Related Experiment Videos
Giant cell myocarditis: clinical presentation, bridge to transplantation with mechanical circulatory support, and
Ross A Davies1, John P Veinot, Stuart Smith
1Division of Cardiology, Department of Medicine, University of Ottawa Heart Institute, Ottawa, Ontario, Canada. radavies@ottawaheart.ca
Insights
Giant cell myocarditis can be challenging to diagnose before heart transplantation. However, mechanical circulatory support can bridge patients to transplant, and recurrence after transplantation may respond to intensified immunosuppression.
Area of Science:
- Cardiology
- Transplantation Medicine
- Immunology
Background:
- Giant cell myocarditis (GCM) is a rare and aggressive cardiac condition.
- Historical data show poor transplant-free survival and significant recurrence rates post-transplant.
Purpose of the Study:
- To evaluate the outcomes of heart transplantation in patients with giant cell myocarditis.
- To assess the efficacy of mechanical circulatory support and augmented immunosuppression in managing GCM.
Main Methods:
- Retrospective review of 340 heart transplantations performed since 1984.
- Identification of 7 patients with unexpected giant cell myocarditis in explanted hearts.
- Analysis of clinical presentation, management strategies, and post-transplant outcomes.
Main Results:
- Patients presented with varied timelines, from rapid deterioration to late decline, often undiagnosed pre-transplant.
- Mechanical circulatory support (total artificial heart, LVAD) was crucial for bridging 4 patients to transplant.
- Six patients survived with no recurrence; one died from graft vascular disease. Recurrence was observed in 4 patients, with one responding to augmented immunosuppression.
Conclusions:
- Giant cell myocarditis frequently presents insidiously or with rapid decline, often missed pre-transplant.
- Mechanical circulatory assist is effective for bridging GCM patients to heart transplantation.
- Recurrence of GCM post-transplant is possible but may be managed with intensified immunosuppressive therapy.
Background:
The multicenter Giant Cell Myocarditis Registry recorded 64 cases from 36 centers before 1996. The median transplant-free survival of 30 patients without immunosuppression was 3 months. Of 34 patients who received heart transplantations, 9 experienced recurrence of giant cell myocarditis in their transplanted hearts and 1 patient died.
Methods:
We reviewed our experience in 340 heart transplantations since 1984. Unexpected giant cell myocarditis was found in the explanted hearts of 7 patients (6 men and 1 female, aged 18-65 years).
Results:
The duration from the onset of symptoms to assist-device implant or transplantation ranged from 11 days to 9 years, whereas the time interval from referral or deterioration ranged from 2 days to 4 months. Four patients required mechanical circulatory support before surgery (total artificial hearts in 2 and left ventricular assist devices in 2), and 3 patients required inotropic drugs. Six patients are alive with no sign of recurrent giant cell myocarditis at 12 to 113 months after surgery. One patient died suddenly 75 months after surgery, and autopsy showed severe graft vascular disease with no recurrence of giant cell myocarditis. Surveillance, right ventricular endomyocardial biopsy specimens showed recurrent asymptomatic giant cell myocarditis in 3 patients at 5 to 13 months after surgery, and found recurrence in 1 patient 30 months after surgery. This patient received augmented immunosuppression.
Conclusions:
Giant cell myocarditis often is not diagnosed before transplantation. It can present as dilated cardiomyopathy with late deterioration, or it can present with rapid hemodynamic deterioration. In our experience, these patients can be bridged successfully to transplant with mechanical circulatory assist. Giant cell myocarditis may recur after transplantation but may respond to augmented immunosuppression.