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Cardiac tamponade and large pericardial effusions in systemic sclerosis

A report of four cases and a review of the literature

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Abstract

Cardiac tamponade in systemic sclerosis is rare. We report four cases of SSc with hemodynamically significant pericardial effusions associated with pulmonary arterial hypertension, three of whom died, two following pericardiocentesis. Of 26 SSc cases reported in the literature with large pericardial effusions, seven were associated with PAH. Including our series, the mortality rate is 55%.The potential contributory role of PAH in the development of pericardial effusion and the management implications are explored. In SSc patients with hemodynamically significant pericardial effusions and severe pulmonary hypertension, initial stabilization of pulmonary artery pressure and right heart function with vasoactive therapy and then cautious pericardial drainage should be considered.

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Abbreviations

SSc:

Systemic sclerosis

lSsc:

Limited scleroderma

dSSc:

Diffuse scleroderma

PAH:

Pulmonary artery hypertension

iPAH:

Idiopathic pulmonary hypertension

RVSP:

Right ventricular systolic pressure

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Acknowledgment

The authors thank Dr. A. Al-Riyami for his assistance in selecting echocardiogram images for this article.

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None.

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Authors and Affiliations

  1. Department of Medicine, St. Paul’s Hospital and University of British Columbia, Vancouver, BC, Canada

    James V. Dunne, Pearce Wilcox & Simon H. Huang

  2. Department of Medicine, University of Calgary, Calgary, AB, Canada

    Julie P. Chou

  3. Department of Cardiology, Medical Center, University of Washington, Seattle, WA, USA

    Mohan Viswanathan

Authors
  1. James V. Dunne
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  2. Julie P. Chou
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  3. Mohan Viswanathan
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  4. Pearce Wilcox
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  5. Simon H. Huang
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Corresponding author

Correspondence to James V. Dunne.

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Dunne, J.V., Chou, J.P., Viswanathan, M. et al. Cardiac tamponade and large pericardial effusions in systemic sclerosis. Clin Rheumatol 30, 433–438 (2011). https://doi.org/10.1007/s10067-010-1667-0

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  • DOI: https://doi.org/10.1007/s10067-010-1667-0

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