| Cardiac (Heart) Involvement |
| This page was written by Shelley Ensz, and has not yet been medically edited. See Disclaimer. |
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| Systemic Scleroderma Cardiac (Heart) Involvement |
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| Overview of Systemic Scleroderma Cardiac (Heart) Involvement |
| The systemic forms of scleroderma can cause cardiac (heart) problems. One of the earliest symptoms reported by diffuse systemic scleroderma patients is heart irregularities. |
| Systemic scleroderma can cause arrhythmias, pericardial effusion, and heart failure. Formation of scar tissue in the heart and lungs has recently been recognized as a general pattern probably contributing to overall fatigue. |
| Evaluation of cardiac abnormalities by Doppler echocardiography in a large nationwide multicentric cohort of patients with systemic sclerosis (SSc). Left and right heart diseases, including pulmonary arterieal hypertension, Left Ventricle hypertrophy and diastolic dysfunction, are common in SSc. (PubMed) Ann Rheum Dis. 2008 Jan;67(1):31-6. |
| Clinical features of scleroderma patients with contracture of phalanges (CP). The study suggested that the presence of CP may be a marker of oesophageal involvement, pulmonary fibrosis and heart involvement. PubMed. Clin Rheumatol. 2006 Dec 15. (Also see: Muscle and Joint Involvement, Esophageal Involvement, and Pulmonary Fibrosis) |
| Relationship Between Lung and Heart Involvement and Cardiopulmonary Functional Status in Patients with Systemic Sclerosis (SSc). According to our results, presence of tricuspid pressure gradient and high right ventricle systolic pressure were the most important factors that predict worsening of cardiopulmonary functional status in patients with SSc. M. B. Zlatanovic. SAT0264 EULAR 2006. (Also see: Pulmonary Fibrosis) |
| Cardiopulmonary Manifestations in Systemic Sclerosis (SSc) - Correlation with Disease Duration and the Extent of Skin Involvement. Interstitial pulmonary fibrosis in patients with SSc causes restrictive lung disease and progresses to severe lung damage and immunoinflammatory heart damage leading to ventricular diastolic dysfunction. B. N. Stamenkovic. AB0291 EULAR 2006. (Also see: Pulmonary Fibrosis and Pulmonary Hypertension) |
| Raynaud's Phenomenon (RP), Systemic Sclerosis (SSc), and Macrovascular Peripheral Disease. In this sample of atherosclerotic patients, patients with RP were five times more prone to coronary disease, compared to patients without RP, which could suggest cardiac Raynaud's. SSc was more frequent than expected. J. F. J. Olmedo. AB0284 EULAR 2006. (Also see: Raynaud's) |
| Heart involvement and systemic sclerosis. Scleroderma heart involvement (SHI) is often manifest, and virtually always present when accurately searched and holds a significant prognostic value. Accurate cardiologic baseline screening and subsequent follow-up are mandatory in all patients. PubMed. Lupus. 2005;14(9):702-7. |
| The heart in systemic sclerosis. Cardiac involvement can be manifested by myocardial disease, conduction system abnormalities, arrhythmias, or pericardial disease. Additionally, scleroderma renal crisis and pulmonary hypertension lead to significant cardiac dysfunction secondary to damage in the kidney and lung. New cardiac technology shows significant frequencies of asymptomatic cardiac abnormalities. PubMed. Curr Rheumatol Rep. 2004 Apr;6(2):137-40. (Also see: Kidney Involvement and Lung Involvement) |
| The Heart in Systemic Sclerosis. Scleroderma heart disease is often manifest, and virtually always present, though silent, when accurately searched. Cardiac involvement in SSc, frequently linked to lung parenchimal and vascular disease, determines symptom occurrence (particularly dyspnoea, fatigue, palpitations, and chest pain, when pericardium is affected), and is responsible of patients clinical evolution and prognosis, in many instances. M. Emdin. SP0012 EULAR 2003. |
| Antiphospholipid Antibodies in Scleroderma (Systemic Sclerosis) |
| Antiphospholipid Antibodies in Systemic Sclerosis. Antiphospholid antibodies are frequently found in systemic scleroderma patients. They are correlated with pulmonary hypertension in scleroderma. ISN. |
