A Real Circuit Breaker: Hyperhemolysis Syndrome Related to the VA-ECMO Circuit?

Abstract

<h3>Introduction</h3> Myocarditis is an inflammatory disease of cardiac muscle caused by a variety of infectious and non-infectious conditions. Viral infection is the most frequent cause of myocarditis; however, herpes simplex virus 1 (HSV-1) infection causing myocarditis has been rarely described. We present a case of a young woman with HSV-1 viremia and fulminant myocarditis presenting with cardiogenic shock requiring venoarterial extracorporeal membrane oxygenation (VA-ECMO), complicated by hyperhemolysis. <h3>Case Report</h3> A 35 year-old immunocompetent woman with moderate alcohol consumption presented to hospital with a 4-day history of fever and flu-like symptoms. She was fully vaccinated for COVID-19 two months prior to symptom onset. Her COVID-19 testing was negative and she was discharged home. She returned to hospital 4 days later in cardiogenic shock. Transthoracic echocardiogram demonstrated LVEF of 30% with a small pericardial effusion. Coronary angiogram revealed normal coronaries. She was placed on peripheral VA-ECMO for worsening cardiogenic shock. Due to inadequate LV unloading, she underwent atrial septostomy. Five days after VA-ECMO cannulation, HSV-1 was detected in the blood and she was started on intravenous acyclovir. Her ECMO course was complicated by acute kidney injury requiring dialysis, and hyperhemolysis with a peak LDH of 12,000 U/L. The mechanism of hemolysis was attributed to an intravascular process (plasma free hemoglobin 7487 mg/L, normal < 150 mg/L) likely from a combination cold agglutinins and the mechanical circuit. Interestingly the membrane pressure gradient was within normal. The patient received treatment with plasmapheresis (Table 1), and was eventually decannulated after 12 days following hemodynamic improvement. <h3>Summary</h3> This case report highlights a rare viral cause of fulminant myocarditis and emphasizes the need for collaboration among various specialists in the management of complex cases.