Hemorrhagic Cardiac Tamponade as a Complication of Limited Cutaneous Systemic Sclerosis

Abstract

Cardiac tamponade is an uncommon complication of systemic sclerosis (SSc) with a high mortality rate. Here, we report a case of a 58-year-old patient with limited cutaneous systemic sclerosis (lcSSc), gastroesophageal reflux disease (GERD), diabetes mellitus, pulmonary hypertension (PHTN), and COVID-19 infection, which occurred one month ago, presenting with a large hemorrhagic pericardial effusion and early cardiac tamponade. The patient had an acute onset of progressive dyspnea and anasarca. On examination, she was tachypneic, tachycardic, desaturating on room air, and hypotensive. Pitting edema up to thighs and bilateral basilar crackles were also appreciated. Labs were remarkable for negative troponin, chest X-ray with pulmonary congestion, D-dimer at 6.01, CT angiogram negative, brain natriuretic peptide level at 73 pg/mL, C-reactive protein level at 7.64 mg/dL, normal complement levels, and negative COVID-19 test results. Echocardiography showed early tamponade and a large circumferential effusion with chamber collapse. Right heart catheterization was performed finding PHTN at 54 mmHg. Pericardiocentesis drained 500 mL of the hemorrhagic effusion. Fluid analysis showed RBC at 220,000/uL, WBC at 5000/uL, protein 4.8 g/dL, lactate dehydrogenase level of 1275 U/L, and negative cytology. The patient was treated for serositis from lcSSc flare with mycophenolate mofetil and steroids, and responded very well. Hemorrhagic cardiac tamponade is a very rare phenomenon in limited scleroderma. A recent COVID-19 infection could have served as a trigger factor for our patient’s lcSSc in long remission to flare up. Clinicians should maintain a high index of suspicion and a low threshold for intervention when lcSSc patients have an acute onset of cardiac compromise, especially with a history of a recent COVID-19 infection.