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| Aortic Stiffness |
| Aortic Stiffness is a loss of elasticity in the aortic wall. Aortic stiffness is an important prognosticators of cardiovascular disease risk. |
| Increased Arterial Stiffness as the Marker of Vascular Involvement in Systemic Sclerosis. Augmentation index (AI) and pulse wave velocity (PWV) of the brachial artery were measured. PWV and AI are reproducible indicators of the presence and degree of arterial stiffening in patients with SSc and therefore, may be a useful diagnostic test in the assessment of arterial function. J Rheumatol First Release May 15 2008. |
| Ascending aortic aneurysm in a man with scleroderma. To the best of our knowledge, there is only one other report to date in the English language of a thoracic aortic aneurysm associated with scleroderma. PubMed. Clin Rheumatol. 2006 Mar 30. |
| Chronic coffee consumption has a detrimental effect on aortic stiffness and wave reflections. Chronic coffee consumption unfavorably affects arterial stiffness and wave reflections and suggests that studies that investigate arterial function should control for coffee intake. American Journal of Clinical Nutrition, Vol. 81, No. 6, 1307-1312, June 2005. |
| Aortic stiffness in systemic sclerosis is increased independently of the extent of skin involvement. Stiffness of the aorta is increased in patients with established SSc regardless of the extent of the inflammatory fibrotic process in the skin and lungs, suggesting that additional pathogenetic mechanisms contribute to the compromise of large arteries. PubMed. Rheumatology (Oxford). 2004 Nov 16. |
| Atherosclerosis |
| Atherosclerosis the process in which deposits of fatty substances, cholesterol, cellular waste products, calcium and other substances build up in the inner lining of an artery. This buildup is called plaque. American Heart Association. |
| Evaluation of Paraoxonase Activity in Patients with Mixed Connective Tissue Disease (MCTD). Our results indicate that in patients with MCTD there is an increased risk for atherosclerosis. J Rheumatol 2008;35:237-43. (Also see: MCTD) ( NOTE: The antioxidant paraoxonase (PON1) is an endogenous free radical scavenger in the human body.) |
| Antiphospholipid antibody syndrome and autoimmune diseases. Evidence is growing that antiphospholipid antibodies may have a pathogenic role in pulmonary hypertension and accelerated atherosclerosis of autoimmune diseases. (PubMed) Hematol Oncol Clin North Am. 2008 Feb; 22(1):53-65. (Also see: Antibodies, Lupus,and PH) |
| Early Atherosclerosis in Systemic Sclerosis (SSc) and Its Relation to Disease or Traditional Risk Factors. SSc is not associated with increased early signs of atherosclerosis. Martha E. Hettema. 1196/428. ACR 2007. |
| Coronary Atherosclerosis in Systemic Sclerosis. Coronary calcium, a surrogate marker for atherosclerosis, is found at significantly higher levels in systemic sclerosis (SSc) patients compared to age-, sex-, and race-matched controls. Levels of serum homocysteine are also significantly higher in SSc patients. Vandana Khurma. F9 ACR 2006. |
| Asymptomatic Pericardial Disease |
| Asymptomatic means without symptoms. Pericardial means heart, and the area around the heart. |
| Echocardiographically Guided Pericardiocentesis for Treatment of Clinically Significant Pericardial Effusion in Rheumatoid Arthritis (RA). Echocardiographically guided pericardiocentesis is a safe and effective treatment for this uncommon but serious complication of RA. J Rheumatol 2006;33:2173–7. (Also see: RA) |
| Heart Scan May Predict Hidden Heart Disease. Heart scans may be able to detect the beginning of atherosclerosis, which is the buildup of cholesterol on blood vessels to form a waxy plaque. A recent research study performed on 100 women using the heart scan revealed that 42 of the women had undiagnosed heart disease. About.com. |
| Asymptomatic Pericardial Disease in Scleroderma-Associated Progressive Interstitial lung disease (ILD) and its Relationship to Pulmonary Hypertension. Asymptomatic pericardial disease is common in patients with SSc-associated progressive ILD, and its presence is associated with a high risk of echocardiographically defined pulmonary hypertension. Aryeh Fischer. 372/372. ACR 2005. (Also see: Pulmonary Fibrosis, and Pulmonary Hypertension) |
