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)

Patient Subgroups and Potential Risk Factors in Systemic Sclerosis (SSc): Is There a Possibility of an Early Diagnosis: Sudden Cardiac Death. All SSc patients should be monitored routinely by ambulatory electrocardiography, echocardiography and Holter ECG and, if necessary, further examinations should be performed (MRI, single photon emission computed tomography). Medscape.


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 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).

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.

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.

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

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.

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), 10/26/2015. (Also see Dermatomyositis and Polymyositis)

Left atrial (LA) dysfunction detected by speckle tracking in patients with systemic sclerosis (SSc). 2D speckle-tracking echocardiography is a sensitive tool to assess impairment of LA mechanics, and may represent an early sign of cardiac involvement in patients with systemic sclerosis. PubMed, Cardiovasc Ultrasound, 2014 Aug 5;12(1):30.

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 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)

Go to Diagnosis of Scleroderma Heart Involvement

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