Abstract
Treatment of giant cell myocarditis (GCM) can require bridging to orthotopic heart transplantation (OHT) or recovery with mechanical circulatory support (MCS). Since the roles of MCS and immunotherapy are not well-defined in GCM, we sought to analyze outcomes of patients with GCM who required MCS. A systematic search was performed in June 2019 to identify all studies of biopsy-proven GCM requiring MCS after 2009. We identified 27 studies with 43 patients. Patient-level data were extracted for analysis. Median patient age was 45 (interquartile range (IQR): 32–57) years. 42.1% (16/38) were female. 34.9% (15/43) presented in acute heart failure. 20.9% (9/43) presented in cardiogenic shock. Biventricular (BiVAD) MCS was required in 76.7% (33/43) of cases. Of the 62.8% (27/43) of patients who received immunotherapy, 81.5% (22/27) used steroids combined with at least one other immunosuppressant. Cyclosporine was the most common non-steroidal agent, used in 40.7% (11/27) of regimens. Immunosuppression was initiated before MCS in 59.3% (16/27) of cases, after MCS in 29.6% (8/27), and not specified in 11.1% (3/27). Immunosuppression started prior to MCS was associated with significantly better survival than MCS alone (p = 0.006); 60.5% (26/43) of patients received bridge-to-transplant MCS; 39.5% (17/43) received bridge-to-recovery MCS; 58.5% (24/41) underwent OHT a median of 104 (58–255) days from diagnosis. GCM recurrence after OHT was reported in 8.3% (2/24) of transplanted cases. BiVAD predominates in mechanically supported patients with GCM. Survival and bridge to recovery appear better in patients on immunosuppression, especially if initiated before MCS.
Highlights
Giant cell myocarditis (GCM) is a rare autoimmune disorder characterized by diffuse myocardial necrosis with multinucleated giant cells [1]
Patients reported the onset of symptoms a median of 7 (7–10) days prior to presentation. 34.9% (15/43) of patients presented in acute heart failure, and 20.9% (9/43) presented in cardiogenic shock
Patients who received mechanical circulatory support (MCS) + IS had a significantly higher left ventricular ejection fraction (LVEF) at presentation compared to patients who received MCS alone (28% (20–30) vs. 17% (13–20), p = 0.03)
Summary
Giant cell myocarditis (GCM) is a rare autoimmune disorder characterized by diffuse myocardial necrosis with multinucleated giant cells [1]. First described in 1905 by Saltikow [2], GCM is often mistaken for other infiltrative cardiomyopathies [3] such as cardiac sarcoidosis and lymphocytic myocarditis. These diseases may present and share some histopathologic features, GCM is most associated with a poor prognosis [4], resulting in severe heart failure and ventricular arrhythmias. Jh. iCsltion.pMatehd.o2l0o2g0,i9c,f3e9a05tures, GCM is most associated with a poor prognosis [4], resulting in severe 2hoefa1r1t failure and ventricular arrhythmias. He teiamret foafilduiraegbnyosciosnistr5a2s.t5hyaesaarsm[6e]afnoar gpeatoifendtisagwniothsiGs aCtM75, wyeitahrsso[7m].eSpinrecseepnatitniegnitns yafofuecntgedadbuylthgoiaondt. Tuhtceopmuersposoef oimf tmhiusnsotusudpypirsetsosieovnailnuamteectheancihcaarlalyctseurpisptiocrsteadndpaotuietcnotsmwesitohfgimianmtucnelolsmupyopcraesrdsiiotins.in mechanically supported patients with giant cell myocarditis
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