| Cardiac Remodeling |
| Cardiac remodeling in patients with systemic sclerosis with no signs or symptoms of heart failure: an endomyocardial biopsy study. It is possible to identify cardiac remodeling, characterized by myocardial fibrosis deposits, particularly within the interstitium in SSc patients before any signs or symptoms of heart failure appear. PubMed. J Card Fail. 2003 Aug;9(4):311-7. |
| Carotid Artery Disease (CAD) |
| Carotid Artery Disease in Patients with Systemic Sclerosis. It seems that carotid artery disease is more common in patients with systemic sclerosis (SSc). V. B. Bosnjakovic. AB0189 EULAR 2005. |
| Cholesterol (Better in Systemic Scleroderma Patients) |
| On average, systemic scleroderma patients do not differ from healthy people, in terms of total body fat, despite the ravages of illness and frequent complications of gastrointestinal difficulties. Another unusual finding in the following study is that scleroderma patients had significantly better cholesterol profiles than healthy people. (Also see: Systemic Scleroderma) |
| Body Composition Analysis in Patients with Scleroderma. Total body fat and fat distribution seems not to be influenced in scleroderma patients when compared to healthy controls.However, high density lipoprotein (HDL) levels were significantly higher and low density lipoprotein (LDL) levels were significantly lower in the patient group. O. Gurler. AB0289 EULAR 2006. (Also see: Skeletal Involvement) |
| Conduction Problems |
| Cardiac arrhythmias and conduction disturbances in autoimmune rheumatic diseases. Rhythm and conduction disturbances and sudden cardiac death (SCD) are important manifestations of cardiac involvement in autoimmune rheumatic diseases. PubMed. Rheumatology (Oxford). 2006 Oct;45 Suppl 4:iv39-iv42. |
| Left Ventricular (LV) Diastolic Dysfunction |
| What Is Diastolic Dysfunction and Diastolic Heart Failure? An abnormal "stiffening" of the ventricles, and the resulting abnormal ventricular filling during diastole, is referred to as diastolic dysfunction. About.com.: Heart Disease. |
| Doppler Echocardiographic Evaluation in Mixed Connective Tissue Disease (MCTD). Signs of left ventricle diastolic function disorder were observed in patients with MCTD. Results suggest that global left ventricle function loss is the consequence of the disease itself and not of the treatment. J. Vegh. SAT0255 EULAR 2006. (Also see: MCTD) |
| Heart Involvement in Patients with Systemic Sclerosis: Tissue Doppler Echocardiography. Significant decrease of early diastolic velocities in SSc patients was found. The left ventricle systolic function in SSc patients is preserved. W. Plazak. AB0286 EULAR 2006. |
| Tissue Doppler echocardiography and myocardial performance index in patients with scleroderma. In scleroderma patients the global left ventricular functions were depressed and diastolic function abnormalities were seen in both right and left ventricles. In addition, longitudinal muscle functions of the ventricles were depressed in scleroderma patients, as shown by tissue Doppler imaging parameters. PubMed. J Int Med Res. 2005 Jul-Aug;33(4):417-24. |
| Echocardiographic alterations in systemic sclerosis: A longitudinal study. Our data confirm the significant prevalence of LV (left ventricular) diastolic dysfunction in SSc patients and the role of primary myocardial involvement. LV filling dysfunction is progressive and precedes the occurrence of LV remodeling. PubMed. Semin Arthritis Rheum. 2005 Apr;34(5):721-7. |
| Lipoprotein[a] and Thrombotic Complications |
| Hypothyroidism Contributes to Increased Triglyceride Levels Among Patients with Systemic Sclerosis (SSc). In a cohort of 49 patients with diffuse SSc clinical and laboratory features of hypothyroidism were observed in 27% of the patients and increased triglyceride levels in more than 55%. Hypothyroidism is a common finding in patients with SSc. J. Rheumatol 2006 April;33:827. Correspondence. Full Text. (Also see: Thyroid Disease and Cardiac Involvement) |
| Lipoprotein[a] and the lipid profile in patients with systemic sclerosis. Lp[a] measurement might be useful in SSc to identify and eventually treat subsets of patients more predisposed to develop thrombotic complications. PubMed. Clin Chim Acta. 2005 Aug 17. |
