Aims: Giant-cell myocarditis (GCM) is a rare and often fatal form of myocarditis. Only a few reports have focused on fulminant forms. We describe the clinical characteristics, management and outcomes of GCM patients rescued by mechanical circulatory support (MCS).
Methods and results: The clinical features, diagnoses, treatments and outcomes of MCS-treated patients in refractory cardiogenic shock secondary to fulminant GCM admitted to eight French intensive care units (2002-2016) were analysed. We also conducted a systematic review of this topic. Thirteen patients (median age 44 [range 21-76]years, Simplified Acute Physiology Score II 55 [40-79]) in severe cardiogenic shock (median [range] left ventricular ejection fraction 15% [15-35%] and blood lactate 4 mmol/L) were placed on MCS 4 [0-28]days after hospital admission. Severe arrhythmic disturbances were frequent (77%), with six (46%) patients experiencing an electrical storm prior to MCS. Venoarterial extracorporeal membrane oxygenation was the first MCS option for 11 (85%) patients. GCM was diagnosed in five (38%) patients before transplant or death and treated with immunosuppressants; infections were the main complication (80%). Four patients died on MCS and no patient presented long-term survival free from heart transplant (nine patients, 69%). All transplanted patients were alive 1year later and no GCM recurrence was reported after median follow-up of 42 [12-145]months.
Conclusion: Outcomes of fulminant GCMs may differ from those of milder forms. In this context, heart transplant might likely be the only long-term survival option.
Keywords: Extracorporeal membrane oxygenation; Giant-cell myocarditis; Heart transplant; Immunosuppressive therapy; Myocardial biopsy.
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