Complications with Systemic Scleroderma (SSc) Heart Involvement

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

Overview of Cardiac Complications with SSc

Systemic sclerosis might be a vascular disease, which begins by targeting blood vessels.

Photo of butterfly needleComplications 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)

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 atherosclerosis. Diseases 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 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

What is Arrhythmia? General Physician Home.

Incidental significant arrhythmia in scleroderma (SSc) associates with cardiac magnetic resonance measure of fibrosis and hs-TnI and NT-proBNP. This first implantable loop recorder study identified potentially life–threatening arrhythmias in asymptomatic SSc patients attributable to a primary SSc heart disease. PubMed, Rheumatology (Oxford), 01/28/2019.

Improving overall heart health may reduce risk of atrial fibrillation. Following practices recommended for “optimal” heart health may also reduce the risk of developing a serious heart–rhythm disorder, researchers say. Reuters Health, 04/19/2018.

A ‘Game Changer’ for Patients With Irregular Heart Rhythm. Rapid, erratic heartbeats — called ventricular tachycardia — can lead to sudden death. An experimental radiation treatment has eased the condition in five patients. New York Times, 12/13/2017.

Cardiac complications in rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). Since an increased risk of cardiovascular complications applies to many rheumatic diseases, the European League Against Rheumatism (EULAR) has developed a number of recommendations related to the prevention and therapy of cardiovascular events. PubMed, Przegl Lek, 2017;74(4):179-82. (Also see Symptoms and Complications of Lupus and Symptoms and Complications of Rheumatoid Arthritis)

Overlap of symptoms of gastroesophageal reflux disease (GERD), dyspepsia (FD) and irritable bowel syndrome (IBS) in the general population. GERD, FD and IBS are common conditions in the general population and the overlap between these conditions is also quite common. PubMed, Scand J Gastroenterol, 2015 Feb;50(2):162-9. (Also see Irritable Bowel Syndrome and Reflux)

Arterial Stiffness

Cardiovascular disease in systemic sclerosis. In summary, cardiovascular disease prevention and surveillance represent an opportunity to further reduce morbidity and mortality in systemic sclerosis and, in consequence, modification of traditional cardiovascular risk factors should be part of standard care for these patients. Annals of Translational Medicine, Vol 3, No 1, January 2015.

Atrioventricular Heart Block

Case Report: Complete heart block (CHB) in systemic sclerosis (SSc). Pacemaker implantation might be unavoidable if CHB is secondary to SSc, even if it is asymptomatic. PubMed, Medicine (Baltimore), 2018 Nov;97(46):e13226.

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.

Cardiogenic Shock

Cardiogenic shock is a condition in which your heart suddenly can't pump enough blood to meet your body's needs. The condition is most often caused by a severe heart attack. Mayo Clinic.

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.

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.

Evaluation of left atrial (LA) volume and function in systemic sclerosis (SSc) patients using speckle tracking and real–time three–dimensional echocardiography. LA volumes were significantly increased, and LA reservoir, conduit, and contractile functions were significantly impaired in SSc patients compared with controls. PubMed, Anatol J Cardiol, 2016 May;16(5):316-22.

Cardiac abnormalities in adult patients with polymyositis (PM) or dermatomyositis (DM) as assessed by non-invasive modalities. Patients with PM or DM had an increased prevalence of cardiac abnormalities compared to healthy controls and left ventricular diastolic dysfunction was a common occurrence and correlated to disease duration. PubMed, Arthritis Care Res (Hoboken), 2016 Jul;68(7):1012-20. (Also see Dermatomyositis and Polymyositis)

Macrovascular Disease

Evaluation of differences in carotid intima–media thickness in patients affected by systemic rheumatic diseases. Systemic sclerosis (SSc) patients had a significant increase in C-IMT as compared to NoSSc patients and healthy controls. PubMed, Intern Emerg Med, 2015 Oct;10(7):823-30.

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 is common in systemic sclerosis.

Evaluation of early myocardial damage in systemic sclerosis (SSc): a cardiovascular magnetic resonance study. Scleroderma patients with no cardiac symptoms and preserved left ventricular size and function have nonetheless cardiac involvement, which can be detected with conventional T2 weighted and LGE imaging. Journal of Cardiovascular Magnetic Resonance, 2015, 17 (Suppl 1):P264.

Skeletal Myopathy

Cardiac troponin testing in idiopathic inflammatory myopathies and systemic sclerosis-spectrum disorders: biomarkers to distinguish between primary cardiac involvement and low-grade skeletal muscle disease activity. Within these two conditions, traditional skeletal muscle enzyme testing may not effectively distinguish between skeletal and cardiac muscle involvement, especially in patients with subclinical cardiac disease. PubMed, Ann Rheum Dis, 2015 May;74(5):795-8. (Also see Myositis)

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