| Mitral and Tricuspid Valve Regurgitation |
| Mitral Regurgitation. Mitral regurgitation (mitral valve regurgitation, mitral incompetence, mitral insufficiency) is leakage of blood backward through the mitral valve each time the left ventricle contracts. Merck. |
| Tricuspid Regurgitation. Tricuspid regurgitation (tricuspid incompetence, tricuspid insufficiency) is leakage of blood backward through the tricuspid valve each time the right ventricle contracts. Merck. |
| Heart Involvement in Patients with Systemic Sclerosis. Increased intraventricular septum diameter (IVSD), mitral and tricuspid valve regurgitation were common in the studied group. Heart involvement was associated with the presence of antinuclear antibodies (anti-scl-70, ACA). B. Trzcinska-Butkiewicz. FRI0155 EULAR 2005. |
| Myocardial Ischemia |
| Cardiac (Myocardial) ischemia. Cardiac ischemia occurs when blood flow to the heart muscle (myocardium) is obstructed by a partial or complete blockage of a coronary artery. Mayo Clinic. |
| Myocardial involvement in systemic sclerosis. Myocardial perfusion defects in SSc patients are frequent, and the presence of severe skin thickness, digital ulcers and perhaps oesophageal involvement might warrant screening for myocardial involvement. L. Belloli. Rheumatology. May 25, 2008. |
| The Incidence of Pulmonary Hypertension and Myocardial Ischemia in Systemic Sclerosis by Echocardiography Exercise Testing. Echocardiography exercise test is more sensitive, baseline method as compared to the rest echocardiography. The confirmation of PAH with right heart catheterization (RHC) might be applied to all the patients with systemic sclerosis. B. N. Stamenkovic. FRI0127 EULAR 2005. (Also see: Pulmonary Hypertension) |
| Right Ventricular Dysfunction |
| Right Ventricular Failure. The right ventricle's main function is a volume pump whereas the left ventricle functions as a pressure pump. The thin-walled right ventricle is sensitive to changes in afterload (pulmonary artery pressure). An increase in afterload may result in a marked decrease in right ventricular function. e-echocardiography.com. |
| Right ventricular function in scleroderma-related pulmonary hypertension (SSc-PAH) . Recent echocardiographic and haemodynamic studies suggest that right ventriculer (RV) pump function and filling characteristics are altered in SSc-PAH as compared with idiopathic PAH. (UnBound MedLine) Vonk Noordegraaf A. Rheumatology (Oxford) 2008 Oct.:v42-3. (Also see: PAH) |
| Cardiac Magnetic Resonance Imaging Detects Subclinical Right Ventricular Impairment in Systemic Sclerosis. Compromised right ventricular failure was found in patients with asymptomatic SSc. J Rheumatol 2007;34:2431-7. |
| Right heart function (RV) in scleroderma: insights from myocardial Doppler tissue imaging. In progressive systemic sclerosis, RV systolic dysfunction is common and appears to be a result of pulmonary hypertension, disturbance of myocardial microcirculation, and myocardial fibrosis. PubMed. J Am Soc Echocardiogr. 2006 May;19(5):507-14. |
| Disturbed right ventricular diastolic function in patients with systemic sclerosis (SSc): a Doppler tissue imaging study. Patients with SSc exhibit altered RV (right ventricular) diastolic function together with an increase in RV wall thickness and RA (right atrial) area. These findings appear to be early markers of RV disturbance, probably in response to intermittent pulmonary arterial hypertension. PubMed. Chest. 2005 Aug;128(2):755-63. |
| Isolated Right Ventricular Failure in Scleroderma Heart Disease. Right ventricular failure (RVF) is a well-recognized cardiac complication of scleroderma and most often is related to underlying pulmonary hypertension (PH). We describe a case of predominant RVF in the absence of significant PH in a patient with longstanding scleroderma. PubMed. Cardiol Rev. 2004 Sep-Oct;12(5):279-281. |
| Stroke and Mini-Stroke (Transient Ischemic Attack, or TIA) |
| Carotid Artery Disease (CAD) is more common in patients with systemic sclerosis and can lead to a stroke. Also, Raynaud's doubles the risk of coronary artery disease and stroke. |
