Diagnosis of Systemic Scleroderma Heart Involvement

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
CT Scans
--ECHO (General)
--Tissue Doppler
Heart Rate
Holter Monitoring


Screening for heart involvement is mandatory for all systemic scleroderma patients.

It is common for patients with systemic scleroderma to develop heart involvement even without any overt cardiac symptoms, so mandatory assessment of systemic scleroderma heart involvement requires a thorough baseline screening and follow-up monitoring. Diagnostic tests for scleroderma heart involvement may include electrocardiograms, echocardiography, Holter monitoring, CT scans, and MRI's. (Also see What is Scleroderma?, Types of Scleroderma, Symptoms of Scleroderma and Overview of Cardiac Involvement)

Diagnostic tests for cardiac problems include Electrocardiogram (ECG) and Holter Monitor. (See Medical Tests)

CT Scans

Usefulness of technetium-99 m sestamibi myocardial perfusion SPECT in detection of cardiovascular involvement in patients with systemic lupus erythematosus or systemic sclerosis. Tc-99m sestamibi SPECT is a useful noninvasive imaging modality to detect cardiac involvement in symptomatic or asymptomatic SLE or SS patients. Lin CC. (PubMed) Int J Cardiol. 92(2-3):157-61.

Echocardiography (ECHO)

ECHO (General) Tissue Doppler Analysis

ECHO (General)

Aortic regurgitation is common in ankylosing spondylitis - time for routine echocardiography evaluation? As aortic regurgitation and/ or conduction abnormalities might cause insidious symptoms, both electrocardiography and echocardiography evaluation should be part of the routine management of patients with ankylosing spondylitis. PubMed, Am J Med, 06/04/2015. (Also see Ankylosing Spondylitis)

Value of systolic pulmonary arterial pressure as a prognostic factor of death in the systemic sclerosis EUSTAR population. An estimated systolic pulmonary artery pressure >36 mmHg at baseline echocardiography was significantly and independently associated with reduced survival. PubMed, Rheumatology (Oxford), 01/17/2015.

Tissue Doppler Analysis ECHO

Right ventricular systolic dysfunction often precedes the onset of symptoms in systemic sclerosis, however it is not apparent by Tissue Doppler, but can be detected by Strain.

Tissue Doppler ECHO. This test is used to look at how blood flows through the heart chambers, heart valves, and blood vessels. WebMD.

Early Detection of PAH in Systemic Sclerosis Achieved Using Ultrasound Technology. Using right heart catheterization as the gold standard in all patients showed that stress Doppler-echocardiography markedly improved sensitivity in detecting manifest Pulmonary Hypertension. Scleroderma News, 06/22/2015. (Also see Diagnosis of Pulmonary Hypertension)

Electrocardiogram (ECG or EKG)

Electrocardiogram. An electrocardiogram (EKG or ECG) is a test that checks for problems with the electrical activity of your heart. WebMD.

Heart rate turbulence assessment in systemic sclerosis: the role for the detection of cardiac autonomic nervous system dysfunction. HRT, like HRV assessment, indicates a frequent impairment of the cardiac autonomic nervous system in SSc patients, irrespective of the SSc type. Rheumatology.

Heart Rate

Problem with Heart Rate can be determined with ECG/EKGs and Holter Monitors.

Panic attacks. Panic attacks were once dismissed as nerves or stress, but they're now recognized as a real medical condition. Signs and symptoms may include rapid heart rate, sweating, trembling, shortness of breath and hyperventilation, chills, hot flashes, nausea, abdominal cramping, chest pain, headache, dizziness, faintness, tightness in your throat, trouble swallowing, a sense of impending death. Other health problems— such as an impending heart attack, an overactive thyroid gland (hyperthyroidism) or drug withdrawal— can cause similar signs and symptoms. Mayo Clinic. (Also see Lung Involvement, Heart Involvement and Thyroid Disease)


Relationship Between Ventricular Arrhythmias, Conduction Disorders, and Myocardial Fibrosis in Patients With Systemic Sclerosis. Delayed–enhancement magnetic resonance imaging can identify myocardial fibrosis in a high percentage of scleroderma patients. PubMed, J Clin Rheumatol, 12/01/2017.

Cardiac magnetic resonance (CMR) in systemic sclerosis patients with cardiac symptoms. Myocardial involvement in systemic sclerosis can be assumed by the presence of multiple pathologic MRI findings and CMR seems to be a valuable tool to identify and assess the presence of cardiac involvement. PubMed, Eur Rev Med Pharmacol Sci, 2017 Nov;21(21):4797-4803.

Pseudo–infarction pattern in diffuse systemic sclerosis (dSSc). Evaluation using cardiovascular magnetic resonance (CMR). CMR unveiled that the pattern of myocardial fibrosis in dSSc with Q waves is due to the systemic disease and not to coronary artery disease. PubMed, Int J Cardiol, 2016 Apr 8;214:465-468.

Holter Monitoring

Scleroderma Heart Disease Deaths Might Be Prevented with Heart Monitor. Magnetic resonance imaging (MRI) confirmed rhythm changes in both the upper and lower chambers of the heart, as well as an abnormally fast heart rate and complete heart block which was shown on the heart monitors. Scleroderma News, 06/10/2016.


Scintigraphy may help detect scleroderma cardiac involvement, even before there are heart symptoms.

Definition of Scintigraphy. A diagnostic test that uses radioisotopes. MedicineNet.

Go to Research for Cardiac Involvement

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