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Outcomes of Mechanical Circulatory Support for Giant Cell Myocarditis: A Systematic Review
Preeyal M Patel1, Abhiraj Saxena1, Chelsey T Wood1
1Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA 19107, USA.
Insights
Giant cell myocarditis (GCM) treatment often requires mechanical circulatory support (MCS). Immunosuppression, particularly before MCS initiation, significantly improves survival and bridge-to-recovery outcomes in GCM patients.
Area of Science:
- Cardiology
- Immunology
- Critical Care Medicine
Background:
- Giant cell myocarditis (GCM) is a rare and aggressive form of heart inflammation.
- Treatment strategies for GCM, especially involving mechanical circulatory support (MCS) and immunotherapy, are not well-defined.
- Patients with GCM often require advanced support, including MCS, to bridge to recovery or heart transplantation.
Purpose of the Study:
- To analyze the outcomes of patients diagnosed with biopsy-proven GCM who required MCS.
- To investigate the impact of immunotherapy timing on survival and bridge-to-recovery rates in MCS-supported GCM patients.
Main Methods:
- A systematic literature search was conducted in June 2019, identifying studies of GCM patients requiring MCS published after 2009.
- Patient-level data from 27 studies involving 43 patients were extracted and analyzed.
- Outcomes were assessed based on MCS type, immunotherapy use and timing, bridge-to-transplant or bridge-to-recovery status, and orthotopic heart transplantation (OHT) outcomes.
Main Results:
- Biventricular assist device (BiVAD) MCS was predominantly used (76.7%).
- Of patients receiving immunotherapy (62.8%), most used steroids combined with other immunosuppressants (e.g., cyclosporine).
- Initiating immunosuppression before MCS was associated with significantly better survival (p = 0.006) compared to MCS alone. Bridge-to-recovery rates were also improved.
Conclusions:
- Biventricular MCS is the primary support method for GCM patients.
- Immunotherapy, especially when initiated prior to MCS, is crucial for improving survival and facilitating bridge-to-recovery in GCM.
- Orthotopic heart transplantation (OHT) is a viable option, with a low rate of GCM recurrence post-transplant.
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.
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