| What is a Stroke? Stroke is the third leading cause of death in America and the No. 1 cause of adult disability. 80% of strokes are preventable; you can prevent a stroke! Someone who has a small stroke may experience only minor problems such as weakness of an arm or leg. People who have larger strokes may be paralyzed on one side or lose their ability to speak. National Stroke Association. |
| What is a Transient Ischemic Attack (TIA)? It is a stroke that lasts only a few minutes. Symptoms can include numbness or weakness in the face, arm, or leg, especially on one side of the body; confusion or difficulty in talking or understanding speech; trouble seeing in one or both eyes; and difficulty with walking, dizziness, or loss of balance and coordination. National Institute of Neurological Disorders and Stroke, 08-07-06. |
| The Impact of Immunosuppressive Medications on Cardiovascular Events in Rheumatoid Arthritis Patients. TNF blockers were not associated with either a reduction or an increase in the risk of heart attack or stroke compared with the most commonly used RA treatment, methotrexate. American College of Rheumatology. 11/29/06. (Also see: Rheumatoid Arthritis) |
| Too Many Ignore Symptoms of Mini-Stroke. Anyone feeling the symptoms of a mini-stroke (TIA) should go directly to an emergency room. Symptoms typically include a sudden numbness or weakness of part of the body, sudden confusion, visual problems, dizziness or a sudden severe headache. HealthyWomen.org, 03-30-06. |
| Antiphospholipid antibodies can come and go in an individual. There are many ways to measure these antibodies, and different methods may not always give the same result. The presence of these antibodies may also increase the future risk of deep venous thrombosis (thrombophlebitis), stroke, gangrene, and heart attack. Lupus Foundation of America. (Also see: Antiphospholipid Antibodies) |
| Thrombotic thrombocytopenic purpura (TTP) mimicking acute ischemic stroke. TTP is an autoimmune disorder characterised by thrombocytopenia, haemolytic anemia, fluctuating neurological deficits, fever, and renal impairment. PubMed. Emerg Med J. 2006 Sep;23(9):e51. (Also see: Associated Diseases: Thrombotic Thrombocytopenic Purpura) |
| Raynaud's Phenomenon (RP), Systemic Sclerosis (SSc), and Macrovascular Peripheral Disease. In this sample of atherosclerotic patients, patients with RP were five times more prone to coronary disease, compared to patients without RP, which could suggest cardiac Raynaud's. SSc was more frequent than expected. J. F. J. Olmedo. AB0284 EULAR 2006. (Also see: Raynaud's) |
| Ischaemic stroke in progressive systemic sclerosis. We have documented a cerebral infarct in a PSS patient. In our opinion, the ischaemic stroke was caused by a localised autoimmune angiopathy. PubMed. Neurol Sci. 2004 Oct;25(4):230-233. (Also see: Systemic Scleroderma Brain Involvement) |
| Scleroderma, stroke, optic neuropathy: a rare association. A known case of scleroderma presented with right hemiparesis, focal seizures, optic atrophy and gangrene of digits. MRI showed multifocal infarcts in both cerebral hemispheres. The rarity of central nervous system affliction in scleroderma and large vessel vasculitis is discussed along with review of literature. PubMed. Neurol India 2002 Dec;50(4):504-7. (Also see: Eye Involvement) |
| A 1998 study indicated that people who have Raynaud's may have double the risk of coronary artery disease and stroke. "Hopkins: Is Raynaud's a Predictor of Heart Disease?" by Fredrick M. Wigley, M.D., Professor of Medicine and Director of Molecular and Clinical Rheumatology, Johns Hopkins School of Medicine, August 19, 1998, Aetna InteliHealth. |
| Also see: Antiphospholipid Antibodies, Hypertension, Raynaud's, Scleroderma Brain Involvement and Vasculitis. |
| Stroke Patient Stories |
| Barb: Diffuse Systemic Scleroderma I was diagnosed in 1985 with systemic scleroderma and Raynaud's. It has affected my esophagus, fingers, toes and bowels, and recently I started having mini strokes... |
| Papa V: Lupus, Antiphospholipid Syndrome, Myasthenia Gravis, and Scleroderma About thirty years passed, and then—BAM !—I got a heart blockage, stroke, liver biopsy due to elevated enzymes, skin rash, high blood pressure, very high ANA... . |
