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
Complications with Systemic Scleroderma Heart Involvement
Overview
Aneurysms
Aortic Valve Insufficiency

Arrhythmia
Asymptomatic, Multiple Associations
Atrioventricular Heart Block
Cardiogenic Shock
Cardiac Tamponade
Diastolic Dysfunction
"No Reflow" Phenomenon
Macrovascular Disease
Myocardial Infarction
Skeletal Myopathy
Overview
Complications with systemic sclerosis (scleroderma) heart involvement include atrioventricular heart block, cardiogenic shock, myocardial infarction, and skeletal myopathy. (Also see: What is Scleroderma?)
Doppler myocardial imaging (DMI) can identify patients with a more diffuse and severe form of scleroderma, ideally before they develop severe vascular problems. (Also see: Diagnosis of Scleroderma Cardiac Involvement)
Aneurysms
Left ventricular aneurysms developed in a patient with systemic sclerosis. In this case, the patient with systemic sclerosis developed a rare complication of ventricular aneurysms without coronary disease. Taniguchi Y. (PubMed) Mod Rheumatol. 2007;17(6):518-20.
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. Attaran RR, Guarraia D. (PubMed) Clin Rheumatol. 2007 Jun;26(6):1027-8.
Aortic Valve Insufficiency
Aortic valve insufficiency in patients with chronic rheumatic diseases. Patients with rheumatic disease as a potential aetiology of aortic valve insufficiency often had symptomatic valve disease, which required surgical treatment, although great differences between different aetiologies were not found. PubMed. Clin Rheumatol. 2005 Oct 11;:1-5.
Arrhythmias, Palpitations, and Conduction Disturbances
Palpitations. Palpitations are unpleasant sensations of irregular and/or forceful beating of the heart. In some patients with palpitations, no heart disease or abnormal heart rhythms can be found. In others, palpitations result from abnormal heart rhythms (arrhythmias). MedicineNet.com.
What is Arrhythmia? General Physician Home.
Drink for Your Heart but Abstain for Cancer? Based on the many studies finding that moderate alcohol consumption can have a heart-protective benefit, many experts said that moderate drinkers should not worry about this latest research finding an increased risk in cancer. A. Grayson and J. Schaffhausen. ABC News Medical Unit 02/25/09 (Also see: Cancer)
An Angry Heart Can Lead To Sudden Death, Yale Researchers Find. Consider the latest research from Yale School of Medicine researchers that links changes brought on by anger or other strong emotions to future arrhythmias and sudden cardiac arrests. Medical News Today 02/25/09.
Heart rhythm risk seen in women's alcohol drinking. Women who consume more than two alcoholic drinks a day have a higher risk of getting the most common type of heart rhythm disturbance, which can raise the chances of having a stroke, researchers said on Tuesday. Reuters. 12/02/08.
Evaluation of Heart Rhythm Variability and Arrhythmia in Children with Systemic and Localized Scleroderma. The most frequent cardiac arrhythmias in both types of scleroderma in children were of supraventricular origin, whereas ventricular arrhythmias did not occur very often. J. Wozniak. J Rheumatol Dec 1 2008. (Also see: Juvenile Scleroderma)
Heating Heart with Catheter Better than Drugs for Common Heart Rhythm Disorder. Treating a common heart rhythm disorder by burning heart tissue with a catheter works dramatically better than drug treatments, a major international study has found. Loyola Medicine. 11/11/08.
New Mechanism For Cardiac Arrhythmia Discovered. CAR stands for Coxsackievirus-Adenovirus-Receptor. It is embedded in specific cell-cell-contacts (tight junctions) of the specialized heart fibres. When CAR is missing, the signal can not be passed on and the heart does not beat properly. ScienceDaily. 09/18/08.
Sudden Cardiac Death in Infiltrative Cardiomyopathies: Sarcoidosis, Scleroderma, Amyloidosis, Hemachromatosis. Conduction system abnormalities are common, and each of these disorders has been associated with sudden cardiac death. Steven A. Lubitz, Progress in Cardiovascular Diseases. Vol 51, Issue 1, Pages 58-73 (July 2008). (Also see: Sarcoidosis)
Systemic sclerosis sine scleroderma associated with Wolff-Parkinson-White (WPW) syndrome. This case highlights cardiac arrhythmia caused by WPW syndrome as a clinical manifestation of the heart in ssSSc. PubMed. Scand J Rheumatol. 2007 Jan-Feb;36(1):68-70. (Also see: Diffuse Scleroderma Without Skin Involvement)
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.
