The case report of a 31-year-old man with giant cell myocarditis successfully treated with combined immunosuppression and veno-arterial extracorporeal membrane oxygenation for 21 days

Abstract

Introduction: Giant cell myocarditis (GCM) is a life-threatening condition attributed to a T-cell mediated inflammation of the myocardium. The most common early manifestation is acute heart failure. Recovery of myocardial function can occur with non-standardized combined immunosuppression therapy, hemodynamic support, and management of arrhythmias. Case report description: A 31-year-old man with a history of multiple autoimmune disorders (thyroiditis, ulcerative colitis, hepatitis) developed progressive dyspnoea and was transferred to our hospital with severe biventricular dysfunction (left ventricular ejection fraction –LVEF- of 10%) and cardiogenic shock unresponsive to pharmacological treatment 3 days after the onset of symptoms. Mechanically assisted ventilation, intraaortic counterpulsation (IABP) and veno-arterial extracorporeal membrane oxygenation (ECMO) were promptly instituted, and a right ventricle endomyocardial biopsy was performed, which provided the histologic diagnosis of GCM. On this basis, combined immunosuppression with tymoglobulin, steroids, and cyclosporine microemulsion was started. The day after the patient was weaned from the ventilator, but during the next days he remained dependent from circulatory supports, thus was screened for heart transplantion (HTx). After two weeks on IABP and ECMO, LVEF was still 10%, and urgent HTx was requested. No suitable donor heart was offered, and within a few more days a significant recovery of myocardial function was observed, with LVEF increasing up to 50% after 21 days from the initiation of mechanical support and 20 days of immunosuppression. IABP and ECMO were removed on day 21, and after additional 11 days the patient was discharged on cyclosporine and prednisone. One week after discharge, cardiac magnetic resonance (CMR) showed a LVEF 60% with diffuse areas of late gadolinium enhancement. Long term maintenance immunosuppression and surveillance with CMR have been planned. Discussion: Cardiac function may recover completely in GCM, but circulatory support is essential for maintaining hemodynamics and oxygenation, since immunosuppression may require days or weeks to allow resolution of myocardial damage and transplant-free survival. Conclusions: Although GCM is mostly reported to benefit from immunosuppression, no specific recommendations regarding drug combination, dosage and duration are available. In the unusual setting of GCM in a patient with multiorgan manifestations of autoimmunity, long term treatment with a drug combination with a well-known safety and toxicity profile, such as cyclosporine and low-dose prednisone, may be a reasonable choice.