocardial scintigraphy, a subset of SSc pts in a preclinic phase of heart involvement that must be strictly monitored. M. L. Conforti. FRI0096 EULAR 2003. |
| 24-h ECG-Holter Monitoring - Myocardial Involvement in Systemic Sclerosis. Patients with SSc may have cardiac involvement, but no cardiac symptoms. 24 h ECG-Holter monitoring is a significant, noninvasive method for detecting early myocardial involvement in systemic sclerosis. B. N. Stamenkovic. FRI0118 EULAR 2003. |
| Scintigraphy |
| A case of scleroderma with congestive heart failure of which myocardial injury was detected by scintigraphy. Scintigraphy may be useful to detect an early cardiac involvement in patients with systemic sclerosis, and intense pharmacological treatment might be started even before the patient develops symptoms. PubMed. Nihon Rinsho Meneki Gakkai Kaishi. 2003 Jun;26(3):121-6. (Also see: Medical Tests: Cardiac) |