Is Expensive New Heart Rhythm Treatment "worth It"? A new procedure (left atrial catheter ablation) that stops the most common form of irregular heartbeat is expensive, but it may pay off in the long run for many patients. Medical News Today. 06/22/06.
Engineered Heart Tissue Offers Insights Into Arrhythmias. Engineers who have induced heart cells in culture to mimic the properties of the heart have used the tissue to gain new insight into the mechanisms that spawn irregular heart rhythms. Duke University. 02-03-06.
Arterial Stiffness
Impaired Arterial Elasticity In Patients With Systemic Sclerosis. Increased Augmentation index (AIx) and pulse wave velocity (PWV) of brachial artery show an increased arterial stiffness in SSc. In healthy subjects there was an inverse correlation between endothelial function and arterial stiffness. G. Szücs AB0535 EULAR 2007.
Asymptomatic, Multiple Associations with Diffuse Scleroderma
Exercise Performance In Systemic Sclerosis: Relationship With Disease Manifestations. Exercise performance is impaired in most SSc patients, at least in those admitted in a referral clinic. The impairment of exercise performance is correlated to lung, heart and skin involvement. L. Ruocco AB0525 EULAR 2007 (Also see: Pulmonary, and Skin Involvement)
Clinical features of scleroderma patients with contracture of phalanges (CP). Our study suggested that the presence of CP may be a marker of oesophageal involvement, pulmonary fibrosis and heart involvement. PubMed Clin Rheumatol. 2007 Aug;26(8):1275-7. (Also see: Joint Contractures, Esophageal Involvement,and Pulmonary fibrosis)
Plasma Brain Natriuretic Peptide as a Marker of Right Ventricular Overload in Systemic Sclerosis. NT-proBNP is a good marker of diastolic dysfunction frequently found in SSc, determined by various degrees of pulmonary hypertension, well correlated with inflammatory markers. A. E. Musetescu. SAT0217 EULAR 2006. (Also see: Pulmonary Hypertension)
Associations of right ventricular myocardial function with skin and pulmonary involvement in asymptomatic patients with systemic sclerosis. The relationships of RV (right ventricular) myocardial diastolic dysfunction with both skin and pulmonary involvement as well as with the serological antibody pattern emphasizes the ability of DMI (Doppler myocardial imaging) to identify patients with a more diffuse and severe form of SSc. PubMed. Ital Heart J. 2004 Nov;5(11):831-9.
Atrioventricular Heart Block
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.
Second degree atrioventricular block in mixed connective tissue disease. Heart blocks are rarely described in MCTD. Three cases have been reported. It's a systemic complication resulting from an inflammatory process often responsive to steroids. PubMed. Rev Med Interne. 2005 May 27.
The association of high grade atrioventricular heart block with systemic connective tissue diseases is very rare. In patients with systemic connective tissue diseases and emergency symptoms, it is recommended to consider also this rare association. In the last years, there are almost no reports about the association of systemic connective tissue diseases with high grade heart block, presumably because of the efficient new treatment approached to systemic diseases including modern immunomodulation drugs. PubMed. Vnitr Lek 2002 Dec;48 Suppl 1:201-5.
Cardiogenic Shock
Reversible cardiogenic shock in an angry woman. The presence of high titre anticentromere antibodies, anticardiolipin antibodies, protein S deficiency and supportive physical findings, suggested the diagnosis of concurrent antiphospholipid antibody syndrome (with secondary acquired protein S deficiency) and CREST syndrome. The pathogenesis likely involved an interaction between stress, vasospasm, and thrombosis. Expired article. The Canadian Journal of Cardiology 2002. (Also see Antiphospholipid Antibody Syndrome and CREST Syndrome)
Cardiac Tamponade
Cardiac tamponade. Cardiac tamponade is the compression of the heart caused by blood or fluid accumulation in the space between the myocardium (the muscle of the heart) and the pericardium (the outer covering sac of the heart). Medline Plus.
