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
Asymptomatic, Multiple Associations
Atrioventricular Heart Block
Cardiogenic Shock
Cardiac Tamponade
"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)
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: Effects of SSc, 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.
"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.
Acute Myocardial Infarction in Systemic Sclerosis Patients: A Case Series. Acute MI remains an uncommon complication of SSc which in some cases coronary vasospasm may play a central role as was the case in the 3 patients presented here. Of final note, 7/11 SSc patients in our case series who developed MI had a creatinine >1.3 mg/dl. Chris T. Derk. ACR Conference Oct. 2003.
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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