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Effusive constrictive pericarditis in systemic sclerosis.

A 69-year old man presented with a 7-day history of worsening heart failure on a background of systemic sclerosis (SSc). He had a history of pericardial effusion, Raynaud’s phenomenon, and achalasia (Figure 1). Examination revealed advanced sclerodactyly, mild hypertension, soft heart sounds with pericardial rub, and fine inspiratory crackles bilaterally. Echocardiography demonstrated a left ventricular ejection fraction of 45% and a large pericardial effusion (Figure 2a). Thoracic computed tomography also showed a thickened and densely calcified pericardium (Figure 2b, Figure 2c), leading to a diagnosis of effusive constrictive pericarditis (ECP). Near total pericardiectomy was performed via median sternotomy without using cardiopulmonary bypass (Figure 2c, long arrow). Histopathological examination with hematoxylin and eosin staining reconfirmed calcific pericarditis with extensive hemorrhagic fibrinous exudate (Figure 2d).