Fulminant giant-cell myocarditis on mechanical circulatory support: Management and outcomes of a French multicentre cohort

Abstract 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.