Abstract
Primary myocardial involvement is common in systemic sclerosis. Increasing evidence strongly suggests that this involvement is related to repeated focal ischemic injury causing irreversible myocardial fibrosis. The underlying mechanism appears to be microcirculatory impairment with abnormal vasoreactivity, with or without structural vascular abnormalities. Clinically evident cardiac involvement is recognized to be a poor prognostic factor. Pericardial involvement is frequent but usually asymptomatic. Conduction system abnormalities appear common but not serious, while arrhythmias may be life-threatening. No significant valvular involvement appears to be associated with systemic sclerosis. Treatment for myocardial involvement includes long-term systematic administration of calcium channel blockers and possibly angiotensin-converting enzyme inhibitors, frequently given at high dosage.
MeSH terms
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Angiography
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Angiotensin-Converting Enzyme Inhibitors / administration & dosage
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Angiotensin-Converting Enzyme Inhibitors / therapeutic use
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Arrhythmias, Cardiac / etiology
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Calcium Channel Blockers / administration & dosage
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Calcium Channel Blockers / therapeutic use
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Cardiomyopathies / etiology
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Cohort Studies
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Coronary Circulation
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Endomyocardial Fibrosis / etiology
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Follow-Up Studies
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Heart Diseases / diagnosis
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Heart Diseases / diagnostic imaging
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Heart Diseases / drug therapy
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Heart Diseases / etiology*
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Heart Diseases / mortality
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Heart Valve Diseases / etiology
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Humans
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Magnetic Resonance Imaging
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Microcirculation
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Myocardial Contraction
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Pericardial Effusion / etiology
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Positron-Emission Tomography
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Prognosis
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Scleroderma, Systemic / complications*
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Scleroderma, Systemic / physiopathology
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Sensitivity and Specificity
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Time Factors
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Ventricular Dysfunction, Left / etiology
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Ventricular Dysfunction, Right / etiology
Substances
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Angiotensin-Converting Enzyme Inhibitors
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Calcium Channel Blockers