This article appears in the following Rheumatology issue: Ten years of partnership: translating ideas into progress in systemic sclerosis [View the issue table of contents]
Reviews |
Cardiac complications of systemic sclerosis
1University of Paris Descartes, Department of Rheumatology A, Cochin Hospital, AP-HP, Paris, France, 2Royal Free & University College Medical School, London, UK and 3Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.
Correspondence to: A. Kahan, Service de Rhumatologie A, Hôpital Cochin, 27 rue du Faubourg Saint-Jacques, 75014 Paris, France. E-mail: andre.kahan{at}cch.aphp.fr
 |
Abstract |
|---|
The majority of patients with SSc are believed to have subclinical primary cardiac involvement. Overt cardiac manifestations of SSc are associated with poor prognosis and can be difficult to manage. Primary myocardial disease, i.e. without systemic or pulmonary hypertension and without significant pulmonary or renal disease, is postulated to be due to microvascular ischaemia. Undetected early cardiac manifestations can progress silently to myocardial fibrosis. Symptoms may manifest without warning and can rapidly lead to arrhythmia and left and right heart dysfunction and failure. Of the currently practical screening methods, annual echocardiography and/or evaluation of N-terminal portion of pro-B-type natriuretic peptide concentrations should therefore be employed in SSc patients, in order to anticipate the development of cardiac symptoms. Although there is limited evidence in respect of specific therapeutic options, treatment of early abnormalities with calcium channel blockers and angiotensin-converting enzyme inhibitors may improve myocardial perfusion and function, while standard management of overt cardiac disease is equally appropriate in the SSc population. However, it remains to be seen if early intervention can limit the progression of these life-threatening complications.
KEY WORDS: Cardiomyopathy, Small coronary artery disease, Myocardial fibrosis, Pericarditis, Arrhythmias, Systemic sclerosis, Vasodilators
Submitted 30 July 2008;
revised version accepted 2 April 2009.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  P. Dorfmuller, D. Montani, and M. Humbert Beyond arterial remodelling: pulmonary venous and cardiac involvement in patients with systemic sclerosis-associated pulmonary arterial hypertension Eur. Respir. J., January 1, 2010; 35(1): 6 - 8. [Full Text] [PDF]
|
|