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Complications with Systemic Scleroderma (SSc) Heart Involvement

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Aortic Valve Insufficiency
Asymptomatic, Multiple Associations
Atrioventricular Heart Block
Cardiogenic Shock
Cardiac Tamponade
Diastolic Dysfunction
Macrovascular Disease
Myocardial Infarction
Skeletal Myopathy

Overview of Cardiac Complications with SSc

Complications with systemic sclerosis (scleroderma) heart involvement include atrioventricular heart block, cardiogenic shock, myocardial infarction, and skeletal myopathy. (Also see: What is Scleroderma?, Types of Scleroderma, Symptoms of Scleroderma, and Overview of Cardiac Involvement)
Cardiac complications of systemic sclerosis: recent progress in diagnosis. Early detection of scleroderma heart disease will allow exploration of novel therapies with potential positive impact on the quality of life and life expectancy of this patient population. Adel Boueiz. (PubMed) Current Opinion in Rheumatology, 22 July, 2010.
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)


Aortic Aneurysms. Approximately 95 percent of aortic aneurysms are caused by atherosclerosisDiseases that cause aortic aneurysms include Marfan syndrome, Loeys-Dietz Syndrome, Turner's Syndrome, and Scleroderma. Johns Hopkins Medicine. (Also see: What is Scleroderma?)

Aortic Valve Insufficiency

Aortic Valve Disease. Aortic Valve Disease entails damage to, and dysfunction of, the aortic valve, one of the four valves in the heart. The valve may become abnormally narrow (aortic stenosis), or the valve may not close properly. In either case, the work of the ventricle increases. As a result, its muscular wall thickens (a condition known as hypertrophy) and the left ventricle may become larger (dilate). HealthCentral.com.
Aortic valve insufficiency in patients with chronic rheumatic diseases. Patients with rheumatic disease as a potential aetiology (cause) of aortic valve insufficiency often had symptomatic valve disease, which required surgical treatment, although great differences between different aetiologies were not found. Uusimaa P. (PubMed) Clin Rheumatol.

Arrhythmias, Palpitations, and Conduction Disturbances

New Heart Warning for Popular Z-Pak Antibiotics. Elderly people and those with irregular heart rates, arrhythmias, and low blood levels of potassium or magnesium are at a particularly high risk for the deadly heart condition. ABC News. 03/12/2013.
Safety of Pacemakers and Defibrillators in Electromagnetic Navigation Bronchoscopy (ENB). ENB appears to be safe when performed in patients with pacemakers and automated implantable cardioverter-defibrillators (AICDs.) CHEST, January 2013, Vol 143, No. 1.
Vitamin D warning: Too much can harm your heart. Scientists have long known that low levels of the nutrient can hurt the heart, but new research shows that higher than normal levels can make it beat too fast and out of rhythm, a condition called atrial fibrillation. MSNBC. 11/18/2011. (Also see: Vitamin D and Scleroderma Supplement Guide)
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.
Implantable Cardioverter Defibrillator Prevents Sudden Cardiac Death in Systemic Sclerosis (SSc). In patients with SSc who are affected by ventricular arrhythmias, the implantation of a cardioverter defibrillator may prevent sudden cardiac death. Pasquale Bernardo, J Rheumatology. June 6, 2011.
Coffee associated with reduced risk of hospitalization for heart rhythm disturbances. While it is not proven that coffee is protective, it is unlikely that moderate caffeine intake increases arrhythmia risk, according to a new study. The researchers note the findings may be surprising because patients frequently report palpitations after drinking coffee. Eureka Alert. March 2, 2010. (Also see: Foods and Health)

Arterial Stiffness

Cardiovascular disease in systemic sclerosis (SSc): an emerging association? Excess cardiovascular risk in SSc is suggested by increased arterial stiffness and carotid intima thickening, and reduced flow mediated dilatation. Gene-Siew Ngian. (7th Space) Arthritis Research &Therapy 2011, 13:237.

Asymptomatic, Multiple Associations with Diffuse Scleroderma

Elevated B-Type Natriuretic Peptide Levels Are Associated with Worse Survival in Systemic Sclerosis Associated Pulmonary Hypertension. B-type natriuretic peptide levels, and NT-proBNP in particular, may be useful in monitoring and predicting outcomes in patients with SSc-associated PH. Arthritis & Rheumatism, Volume 60, October 2009.

Atrioventricular Heart Block

Third-Degree Atrioventricular Block. Heart block occurs when slowing or complete block of this conduction occurs. Traditionally, heart block can be divided into first-, second-, and third-degree block. eMedicine.
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. Vinsonneau U. (PubMed) Rev Med Interne. 26(8):656-60.
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. Vachtenheim J. (PubMed) Vnitr Lek.

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.
An unusual case of undifferentiated connective tissue disease (UCTD) presenting as cardiac tamponade. This case presents UCTD as a rare cause of cardiac tamponade and large pericardial effusions and suggests that colchicine can be used to treat UCTD-associated effusions. (SpringerLink) P. Hari. Rheumatology International. (Also see: UCTD)

Diastolic Dysfunction

What is diastolic dysfunction? Diastolic dysfunction is abnormal function of the heart during its relaxation phase, between beats, called diastole. While the heart's ability to contract and pump blood may be maintained, its ability to relax and fill with blood is compromised. Sharecare.
Systolic and diastolic function in patients with systemic sclerosis. The analysis of SSc heart disease, mainly at a preclinical level, is important in all the cases as an asymptomatic patient may have diastolic dysfunction which can be treated and should be closely observed. Laura Poanta. European Journal of Internal Medicine. Volume 20, Issue 4, Pages 378-382 (July 2009).
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)
Systolic and diastolic function in patients with systemic sclerosis (SSc). The analysis of SSc heart disease, mainly at a preclinical level, is important in all the cases as an asymptomatic patient may have diastolic dysfunction which can be treated and should be closely observed. Poanta L. (PubMed) Eur J Intern Med. 2009 Jul;20(4):378-82.

Macrovascular Disease

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.
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.

Skeletal Myopathy

Skeletal and cardiac muscles involvement in systemic sclerosis. Patients with systemic sclerosis (SSc) can have muscle involvement in the form of myositis or non-infl ammatory myopathy. The muscle involvement can be associated with left ventricular dysfunction (LVD) in patients with SSc, resulting in worse prognosis. Schade L. (PubMed) Rev Bras Reumatol. 2011 Aug;51(4):311-313. (Also see: Myositis)
Go to Diagnosis of Scleroderma Heart Involvement

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