Advanced Heart Failure & Transplant Cardiology Fellow The University of North Carolina University of North Carolina Durham, North Carolina, United States
Background: Giant cell myocarditis is a rapidly progressive disease can be fatal if not quickly treated. Temporary mechanical circulatory support is often used to bridge to destination therapies. We present a case of cardiogenic shock due to giant cell myocarditis in which veno-arterial extracorporeal membrane oxygenation (VA-ECMO) was used as bridge-to-recovery.
Methods: A 64-year-old male with a history of polymyalgia rheumatica (PMR), alcohol use disorder, and depression presented to the emergency department with roughly two weeks of progressive body aches, malaise, and fatigue which acutely worsened with new chest tightness and abdominal fullness the night prior to presentation. Five months prior, he had been weaned from PMR-directed therapies which included prednisone and methotrexate. In the emergency department, he was noted to be hypotensive. Lactate was elevated to 3.9 mmol/L. ECG demonstrated complete heart block with junctional tachycardia. He was emergently taken to the catheterization laboratory where an intra-aortic balloon pump was placed and intravenous dobutamine infusion was initiated. Right heart catheterization revealed a severely reduced cardiac output of 2.3 L/min (cardiac index of 1.1 L/min/m2) which improved to only 2.6 L/min after initiation of pacing via transvenous pacemaker. Endomyocardial biopsies were taken at the time of right heart catheterization for expedited evaluation. Given his persistent shock despite IABP and temporary pacing support, he was then intubated and cannulated for peripheral VA-ECMO with a right femoral artery/left atrial configuration, and the IABP was removed.
Outcome: On hospital day 1, endomyocardial biopsy confirmed the diagnosis of giant cell myocarditis. He was initiated on intravenous methylprednisolone 1 g/day for 3 days in addition to mycophenolate and tacrolimus. Transthoracic echocardiography showed severely reduced left ventricular ejection fraction (LVEF) of < 15%. Hemodynamics subsequently improved. On day 5, he developed septic shock due to Enterococcus faecalis bacteremia for which immunosuppression medications were held. Complete heart block resolved, and transvenous pacemaker was then removed. Repeat echocardiogram on hospital day 9 demonstrated improvement of LVEF to 25%. However, blood cultures showed persistent Enterococcus bacteremia. Due to concern for seeding of the ECMO circuit, support was weaned under transesophageal echocardiographic guidance on day 12 which showed stable biventricular function with flow rates at 1.5 L/min. He was de-cannulated on day 13 at which time repeat echocardiography demonstrated recovery of his LVEF to 40%. His prior immunosuppression regimen was resumed, but he developed persistent altered mental status due to reversible cerebral vasoconstriction syndrome from tacrolimus limiting his ability to be extubated. After discontinuation of tacrolimus, his mental status gradually improved, and was later extubated and subsequently discharged. He ultimately returned home, and his ejection fraction recovered to normal within two months of hospital discharge.
Conclusion: This case illustrates successful utilization of VA-ECMO as a bridge-to-recovery in a patient with SCAI Stage E shock from giant cell myocarditis. It highlights the utility of early endomyocardial biopsy in patients with undifferentiated cardiogenic shock, particularly among patients with suspected myocarditis such that immunosuppression can be rapidly initiated.
