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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
Overview of Heart Assessment
CT Scans
Echocardiography
--ECHO (General)
--Tissue Doppler
Electrocardiogram
Heart Rate

MRI and CV-MRI
Holter Monitoring
Scintigraphy
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 electrocardiograms, echocardiography, Holter monitoring, CT scans, and MRI's.
N-terminal brain natriuretic peptide (NT-proBNP) in systemic sclerosis: a new cornerstone of cardiovascular assessment? NT-proBNP reliably detected the presence of cardiac involvement and appears as a very useful marker to risk stratify patients presenting with SSc. Y. Allanore. Annals of Rheumatic Diseases., BMJ 3 December 2008.
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
Computerized tomography—also called CT, CT scan and CAT scan—is an X-ray technique that produces more detailed images of your internal organs than do conventional X-ray exams. Mayo Clinic.
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 (ECHO)
ECHO (General) Tissue Doppler Analysis
ECHO (General)
Echocardiogram. An echocardiogram (also called an echo) is a type of ultrasound test that uses high-pitched sound waves that are sent through a device called a transducer. The device picks up echoes of the sound waves as they bounce off the different parts of your heart. These echoes are turned into moving pictures of your heart that can be seen on a video screen. WebMD.
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)
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.
Tissue Doppler Analysis ECHO
Tissue Doppler ECHO. This test is used to look at how blood flows through the heart chambers, heart valves, and blood vessels. WebMD.
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.
Electrocardiogram (ECG or EKG)
Electrocardiogram. An electrocardiogram (EKG or ECG) is a test that checks for problems with the electrical activity of your heart. WebMD.
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
Problem with Heart Rate can be determined with ECG/EKGs and Holter Monitors.
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. Panic attacks were once dismissed as nerves or stress, but they're now recognized as a real medical condition. 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. Mayo Clinic. (Also see: Lung Involvement, Heart Involvement and Thyroid Disease)
Pulmonary hypertension in systemic sclerosis determines cardiac autonomic dysfunction assessed by heart rate turbulence (HRT). HRT assessment indicates a frequent impairment of cardiac autonomic nervous system in SSc patients. Moreover, older age in these patients and especially PH are independent significant predictors of cardiac autonomic dysfunction. Bienias P. (PubMed) Int J Cardiol. 2008 Dec 19. (Also see: Pulmonary Hypertension)
MRI and CV-MRI
Ventricular mass index (VMI) correlates with pulmonary artery pressure and predicts survival in suspected systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH). The VMI, as demonstrated by magnetic resonant imaging (MRI), correlates well with mean pulmonary artery pressure in patients with SSc and may have a role in non-invasively excluding clinically significant PAH in breathless SSc patients in whom echocardiographic screening has failed. Dan Hagger. Rheumatology. July 14, 2009. (Also see: PAH)
Cardiac magnetic resonance imaging (MRI) in systemic sclerosis (SSc): a cross-sectional observational study of 52 patients. MRI is a reliable and sensitive technique to diagnose heart involvement in SSc and to analyse its mechanisms, including its inflammatory, microvascular and fibrotic components. Compared to echocardiography, MRI appears to provide additional information by visualising myocardial fibrosis and inflammation. A-L Hachulla. Annals of Rheumatic Diseases. 3 December 2008.
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)
Holter Monitoring
What Are Holter and Event Monitors? Holter and event monitors are medical devices that record the heart's electrical activity. They are similar to an EKG, both instead of recording the heart's electrical activity for a few second, they record the electrical activity over several days. National Heart Blood and Lung Institute.
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)
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)
 
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