PERICARDIAL DECOMPRESSION SYNDROME IN A PEDIATRIC PATIENT WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND MACROPHAGE ACTIVATION SYNDROME: A CASE REPORT

Tiep Pham Khac1, Hung Dau Viet2, Kha Ngo Manh2, Dung Tran Ba2
1 Vinmec Times City International Hospital
2 Vietnam National Children’s Hospital

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Abstract

Pericardial Decompression Syndrome (PDS) is a rare but life-threatening complication characterized by high mortality rates following the large-volume drainage of pericardial effusion. We report a clinical case of a 13-year-old female patient diagnosed with Systemic Lupus Erythematosus (SLE) complicated by Macrophage Activation Syndrome (MAS) and multi-organ failure. The patient presented with acute cardiac tamponade on a background of pre-existing left ventricular systolic dysfunction and myocardial injury. Despite technically successful surgical decompression of the pericardium and pleura, the patient experienced sudden hypotension then cardiac arrest immediately after the procedure. This report focuses on analyzing the complex pathophysiological mechanisms of PDS in the context of systemic inflammation and provides recommendations for drainage management in high-risk patient groups.

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