Atypical hemodynamic manifestations of cardiac tamponade. These atypical cardiac tamponade cases include: A patient with systemic sclerosis without hypotension; and a patient with pulmonary hypertension lacking right heart collapse on echocardiography. Recognition of these atypical clinical and hemodynamic manifestations of cardiac tamponade will avoid undue delay in the treatment. PubMed. Catheter Cardiovasc Interv. 2004 Oct 25;63(3):339-345.
Diastolic Dysfunction
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: Cardiac Involvement)

Regional diastolic function by tissue Doppler echocardiography in systemic sclerosis (SSc): correlation with clinical variables. The incidence of left ventricular (LV) diastolic dysfunction is increased in SSc, while systolic dysfunction is present in a small percentage of patients. Linear correlation was observed between the extent of the diastolic abnormalities and the duration of disease. Longitudinal myocardial systolic velocities were significantly reduced in patients with abnormal DTI (doppler tissue imaging). E. Rosato. (SpringerLink) Rheumatology International 12/30/2008.

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.
The diastolic function of the left ventricle in patients with systemic lupus erythematosus and systemic scleroderma. All the patients with rheumatic diseases, with or without arterial hypertension (AH), had diastolic dysfunction, which was manifested by increase of atrial systolic contribution into LV filling, prolongation of blood flow slowdown time in the stage of its early filling, and prolongation of LV isometric relaxation time; heart diastolic disorder was accompanied by significant increase of end diastolic pressure in LV cavity. PubMed. Klin Med (Mosk). 2005;83(7):45-7. (Also see: Lupus)
"No Reflow" Phenomenon
"No-reflow" phenomenon during percutaneous carotid angioplasty in a scleroderma patient. We describe a patient with systemic sclerosis who underwent CAS (carotid angioplasty and stenting) with distal embolic protection device with occurrence of no-reflow phenomenon during the procedure that resolved after retrieval of the filter device. PubMed. Int J Cardiovasc Intervent. 2004;6(2):82-4.
Macrovascular Disease
Macrovascular Disease. Disease of the large blood vessels, including the coronary arteries, the aorta, and the sizable arteries in the brain and in the limbs. MedicineNet.com.
Macrovascular Disease In Systemic Sclerosis (SSc). This study showed an increased intima-media thickness (IMT) in SSc patients compared to healthy controls. Though no differences were seen in traditional risk factors, a subanalysis showed an increased prevalence of a positive family history of cardiovascular disease in the SSc patients with increased IMT. Therefore, besides the vasculopathy, traditional risk factors appear to play a role in the prevalence of atherosclerosis in SSc patients. M. E. Hettema. AB0505 EULAR 2007.
Myocardial Infarction
A myocardial infarction is a heart attack. It occurs when the blood supply to part of the heart muscle itself (the myocardium) is severely reduced or stopped.
Myocardial damages in systemic sclerosis detected by gated myocardial perfusion SPECT and sympathetic imaging. In patients with SSc, either diastolic dysfunction or sympathetic derangement, or both were observed even without induced ischemia and normal ventricular contractility. PubMed. Circ J. 2006 Nov;70(11):1481-7.
Primary myocardial involvement in systemic sclerosis (SSc). Primary myocardial involvement is common in SSc. Evidence suggests that myocardial involvement is due, at least in part, to microcirculation impairment with abnormal vasoreactivity, with or without associated structural abnormalities of the small coronary arteries or arterioles. PubMed. Rheumatology (Oxford). 2006 Oct;45 Suppl 4:iv14-iv17.
Acute myocardial infarction in systemic sclerosis patients: a case series. Acute MI is an uncommon manifestation in systemic sclerosis patients. Normal coronaries are seen more commonly in these patients as compared to the general population, while vascular, gastrointestinal, and renal involvement is prevalent in these patients. PubMed. Clin Rheumatol. 2006 Mar 7.
Skeletal Myopathy
Clinical and laboratory features of scleroderma patients developing skeletal myopathy. These findings suggested that the SSc patients with severe internal organ involvement, such as pulmonary fibrosis and heart disease, and some other complications were prone to develop skeletal myopathy during their clinical course of the disease. PubMed. Clin Rheumatol. 2004 Aug 20. (Also see: Skeletal Involvement and Pulmonary Fibrosis)
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