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.

(PDF) Evidence That Systemic Sclerosis Is a Vascular Disease. Vascular disease is a major pathologic component of SSc and blood vessels are likely the initial target of the disease process. Marco Matucci-Cerinic, Bashar Kahaleh, and Fredrick M. Wigley; Arthritis & Rheumatism, Vol. 65, No. 8, August 2013, pp 19531962. (Also see ISN Dr. Matucci-Cerinic)


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).

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)

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.

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.

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.

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 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

SCLERO.ORG is the world leader for trustworthy research, support, education and awareness for scleroderma and related illnesses, such as pulmonary hypertension. We are a service of the nonprofit International Scleroderma Network (ISN), which is a 501(c)(3) U.S.-based public charitable foundation, established in 2002. Meet Our Team, Volunteer, or Donate.

International Scleroderma Network (ISN)
7455 France Ave So #266
Edina, MN 55435-4702 USA

Email [email protected] to request our Welcome email, or to report or update any page content or links.

Toll Free US/Canada Scleroderma Hotline: 800-564-7099

The most important thing in the world to know about scleroderma is!
Copyright 1998-2015, International Scleroderma Network. AKA Scleroderma from A to Z and SCLERO.ORG. All Rights Reserved.