Which cardiac findings are characteristic of scleroderma?

Updated: Sep 09, 2019
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Cardiac involvement indicates a worse prognosis. Cardiac arrhythmias and conduction abnormalities, including complete A-V block, may be revealed during physical exam but must be confirmed by routine electrocardiography or, more accurately, with 24-hour Holter monitor or echocardiography.

Pericardial effusion may develop in up to one third of patients with systemic sclerosis but is usually asymptomatic. Clinically significant pericarditis is rare.

Other cardiac findings in systemic sclerosis are as follows:

  • Cor pulmonale may develop secondary to pulmonary fibrosis and/or pulmonary artery hypertension

  • Infiltrative cardiomyopathy with replacement of cardiac muscle by fibrous tissue can lead to arrhythmias, heart failure, or both

  • Contraction band necrosis results from global ischemia and reperfusion; patients may have recurrent episodes of vasospasm that are caused by the same mechanism involved in Raynaud phenomenon; this process can lead to cardiomyopathy and heart failure

  • Non-atherosclerotic myocardial infarction may result from inflammation and fibrotic narrowing of coronary vessels

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