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Cardiac (Heart) Involvement
This page was written by Shelley Ensz, and has not yet been medically edited. See Disclaimer.
Overview
Antiphospholipid
Scleroderma
Complications
Heart Symptoms
Diagnosis
Treatments
Research
Patient & Caregiver Stories
Systemic Scleroderma Cardiac (Heart) Involvement
Overview of SD Cardiac Involvement
Antiphospholipid Antibodies
Aortic Stiffness
Asymptomatic Pericardial Disease
Cardiac Remodeling
Carotid Artery Disease
Cholesterol
Atherosclerosis
Left Ventricular Diastolic Dysfunction
Lipoprotein[a], Thrombotic
Mitral and Tricuspid Valve
Myocardial Ischemia
Right Ventricular Dysfunction
Stroke and TIA
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)
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)
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.
What is APS?
APS and Heart Disease
Scleroderma and APS
Symptoms of APS
Complications of APS
Treatments for APS
Causes of APS and Research
Support Organizations for APS
Personal Stories about APS
Media Stories about APS
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.
Coronary atherosclerosis using computed tomography coronary angiography in patients with systemic sclerosis. Coronary atherosclerosis is not uncommon in asymptomatic SSc patients computed tomography coronary angiography (CTCA) is a convenient and non-invasive method for studying coronary atherosclerosis. MY Mok (Informaworld) Scandinavian Journal of Rheumatology 07 July 2009.
Premature Coronary Atherosclerosis in Scleroderma. The purpose of this trial is to study the proportion of scleroderma patients who suffer from asymptomatic coronary atherosclerosis compared to healthy controls. This study is currently recruiting participants. clinicaltrials.gov, May, 2009. (Also see: Clinical Trials)
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. (PubMed) Arthritis Res Ther. 2008;10(2):R49. Epub 2008 Apr 25.
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.
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 and femoral arterial wall mechanics in scleroderma. The elastic properties of the carotid artery are significantly altered in SSc, and the two major subsets of SSc may be distinguished by their carotid artery biomechanics. This suggests that connective tissue abnormality occurs at sites not previously assessed. Wellsphere, Nov 2009.
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)
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.
Diagnosis and Management of Pulmonary Hypertension in Systemic Sclerosis. Patients with systemic sclerosis (SSc) can develop pulmonary hypertension (PH) caused by pulmonary arterial hypertension (PAH), left ventricular disease, or pulmonary fibrosis. Prognosis and therapeutic response are worse in PAH-SSc than in other PAH categories (median survival, 1–3 yr). Current Rheumatology Reports, Volume 12, Number 1 / February, 2010. (Also see: Secondary Pulmonary Hypertension and Pulmonary Fibrosis)
Prevalence and factors associated with left ventricular (LV) dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of systemic sclerosis (SSc) patients. The prevalence of LV dysfunction in SSc is 5.4%. Age, male gender, digital ulcerations, myositis and lung involvement are independently associated with increased prevalence of LV dysfunction. Conversely, calcium channel blocker (CCB) use may appear as protective. Y Allanore Ann Rheum Dis. 10 March 2009 (Also see: Diastolic Dysfunction)
Diastolic Dysfunction can be a complication of systemic scleroderma. ISN
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.
Mitral regurgitation. The main causes are classified as degenerative (with valve prolapse) and ischaemic (ie, due to consequences of coronary disease) in developed countries, or rheumatic (in developing countries). These results emphasise the importance of early detection and assessment of mitral regurgitation. M. Enriquez-Sarano FACC. The Lancet, Volume 373, Issue 9672, Pages 1382 - 1394, 18 April 2009.
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.
Right Ventricular Dysfunction
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.
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.
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)
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... .
 
Go to Patient and Caregiver Stories of Cardiac Involvement
 
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