Heart involvement in systemic sclerosis: evolving concept and diagnostic methodologies

Arch Cardiovasc Dis. 2010 Jan;103(1):46-52. doi: 10.1016/j.acvd.2009.06.009. Epub 2009 Nov 28.


Heart involvement, including primary myocardial involvement, is very common in systemic sclerosis. There is strong evidence that primary myocardial involvement is related to repeat focal ischaemic injury causing subsequent irreversible myocardial fibrosis. Clinically evident cardiac involvement is recognized to be a poor prognostic factor; thus preclinical identification is highly encouraged. The severity of heart involvement has been confirmed recently. Echocardiography, including pulsed tissue Doppler echocardiography, is the cornerstone of routine heart assessment. Myocardial perfusion may be assessed by single photon emission computed tomography. If available, cardiac magnetic resonance imaging should be considered as it allows simultaneous measurement of ventricular volumes and function and myocardial perfusion, and assessment of possible inflammation and/or fibrosis. Biological variables, such as B-type natriuretic peptides, are highly relevant, valuable markers of global heart involvement in systemic sclerosis and should be considered for screening of patients and/or research purposes.

Publication types

  • Review

MeSH terms

  • Biomarkers / blood
  • Diagnostic Imaging* / methods
  • Echocardiography, Doppler
  • Fibrosis
  • Heart / physiopathology*
  • Heart Diseases / diagnosis*
  • Heart Diseases / etiology
  • Heart Diseases / pathology
  • Heart Diseases / physiopathology
  • Heart Function Tests* / methods
  • Humans
  • Magnetic Resonance Imaging
  • Myocardial Perfusion Imaging
  • Myocardium / pathology*
  • Practice Guidelines as Topic
  • Predictive Value of Tests
  • Prevalence
  • Prognosis
  • Risk Assessment
  • Risk Factors
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / pathology
  • Scleroderma, Systemic / physiopathology
  • Severity of Illness Index


  • Biomarkers